Rheumatology Conference

8/7/2019 Rheumatology Conference

http://slidepdf.com/reader/full/rheumatology-conference 1/47

Question Number: 1

CaseA 70-year-old man with chronic obstructive pulmonary disease has a T8

vertebral compression deformity noted on a lateral chest radiograph taken

for evaluation of a chronic cough. The deformity was not present onradiographs taken 1 year ago. The patient does not have back pain and does

not recall any injury to his back. He has not been taking corticosteroids.

Laboratory data include:Serum testosterone: 205 ng/ml

DEXA bone densitometry:

lumbar spine T-score = -1.8

Femoral neck T-score = -1.9Question

Which of the following is the most appropriate therapeutic intervention at this

time?

A.Increase calcium intake to 1200 mg/day with vitamin D 800 IU/d

B.Oral alendronate 70 mg once weekly in addition to supplemental calcium and vitamin D

C.

Transdermal testosterone 5 mg at night in addition to supplemental calcium and vitamin

DD.

Teriparatide 20 mcg SC daily in addition to supplemental calcium and vitamin D

E.Intranasal calcitonin, 200 units once daily

Correct Answer

BAnswer RationaleThe presence of a vertebral compression fracture is sufficient to establish a

diagnosis of osteoporosis, even though the bone mineral density T-scores are

in the osteopenic range. If the bone mineral density in a man is evaluated

using reference data from a healthy female population, then osteoporosis will be significantly underdiagnosed. Given the presence of one vertebral

fracture, this patient is at high risk of additional fractures and needs to

receive drug therapy for his osteoporosis. The most effective therapies would be a bisphosphonate (e.g. alendronate) or teriparatide. Given the expense

and inconvenience of teriparatide, alendronate is the most appropriate first

therapy for this patient. Intranasal calcitonin has not been shown to reducefracture risk in men with osteoporosis. This patient has a low normal

testosterone level, most likely a consequence of an age-related decline in his

gonadal function. The benefits of testosterone therapy for the prevention of

osteoporotic fractures in hypogonadal men have not been established. There





is also uncertainty as to whether testosterone replacement improves bone mineral densityin older men with borderline low testosterone levels. Since androgen therapy has possible

undesirable effects on other organ systems, particularly the prostate gland, testosteronetherapy would not be the first choice of therapy for this man.

Question Number: 2

Case

An over-weight 14-year-old boy had been active at a soccer tournamentyesterday and complained of right knee pain when he returned home. Today

the pain is still present and worsens with weight bearing. He has been

previously diagnosed with mild hypothyroidism, and sometimes he forgets totake his replacement therapy.

Physical examination shows no abnormalities of the knee, but internal

rotation at the hips is limited.

Radiograph of the hips is shown in figure below.



Figure

Question

Which of the following is the most appropriate next step in management?A.

Nonsteroidal anti-inflammatory drug

B.Antibiotics after aspiration for presumed septic hip

C.

Non-weight bearing and orthopedic evaluationD.

Use of crutches for two weeks and weight bearing as tolerated

E.

Physical therapy program to increase the range of movement of his hips

Correct Answer

CAnswer RationaleChildren often complain of knee pain when the hip is the source of the

problem. SCFE is more common in obese adolescent boys and also in

children with hypothyroidism. It is a surgical emergency; the child should bemade non-weight bearing immediately, additional radiographs (including

frog-leg lateral view) should be obtained, and urgent orthopedic consultation

should be ordered. Treatment is surgical pinning. There is increased risk of slip on the other side, and some children develop aseptic necrosis or chondrolysis as a consequence of this condition.

Question Number: 3

CaseA 26-year-old man has had gradual onset of fixed proximal muscle weakness

over the past 3 to 4 years. He had a healthy childhood but began

experiencing exercise intolerance during his teen years. He often found that

when he exercised, he would experience dyspnea and muscle pain shortlyafter beginning the activity. These symptoms would force him to stop what

he was doing. However, after resting for 2 or 3 minutes, he could resume

the activity without discomfort. On one occasion, he experienced severecramping and muscle tenderness after going fishing and carrying his catch in

a bucket of water back to car, a distance of about a half mile. The cramps

lasted more than an hour and the muscle tenderness resolved in 48 hours.



On examination, he has 4/5 strength in proximal muscles of the upper and lower

extremities. Findings on examination of the joints and the remainder of the neurologic

examination are normal.Laboratory studies reveal CK of 900 U/L. EMG shows fibrillation potentials

and early recruitment of short-duration, low-amplitude motor unit action

potentials. A decremental response was observed to repetitive 20-Hz nervestimulation.

Question

Which of the following is the most likely finding on muscle biopsy?A.

Perivascular infiltration of lymphocytes with perifasicular atrophy and capillary plugging

B.

Occasional degenerating and regenerating fiber with PAS positive subsarcolemmalvacuoles

C.

Many small triangular fibers and fiber type grouping

D.Some type II fiber atrophy and increased oil red O staining in type I fibers

E.Many fibers with central nuclei, occasional hypertrophic cells, and ragged red changes on

Gamori

Trichrome staining

Correct Answer

BAnswer Rationale

McArdle’s disease is the most common glycogen storage disease. It may present in childhood with exercise intolerance; in teen years with episodes of

acute rhabdomyolysis associated with severe cramps, tenderness, and

weakness that often occur after heavy exertion; or in adulthood with thegradual onset of fixed proximal muscle weakness. The fixed weakness is felt

to be the result of recurrent rhabdomyolysis and an inability, after a time, to

regenerate normal muscle. Many patients report a “second wind”

phenomenon. They start to exercise but must stop, only then to be able toresume the activity after a brief rest. This is presumably the result of

increased blood flow and delivery of nutrients that occur in response to the

initial exercise. Most of these patients have an elevated CK level even whenasymptomatic and have myopathic changes on EMG. The finding of PAS

positive vacuoles confirms the diagnosis of a glycogen storage disease.

Perivascular infiltration of lymphocytes with perifasicular atrophy andcapillary plugging are classically seen in dermatomyositis.

Many small triangular fibers and fiber type grouping would be seen in a

neuropathic condition and imply denervation and reinervation.Some type II fiber atrophy and increase oil red O staining in type I fibers

occurs with lipid storage diseases like carnitine deficiency of some

mitochondrial myopathies.



Many fibers with central nuclei, occasional hypertrophic cell, and ragged red

change on Gamori Trichrome staining are indicative of a mitochondrial

myopathy.

Question Number: 3

Case

A 26-year-old man has had gradual onset of fixed proximal muscle weaknessover the past 3 to 4 years. He had a healthy childhood but began

experiencing exercise intolerance during his teen years. He often found that

when he exercised, he would experience dyspnea and muscle pain shortly

after beginning the activity. These symptoms would force him to stop whathe was doing. However, after resting for 2 or 3 minutes, he could resume

the activity without discomfort. On one occasion, he experienced severe

cramping and muscle tenderness after going fishing and carrying his catch in

a bucket of water back to car, a distance of about a half mile. The cramps

lasted more than an hour and the muscle tenderness resolved in 48 hours.On examination, he has 4/5 strength in proximal muscles of the upper and lower

extremities. Findings on examination of the joints and the remainder of the neurologic

examination are normal.Laboratory studies reveal CK of 900 U/L. EMG shows fibrillation potentials

and early recruitment of short-duration, low-amplitude motor unit action

potentials. A decremental response was observed to repetitive 20-Hz nervestimulation.

Question

Which of the following is the most likely finding on muscle biopsy?

A.Perivascular infiltration of lymphocytes with perifasicular atrophy and capillary plugging

B.

Occasional degenerating and regenerating fiber with PAS positive subsarcolemmal

vacuolesC.

Many small triangular fibers and fiber type groupingD.

Some type II fiber atrophy and increased oil red O staining in type I fibers

E.Many fibers with central nuclei, occasional hypertrophic cells, and ragged red changes on

Gamori



Trichrome staining

Correct Answer

BAnswer RationaleMcArdle’s disease is the most common glycogen storage disease. It may

present in childhood with exercise intolerance; in teen years with episodes of

acute rhabdomyolysis associated with severe cramps, tenderness, andweakness that often occur after heavy exertion; or in adulthood with the

gradual onset of fixed proximal muscle weakness. The fixed weakness is felt

to be the result of recurrent rhabdomyolysis and an inability, after a time, toregenerate normal muscle. Many patients report a “second wind”

phenomenon. They start to exercise but must stop, only then to be able to

resume the activity after a brief rest. This is presumably the result of

increased blood flow and delivery of nutrients that occur in response to theinitial exercise. Most of these patients have an elevated CK level even when

asymptomatic and have myopathic changes on EMG. The finding of PAS

positive vacuoles confirms the diagnosis of a glycogen storage disease.

Perivascular infiltration of lymphocytes with perifasicular atrophy andcapillary plugging are classically seen in dermatomyositis.

Many small triangular fibers and fiber type grouping would be seen in a

neuropathic condition and imply denervation and reinervation.

Some type II fiber atrophy and increase oil red O staining in type I fibersoccurs with lipid storage diseases like carnitine deficiency of some

mitochondrial myopathies.

Many fibers with central nuclei, occasional hypertrophic cell, and ragged redchange on Gamori Trichrome staining are indicative of a mitochondrial

myopathy.

Question Number: 5

Case

A 55-year-old woman has had an aching pain in her lower back for the past 2years.

Laboratory data include:Serum calcium: 8.2 mg/dl

Serum phosphorus: 2.3 mg/dl

Alkaline phosphatase: 175 U (normal 33-115 Units)25-hydroxyvitamin D: 8 ng/ml (normal 20-100 ng/ml)



1,25 hydroxyvitamin D: 40 pg/ml (normal 25-45 pg/ml)

Parathormone: 108 pg/ml (normal 10-65 pg/ml)

QuestionWhich of the following radiographic abnormalities is most likely in this

patient?

A.Increased bone mineral density

B.

Subperiosteal resorptionC.

Looser’s zones

D.

Erlenmeyer flask deformity of the distal femoraE.

Mixed areas of lucency and sclerosis in the long bones

Correct Answer

CAnswer RationaleThis patient has osteomalacia as a result of vitamin D deficiency.

Characteristically, both the serum calcium and the serum phosphorus levelsare decreased, while the serum alkaline phosphatase level is elevated. In

vitamin D deficiency, parathormone levels rise, causing an increase in the

metabolism of 25-hydroxyvitamin D to 1,25-hydroxyvitamin D. This in turn

results in a greater deficiency of vitamin D but also a normal or elevated

level of 1,25-hydroxyvitamin D.

The characteristic radiographic feature of osteomalacia is Looser’s zones or pseudofractures that appear as linear areas of rarefaction perpendicular to

the cortical surface of the bone. Increased bone mineral density is a feature

of osteopetrosis. Subperiosteal resorption occurs in primaryhyperparathyroidism. The Erlenmeyer flask deformity is a feature of

Gaucher’s disease. Mixed areas of lucency and sclerosis in the long bones are

a feature of Paget’s disease.

Question Number: 6

Case



A 21-year-old white woman has sudden onset of swelling and pain in her

right knee associated with warmth and erythema. Synovial fluid analysis of

fluid obtained from the knee reveals 21,000 WBC/mm3, and needle-shapednegatively birefringent crystals are found under polarized light microscopy.

Family history reveals that the patient’s father and brother have gout.

Laboratory data include:Serum creatinine : 2.9 mg/dl

Serum urate: 10.5 mg/dl

24-Hour urinary uric acid: 350 mgQuestion

Which of the following findings is most likely in this patient?

A.

She is homozygous for phosphoribosylpyrophosphate (PRPP) synthase overactivityB.

She is heterozygous for hypoxanthine-guanine phosphoribosyltransferase (HPRT)

deficiency

C.She homozygous for adenine phosphoribosyltransferase (APRT) deficiency

D.She has a mutation in the uromodulin (UMOD) gene

E.

She is homozygous for purine nucleoside phosphoylase (PNP) deficiency

Correct Answer DAnswer Rationale

Both PRPP synthase overactivity and partial HPRT deficiency cause

hyperuicemia and gout, but both have X-linked inheritance and, therefore, donot occur in women.

A deficiency of APRT causes affected individuals to have kidney stones

composed of 2,8 dihydroxyadenine.

Mutations in UMOD gene are responsible for familial juvenile hyperuricemicnephropathy (FJHN). FJNG is inherited as an autosomal dominant trait with a

high degree of penetrance and is usually associated with gout. Renal disease

usually develops in the second decade of life and progresses to end-stagerenal disease by mid-life.

PNP deficient individuals have selective T-cell immunodeficiency.

Question Number: 7

Case



A 52-year-old woman with seropositive erosive rheumatoid arthritis has

increasing joint pain, swelling, and stiffness and has been under stress at

work. She has been taking methotrexate for several months. On physicalexamination, she is anxious and has synovitis. ESR is 74 mm/hr. She is

reluctant to take an anti-TNF blocker and agrees to start leflunomide in

addition to continuing methotrexate.After several weeks, the joint pain and stiffness improve and follow-up

examination shows minimal synovitis; her mood is better. She returns for

evaluation two months later and findings on examination are unchangedexcept she has lost 4 pounds. CBC and chemistries are normal and ESR is 30

mm/hr.

At her next visit 2 months later, she has lost an additional 6 pounds and feels

well. Examination shows no synovitis or adenopathy.Laboratory data include:

CBC: normal

ESR: 26 mm/hr

TSH: 0.9 µU/mlLFT: normal

BUN: 23 mg/dlCreatinine: 1.0 mg/dl

Albumin: 4.0 gm/dl

Question

Which of the following is the most likely cause of the patient’s weight loss?A.

Active rheumatoid arthritis

B.Methotrexate associated lymphoma

C.

Hyperthyroidism

D.

AnxietyE.

Idiosyncratic effect of leflunomide

Correct Answer EAnswer Rationale

Rheumatoid arthritis is a systemic inflammatory disease that affects the

joints and extra-articular systems. Constitutional symptoms such as weightloss are not uncommon with uncontrolled disease. Patients with rheumatoid

arthritis are at increased risk for lymphoma, and methotrexate is known to

cause reversible lymphomas, but there is no indication that this patient has

lymphoma. She does not appear to be clinically hyperthyroid, and her anxiety improved.



Leflunomide is a disease-modifying anti-rheumatic drug that works by

inhibiting de novo pyrimidine biosynthesis. Actively dividing lymphocytes are

inhibited by the drug’s effect on the enzyme dihydroorotate dehydrogenase,which is directly involved in pyrimidine biosynthesis. Weight loss caused by

leflunomide is under recognized and may reflect the drug’s effect on

oxidative phosphorylation within the mitochondria, rather than a secondaryeffect resulting from diarrhea or anorexia. Cessation of leflunomide may

result in weight gain. The weight loss averaged between 19 and 53 pounds

and occurred within weeks to months of beginning leflunomide. Recognitionof this side effect is important since unnecessary additional testing may be

undertaken to investigate weight loss.

Question Number: 8

CaseA 49-year-old man has progressive swelling of the left foot. He had noted

tingling and burning in the foot at night for the past year. The foot then became swollen five months ago. One month ago, he began to note

discomfort in the foot with ambulation. He does not have pain in any other

joints. He has a 10-year history of diabetes mellitus type 2 and

hypertension. The patient’s current medications are losartan,hydrochlorothiazide, metformin, glyburide, and simvastatin. He drinks one

or two 12-ounce cans of beer on weekends. He is afebrile.

Physical examination is notable for pain, swelling, and erythema of thedorsum of the left foot and ankle. The midfoot and ankle are tender. There

is restricted range of motion of the ankle with pain. Deep tendon reflexes

are absent at both ankles. There is diminished vibration sensation in bothfeet and decreased proprioception of the toes of both feet. No ulcers or rash





are present. Hemoglobin level is 10.4 gm/dl.A radiograph of the left foot is shown below:Figure

Question

Which of the following is the most likely diagnosis?A.

Calcium pyrophosphate dehydrate deposition disease

B.Chronic gout

C.

Neuropathic osteoarthropathy

D.Osteomyelitis

E.

Reflex sympathetic dystrophy syndromeCorrect Answer

CAnswer Rationale

This patient has a long-standing history of diabetes mellitus type 2 andmonths of foot swelling. The radiograph shows hindfoot and midfoot joint



and osseous destruction, osteopenia, and reactive bone formation. No

chondrocalcinosis is present making CPPD arthropathy unlikely. The

radiographic changes are out of proportion to the pain the patientdescribes. This is unusual for reflex sympathetic dystrophy in which

causalgic pain is usually prominent and allodynia typically

present. Osteopenia and marked osseous destruction are not typical of

osteoarthritis. This radiograph does not show typical features of gout

including “punched out,” rounded defects marginally and erosions withoverhanging edges. Osteomyelitis may be present in diabetic patients with

foot ulcers, but this patient has no preexisting foot ulcer. The patient alsohas no paronychia, cellulitic skin features nor constitutional symptomsincluding fever to suspect infection.

Question Number: 9

CaseAn 8-year-old boy has had hectic fevers for the past 5 days, a diffuse

morbilliform rash on his trunk, swollen hands and feet, erythematous oral

mucosa with no exudates, cracked lips, and red eyes. He has diffuselymphadenopathy and slight hepatomegaly. He is very irritable.

Laboratory data include:

ESR: 80 mm/hr RBC: mild anemia

Hgb: 1.1 gm/dl

WBC: 17,000/mm3

Platelet count: 450,000/mm3Urinalysis: some WBCs but no bacteria or proteinuria

Echocardiography: normal

Spinal fluid analysis: a few lymphocytes but no organisms on gram stain

QuestionWhich of the following is the most appropriate treatment?

A.IVIG 2 gm/kg over 12 hours

B.

Aspirin 325 mg/dayC.

Prednisone 2 mg/kg/day



D.

Ibuprofen 40mg/kg /day

E.Infliximab 5 mg/kg single dose

Correct Answer

AAnswer RationaleThe clinical description is classic for Kawasaki disease. Although staph toxic

shock syndrome and EBV and CMV infections can sometimes present in a

similar way, echocardiographic changes are often later in the course.When there is a clinical index of suspicion for Kawasaki disease, the

recommended treatment is high dose IVIG over 12 hours. This is most

effective early in the course, before periungual desquamation or

echocardiographic changes have occurred. Corticosteroids may be used if

IVIG has failed; aspirin (not ibuprofen) is recommended as an antiplateletagent. Studies are ongoing to determine effectiveness of anti-TNF agents in

Kawasaki disease; their effectiveness in treatment is not yet established.

Question Number: 10Case

A 35-year-old man has had the gradual onset of pain and stiffness in his legs

when he jogs one-half mile on a treadmill. He has cramps in his calves andhamstrings toward the end of the half-mile jog. The pain, stiffness, and

cramps resolve with rest. Physical examination shows 4/5 hip flexor muscle

strength. Serum creatine kinase level is 324 U/L. Electromyography reveals

low amplitude motor unit action potentials. Ischemic forearm muscle testing produces the following results:

Venous Blood

lactate (mg/dL) NH3 (µg/dL)

At rest8

20Two minutes after exercise 9

90

QuestionWhich of the following deficiencies is the most likely diagnosis?

A.



Acid maltase

B.

Carnitine palmitoyltransferase IIC.

Myoadenylate deaminase

D.Myophosphorylase

E.

Phosphorylase b kinaseCorrect Answer

DAnswer Rationale

In phosphorylase b kinase deficiency there is a normal or only partially

impaired rise in venous lactate with exercise. Ischemic forearm testingreveals a rise in blood lactate with impaired increase in ammonia levels in

myoadenylate deaminase deficiency. In acid maltase deficiency the ischemic

forearm muscle test is normal. CPT II deficiency usually begins in childhood

with exercise-induced myalgia. The ischemic forearm test is normal in CPTII. McArdle’s disease, characterized by myophosphorylase deficiency, is the

only choice associated with a flat venous lactate response to ischemic

forearm exercise. Note that testing of muscle biopsy samples has in recent

years become a precise diagnostic tool to confirm enzyme defects inmetabolic muscle disease.

Question Number: 11

CaseA 73-year-old woman who has had deforming rheumatoid arthritis for the

past 20 years now has persistent neutropenia. She has been living in an

assisted care facility for the past 3 years. Despite bilateral knee

arthroplasties, her mobility and ability to live independently have been progressively impaired by arthritis of her ankles, feet, wrists, and hands. Past

therapies have included parenteral gold, penicillamine, hydroxychloroquine,

methotrexate, and etanercept. At the time of admission to the facility, her WBC was 3200/mm3 and her physician chose to maintain her on prednisone

7.5 mg qAM alone for her rheumatoid arthritis. Her only infection was a

urinary tract infection 3 months ago treated with oral ciprofloxacin. Other



than the 10-day course of ciprofloxacin, she has not been on any new

medications for the past 12 months.

WBC: 1800/mm3 (25% neutrophils, 65% lymphocytes)Hgb: 10.3 gm/dl

Peripheral smear: paucity of neutrophils, occasional large lymphocyte with

pale blue cytoplasm and azurophilic granulesQuestion

Which of the following studies is most likely to establish the diagnosis?

A.Abdominal ultrasonography to measure spleen size

B.

Flow cytometry of peripheral blood

C.Test for cyclic citrullinated peptide (CCP) antibodies

D.

Granulocyte antibody test

E.Test for antinuclear antibodies (ANA)

Correct Answer BAnswer Rationale

The leading diagnostic considerations for this patient are Felty’s syndrome

and large granular lymphocyte (LGL) syndrome. The LGL syndrome is a

clonal disorder of cytotoxic T-lymphocytes and has been classified asleukemia, even though it usually has an indolent clinical course. About 25%

of patients with LGL syndrome have rheumatoid arthritis. The demonstration

of a clonal population of CD3+, CD8+, CD16+, CD57+ T cells with flowcytometry of the peripheral blood would serve to establish the diagnosis of

LGL syndrome. Splenomegaly and granulocyte antibodies are common to both disorders.The histopathology of the bone marrow does not usually differentiate the two.

Question Number: 12

CaseA 44-year-old woman with a 20-year history of lupus undergoes follow-up

evaluation. Initial manifestations included photosensitivity, small joint

arthritis, fatigue with a positive ANA, dsDNA, and leukopenia. Her symptomsresponded to anti-inflammatory agents and hydroxychloroquine, which she



continues to take. She feels reasonably well but has noticed some difficulty

climbing stairs. Findings on physical examination are normal. CPK and TSH

levels are normal, and there is no evidence of lupus activity on serologicalstudies. She returns 1 month later, and proximal weakness affecting the hip

flexors is noted.

Laboratory data include:CBC: Normal

ESR: 33 mm/hr

CK: 250 U/LdsDNA: 26 IU

TSH: 4.9 µU/ml

Electrolytes: Potassium 3.4 mmoles/L

Mg: NormalCa: Normal

Question

Which of the following is the most likely cause of the patient’s proximal

muscle weakness?A.

Inclusion body myositisB.

Lupus flare

C.

Hydroxychloroquine myopathyD.

Hypothyroidism

E.Hypokalemia

Correct Answer

C

Answer Rationale

Antimalarial therapy is commonly used in patients with lupus. Side effects of

therapy include irreversible retinal toxicity, rash, and gastrointestinaldisturbance. A less common and under recognized complication of therapy is

myopathy, which does not appear to correspond with the dose used. The

pathogenesis of this myopathy is thought to be related to accumulation of hydroxychloroquine in lysosomes, which then inhibit lysosomal enzymes. It is

a reversible myopathy, and thus important to recognize. Inclusion body

myositis is more common in men, and primarily affects the distalmuscles. There is nothing to suggest a lupus flare, and although the TSH

level is at the high end of normal, hypothyroidism is unlikely to explain the

weakness.




An 86-year-old man was sent to the hospital from an Alzheimer’s unit 2 days

ago because of a 2-day history of a swollen, red, warm, and tender elbow. He has a temperature of 100.9 degrees F (38.27 degrees C). On

admission, WBC was 12,500/mm3 with 60% PMN, and chest radiograph

showed mild right lower lobe atelectasis. His elbow was aspirated and 4 cc of cloudy fluid were obtained. Synovial fluid analysis showed cloudy fluid with a

WBC of 95,000/mm3. Gram stain was negative, and the fluid was sent for

culture. The patient was started on an intravenous antibiotic to cover gram-

positive bacteria for presumed septic arthritis.He is now confused, but his mental status is reported to be his usual. His

temperature is 99.6 degrees F (37.55 degrees C). His elbow is held in a

flexed position and is slightly swollen in the area between the olecranon and

lateral epicondyle. It is minimally erythematous, warm, and tender. Heresists any attempt to move the elbow. Findings on examination of the other

joints are normal. CBC now has a WBC of 10,000/mm3. Radiograph of theelbow shows only soft tissue swelling. His synovial fluid and blood cultures

have no growth. Sputum gram stain shows a few gram-positive cocci in pairs

but grows out mixed flora.

QuestionWhich of the following is the most appropriate next step in management?

A.

Surgical open drainage of the jointB.

Continue present antibiotic regimen

C.Aspirate the elbow and look for crystals

D.

Add another antibiotic to cover gram-negative organisms





Correct Answer

CAnswer RationaleCPPD crystals can elicit an intense inflammatory response in a single jointthat may mimic septic arthritis. It is appropriate to start antibiotics in this

clinical setting, but when cultures are negative, one should suspect a crystal-

induced process. Open drainage is appropriate in septic arthritis, if the joint

effusion cannot be controlled by percutaneous aspiration.

Question Number: 14

CaseA 4-year-old girl has recent onset of rash over her knuckles and knees.

Nailfold capillaroscopy is shown in Figure 1 below.

FigureQuestion

The nailfold changes place this patient at highest risk for which of the

following?

A.



Calcinosis

B.

Pulmonary hemorrhage

C.

Retinal changes

D.

Gastrointestinal vasculitisE.

Corticosteroid resistance


Calcinosis occurs late in disease and mainly in patients who have had

prolonged disease activity.Both pulmonary hemorrhage and retinal changes on the basis of vasculitis

are rare in dermatomyositis, but can occur as a part of the vasculitic picture.

However, nailfold capillary changes are a good predictor of risk for gastrointestinal vasculitis, the most common vasculitic complication in

juvenile dermatomyositis.Corticosteroid resistance or dependence does not correlate with nailfoldchanges.

Educational Objective

To recognize juvenile dermatomyositis and the association of gastrointestinal

vasculitis in children with dermatomyositis and nailfold capillary changes.

Question Number: 15

CaseA 65-year-old woman with osteoporosis sustains her second thoracic

vertebral compression fracture within the past 12 months despite ongoing

therapy with alendronate for the past 3 years. She has routinely taken

calcium and vitamin D supplementation ever since osteoporosis was firstdiagnosed at age 58 years.

Question

Which of the following treatment regimens is most likely to be effective in



improving this patient’s bone mineral density over the next 3 years?

A.

Continue alendronate therapy aloneB.

Continue alendronate and administer daily PTH (1-34) concurrently for the next 18

months, after which both drugs are discontinued

C.

Continue alendronate and administer daily PTH (1-34) concurrently for the next 18months, after

which alendronate alone is continued

D.

Discontinue alendronate therapy and administer daily PTH (1-34) for the next 18 months

Correct Answer

CAnswer Rationale

Cosman et al (2005) randomized 126 women who had been on alendronatefor at least 1 year to continue alendronate and to receive daily PTH (1-34),cyclic (3 month on/3 month off) PTH (1-34), or alendronate alone. Spine

bone mineral density increased 6.1% in the daily PTH (1-34) and 5.4% in the

cyclic PTH (1-34) groups, but remained unchanged in the alendronate alone

group. In a study by Black et al (2005), patients originally randomized to 1year of treatment with PTH (1-84) were subsequently randomized to receive

alendronate or placebo. Lumbar spine bone mineral density continued to

increase in both groups in the first year following discontinuation of PTH (1-84), but continued to rise in the second year only in the alendronate-treated

patients. In patients who have never been treated with alendronate, there is

no clear evidence that the combination of PTH with alendronate provides asynergistic or additive effect on bone mineral density as compared to PTH

alone (Black et al, 2003). These studies thus support the conclusion that this

patient would have the greatest increase in bone mineral density at the end

of 3 years if she receivs PTH in addition to alendronate for the first 18months, and then alendronate alone after the discontinuation of PTH.

Question Number: 16

CaseA 61-year-old man was recently admitted to the medical service for

management of congestive heart failure. While hospitalized, he experiences

acute and spontaneous onset of right knee pain and swelling.He has a longstanding history of seronegative nodular rheumatoid arthritis

that has been refractory to disease-modifying anti-rheumatic drugs

(DMARDs), including hydroxychloroquine and methotrexate, but responsive

to brief courses of prednisone.



On physical examination, his fingers are deformed with nodules at the

proximal but not distal IP joints (see figure below). He has bilateral, cool of

the olecranon bursa. His right knee is visibly swollen, erythematous, andwarm to the touch and has a ballotable effusion.

Figure

Question

Which of the following is the most appropriate course of action?A.

Obtain a radiograph of the inflamed knee

B.



Perform arthrocentesis on the inflamed knee

C.

Start a nonsteroidal anti-inflammatory drug (NSAID)D.

Start “pulse” corticosteroids


Although the patient carries the diagnosis of “nodular rheumatoid arthritis” and is already

being treated with disease-modifying agents, a patient with rheumatoid nodules would beexpected to be seropositive for rheumatoid factor. This merits a reassessment of his

diagnosis and management.

The most appropriate course of action is to perform arthrocentesis to assist

in both reevaluating the underlying diagnosis and in determining why this joint is flaring out of proportion to the other joints in what is normally a

symmetric polyarthritis–rheumatoid arthritis.

Radiography would be useful in ruling out a bony injury if there were a

history of trauma. Instead, the patient’s symptoms developed spontaneouslyand only after admission. NSAIDs and/or pulse corticosteroids are effective

interventions for the acute management of many inflammatory arthritides, but their salt- and fluid-retentive effects could be deleterious to the patient

because he has congestive heart failure.

Question Number: 17

Case

A 42-year-old woman with longstanding lupus treated with low-dosecorticosteroids and hydroxychloroquine has been doing well for many years.

She presented at age 18 with severe Raynaud’s phenomenon and finger

necrosis that was treated with IV cyclophosphamide. Other manifestations

included chronic thrombocytopenia and pulmonary embolus secondary toanti-phospholipid antibodies. She has never had cardiac or kidney

involvement. The patient would like to stop taking hydroxychloroquine

because she has been doing so well and finds the yearly eye examinations

onerous.Question

Which of the following is most appropriate advice to the patient at this time?A.

Stop the annual eye examinations

B.Discontinue the hydroxychloroquine

C.



Continue the hydroxychloroquine

D.

Discontinue visual field testing at her annual eye examinationsE.

Increase the dose of corticosteroids

Correct Answer CAnswer Rationale

Hydroxychloroquine is a useful treatment for the cutaneous and

musculoskeletal manifestations of lupus. It has little effect upon nephritis or other major organ involvement. Assays are available to assess serum

concentrations of hydroxychloroquine, as low levels have been shown to be

associated with increased disease activity. Irreversible retinal toxicity is rare

but occurs with greater frequency the longer the drug is used. The permanent visual damage results from binding of the drug to melanin. The

recommended dose is not greater than 6.5 mg/kg. Discontinuation of

hydroxychloroquine has been associated with an increased risk of flares. Eye

examinations should be continued while the patient takes antimalarialmedications, especially if the patient has been taking them for many

years. A full visual field and color test is required to optimally track theretinal effects of hydroxychloroquine. Increasing steroids in lieu of taking

hydroxychloroquine increases the likelihood of corticosteroid-related

complications; corticosteroid use should be kept at a minimum.


A 66-year-old woman who has a history of corticosteroid-dependent chronic

obstructive pulmonary disease and nondisplaced talus fracture 1 year ago now has

burning, aching, and numbness of the sole of the right foot and toes. The discomfort is

worse at night and improves with ambulation.Question

Compression of which of the following nerves is the most likely cause of this

patient’s discomfort?

A.Anterior tibial nerve

B.Deep peroneal nerve

C.

Posterior tibial nerveD.

Superficial peroneal nerve



E.

Common peroneal nerve


This patient has tarsal tunnel syndrome marked by posterior tibial nerve

compression. Foot trauma including talus or calcaneal fracture predisposesto the development of this condition. Deep peroneal nerve (or anterior tibial

nerve) compression causes paresthesias and numbness of the dorsum of the

foot. Note that the anterior tibial nerve is now more commonly called thedeep peroneal nerve. Superficial peroneal nerve compression causes

paresthesias and numbness over the dorsolateral foot and worsens with

exercise. Common peroneal nerve compression causes foot drop.


A 23-year-old man sustained multiple toe fractures while playing soccer four

months ago. Since cast removal, he has had pain in the lower extremities

and arthralgias. Past medical history reveals a clavicle fracture at age 3years, finger fractures while playing volleyball at age 9 years, and foot

fractures at age 12 years when he kicked a can. Physical examination showsa thin man who is slightly nervous and sweating. His height is 5 feet 5

inches and has normal dentition and no skeletal deformities. All MCP, ankle,

and elbow joints go beyond the normal range of motion. Findings on

examination of the skin and heart are normal. The sclerae are pale blue,which he says have always been like that, and there is no family history of

this finding. He wears hearing aids.

Laboratory data include:CBC: normal

Alkaline phosphatase: 55 U/LVit D: 23 ng/mL (range: 9.0-37.6 ng/mL)

Testosterone: normal

Question

Which of the following studies is the most appropriate next step?A.

Skin fibroblast culture for collagen 1 alpha 1 mRNA production

B.Bone densitometry

C.

COLA1 gene sequencingD.



Quantitative CT scans

E.

Histomorphometric analysis of boneCorrect Answer

BAnswer Rationale

This is a case of type I ostegenesis imperfecta (OI), a rare inheritedconnective tissue disorder with many phenotypic presentations. The cause of

all cases of OI has not been definitely established. Although mutations in

one or the other of two genes which code for proteins that combine to formtype I collagen (COL1A1 and COL1A2), account for many of the qualitative

and quantitative defects in type I collagen seen in OI, its clinical

manifestations vary substantially within families. One member may be quite

affected clinically, whereas another member with the same mutation mayenjoy normal function. Skin biopsy for analysis if type I collagen genes

and/or testing of genomic DNA for mutations in COL1A1 and COL1A2 may be

a helpful in diagnosis especially in those with subtle clinical manifestations.

However, normal results of these tests do not exclude thediagnosis. Identifying a mutation in a particular gene does not necessarily

result in a clear clinical diagnosis for this reason the clinical diagnosis of OI is based on the signs and symptoms as described in the above case. Mild (type

I) OI, is the least severe form. Individuals with type I OI may have few or

no fractures before puberty or numerous fractures throughout their

lives. Deformity is minimal and stature is usually normal. Individuals withtype I OI usually do not begin to have fractures until they begin toddling or

walking. The most frequently involved bones are the long bones of the arms

and legs, ribs, and the small bones of the hands and feet. Clinicalmanifestations of OI include excess or atypical fractures (brittle bones), short

stature, scoliosis, basilar skull deformities, which may cause nerve

compression or other neurologic symptoms, blue sclerae, hearing loss,opalescent teeth that wear quickly (dentinogenesis imperfecta) increased

laxity of the ligaments and skin with easy bruiseability. Measurement of

BMD by dual-energy x-ray absorptiometry (DEXA) at any age discloses asignificant decrease in bone mass. This is the case for mild disease, in which

BMD is reported to be 76 percent of that of an age-matched control in the

spine and 71 percent of control in the femoral neck. T scores (i.e., standarddeviation from the young-adult mean BMD) are frequently in the range of

-2.5 to -4.0 at the lumbar spine or proximal femur. Although bone biopsy is

indicate when there is excessive skeletal fragility under unusualcircumstances and in this case he had a classic clinical picture of IO with

multiple fractures at young age, blue sclerae, short stature, and hearing loss.


A 23-year-old woman has a 4-year history of polymyositis. At the onset of

disease, she had a rash on her face and CK level was 35,000 U/L. EMG was

consistent with inflammatory myopathy. A biopsy of the deltoid muscle wasconsistent with polymyositis. She was subsequently treated with numerous



drugs including high-dose prednisone, methotrexate, mycophenolate mofetil,

and infliximab. She had an anaphylactic reaction to azathioprine. She has

also received several courses of IVIG. None of these treatments have shownsignificant benefits although the CK levels are now lower, in the range of

8,000-10,000 U/L.

She now has significant difficulties with daily activities, requiring help for dressing and ambulation from her mother who lives with her. At times she

uses a scooter. Social history is negative for use of illicit drugs, tobacco, and

alcohol. Her family history is negative for autoimmune disease; she is anonly child. On examination, she is overweight, has cushingoid features, and

is without rash or nail abnormalities. Muscle strength is 2-3/5 in all four

extremities, worst in proximal muscles of both legs. An MRI scan using a

STIR sequence shows increased signal intensity in muscles of the quadriceps,consistent with the presence of inflammation.


CK: 8,000 U/L (normal range 40-150)

Creatinine: 0.6 mg/dL(0.6-1.5)Blood glucose (fasting): 160 mg/dL (70-110)

Westergren ESR: 40 mm/hr (1-30)Thyroid-stimulating hormone: 2 uU/ml (0.5-5.0)

ANA: negative

Jo-1 antibody: negative

Urinalysis: negative for protein, blood, and leukocytesThe decision is made to reevaluate her disease status and obtain another

muscle biopsy.

Question

Which of the following is the most likely finding on the repeat biopsy?

A.Inclusion body myositis

B.

Dermatomyositis

C.Limb girdle muscular dystrophy

D.

PolymyositisE.

Diabetic vasculopathy




The mean age of onset of limb girdle muscular dystrophy (LGMD) Type 2B, a

dysferlinopathy, is 19 years of age. Proximal lower extremity weakness is a

common initial finding, and unlike some types of juvenile dystrophies,hypertrophy of calf muscles usually does not occur. Truncal weakness can be

marked, and this affects balance and gait making walking difficult. MRI STIR

studies in LGMD can show increased signal intensity that is not readilydistinguished from the pattern seen in inflammatory myopathies. For these

reasons, this disorder can be misdiagnosed as polymyositis. However, the

muscle biopsy shows defective staining for dysferlin, a muscle membrane protein, and this feature distinguishes the histologic changes from those seen

with polymyositis.

Another clue that this case may not be PM is the very high initial CK level,

more than 100 times greater than the upper limit of normal, which isuncommon in the inflammatory myositis syndromes. Chronic juvenile DM is

typically associated with more prominent cutaneous features than those

described here and frequently also has vasculitic changes and prominent soft

tissue calcifications, which are not present in this patient. Furthermore, thisyoung woman is not in the demographic category typical for IBM, which is

generally observed in middle-aged or older men. Diabetes mellitus can alter blood flow in muscles and cause MRI changes, but this is usually associated

with longstanding disease, which this patient does not have.

Question Number: 21

CaseA 46-year-old woman has had a 4-month history of recurring confluent

erythematous macules over the sun-exposed areas of the forearms, anterior

torso, face, and neck that are occasionally associated with superficial

ulceration. She has not had associated mouth ulcers, sicca symptoms,

xerostomia, or joint problems. Evaluation at the time her symptoms beganwas remarkable only for the rash and an otherwise very fair complexion;

serology studies were notable for positive ANA at a titer of 1:640 (speckled

pattern) and anti-SSA/Ro antibodies in a significant elevated titer. She was

initially advised to use a high SPF sunscreen and was prescribedhydroxychloroquine 400 mg daily and prednisone 40 mg daily. The rash

promptly responded to corticosteroids but has flared each time the prednisone is tapered below 20 mg/day. Other than persistent leucopenia

(WBC 2,200/mm3), other blood counts, chemistry profile, and urinalysis

have been repeatedly normal.Question

Which of the following is the most appropriate recommendation at this time?



A.

Add pulse intravenous cyclophosphamide 750 mg/m2

B.Add azathioprine 2 mg/kg/day

C.

Add dapsone 50 mg/dayD.

Add quinacrine 100 mg/day

E.Add thalidomide 50 mg/day

Correct Answer

DAnswer Rationale

Cutaneous manifestations of lupus are quite variable, manifesting aserythematous macular or maculopapular rashes, discoid plaques, bullous

lesions, or vasculitis manifest as nonblanching purpura, cutaneous ulcers, or

urticarial lesions. While antimalarials are effective for suppressing many of

the cutaneous manifestations of lupus and corticosteroids in sufficient dosewill usually effect resolution of cutaneous flares, other immunomodulating

therapies are often required to suppress cutaneous manifestations of lupus toavoid the toxicity associated with long-term corticosteroid use.

Intravenous pulse cyclophosphamide is effective in managing severe

manifestations of SLE but is typically reserved for severe lupus nephritis,

severe CNS manifestations, or refractory severe immune-mediatedcytopenias, with other less toxic options employed to manage active skin

disease. Azathioprine is often effective as a corticosteroid-sparing therapy for

a variety of lupus manifestations, including skin disease. However,concurrent leucopenia as is present in this patient may preclude use of

azathioprine in doses sufficient to effectively manage skin disease. In the

setting of leucopenia, mycophenolate mofetil might also be considered as an

alternative to azathioprine as a corticosteroid-sparing therapy because it impacts predominantly lymphocyte proliferation with minimal impact on granulopoiesis, but the

efficacy of mycophenolate in managing cutaneous manifestations of lupus is not well

established.Other alternatives that do not impact granulopoiesis, such as dapsone,

addition of a second antimalarial, or thalidomide, are often quite effective inmanaging cutaneous manifestations of lupus. Dapsone has been shown to be particularly useful in managing cutaneous ulcers in SLE caused by small

vessel vasculitis or bullous disease. Persistent mauclopapular eruptions that

are not adequately suppressed with hydroxychloroquine often will respond toaddition of a second antimalarial such as quinacrine. If this option is used,

combined use of these two antimalarials is more effective than either alone

and is the concurrent use of both is recommended rather than discontinuing



hydroxychloroquine and adding quinacrine. Quinacrine is usually well

tolerated although patients with a fair complexion may find it objectionable

because of predictable yellow discoloration of the skin with prolonged use.Thalidomide is highly effective in managing mucocutaneous ulcers, as well as

refractory erythematous rashes, including the eruption associated with

subacute cutaneous lupus, a variant associated with elevated titers of anti-SSA/Ro antibodies.

Question Number: 22

CaseAn 18-year-old woman has 1-year history of progressive proximal muscle

weakness. Four months ago, her CK level was 12,000 U/L, a short tau

inversion recovery (STIR) MRI showed edema in her proximal thighs, and a

muscle biopsy revealed fiber degeneration and regeneration with infiltrationof some lymphocytes and phagocytic cells. She was treated with high-dose

corticosteroids, methotrexate, and monthly infusions of intravenous

immunoglobulins for 3 months. Despite this treatment, muscle strength has

continued to deteriorate and CK level is 16,000 U/L.Her family history reveals that similar symptoms developed 3 years ago in

her 26-year-old brother.Question

Testing of a repeat muscle biopsy specimen is most likely to show which of

the following?

A.Histochemistry revealing fiber type grouping

B.

Immunohistochemistry showing absence of dysferlinC.

Trichrome staining revealing ragged red fibers

D.

Absence of carnitine palmitoyltransferase (CPT) activity


The idiopathic inflammatory myopathies can be confused with other

myopathies that may have an inflammatory component, such as muscular

dystrophies. Dysferlin deficiency causes limb-girdle muscular dystrophy. This condition is familial with the usual onset between 18 and 20

years of age. CK levels may be 100 times the upper limit of normal. Muscle

histology typically shows inflammation. Corticosteroid therapy is not helpfuland, in some cases, may make the condition worse.

Fiber type grouping is characterisc of neuropathic processes that involve

reinervation.Ragged red fibers are seen by Trichome staining of muscle in patients with

some mitochondrial myopathies. Mitochondrial myopathies may be familial,

can present with proximal weakness and elevated CK levels, and will not



respond to immunosuppressive therapy. Muscle tissue would not show

regeneration or inflammatory cell infiltrate.

This clinical course is not consistent with CPT deficiency. CPT deficiency causesepisodic rhabdomyolysis. Between episodes, strength and muscle enzymes are normal.

Question Number: 23

CaseA physician colleague asks for evaluation because he thinks he may have

rheumatoid arthritis. He is 55 years old and enjoys excellent health; he is an

avid bicyclist. Nine months ago, mild pain and swelling developed in the MCP joint of his right thumb. Two months ago, similar symptoms developed in the

PIP joint of the left ring finger. Both sets of symptoms developed without

antecedent trauma. There is no morning stiffness. On physical examination,

there is mild soft tissue swelling with minimal tenderness of the twosymptomatic joints. No other joint abnormalities are present.


ESR: 1 mm/hr

C-reactive protein: <0.7 mg/dLRheumatoid factor: 256 IU/ml

CCP antibody level: >100

Question

According to the available evidence from clinical trial data, which of thefollowing management approaches is most likely to retard progression of this patient’s arthritis?

A.

Prednisone 10 mg po dailyB.

Naproxen 500 mg po twice daily

C.Sulfasalazine 1000 mg po bid

D.

Etanercept 25 mg subcutaneously twice weekly

E.Methotrexate 15 mg po once weekly

Correct Answer

EAnswer RationaleThis patient does not meet the ACR criteria for rheumatoid arthritis and

would thus be classified as having an undifferentiated arthritis. In contrast to

early rheumatoid arthritis, there are few clinical trials that have examinedtherapy for patients with undifferentiated arthritis. The best such study was



the PROMPT trial in which 110 patients with undifferentiated arthritis (defined

by the 1958 ACR criteria for probable RA) were randomized to receive

methotrexate 15 mg weekly or placebo. The dose of methotrexate wasincreased every 3 months if the Disease Activity Score (DAS) was >2.4. The

primary end-point was the fulfillment of the ACR criteria for RA.

Undifferentiated arthritis progressed to RA in 22 of the 55 methotrexate-treated patients and in 29 of the 55 placebo-treated patients (p=NS). All of

the placebo-treated patients who eventually fulfilled ACR criteria did so within

one year, in contrast to only one-half of the methotrexate-treated patients(p=0.04). At the end of 18 months, fewer methotrexate-treated patients

showed radiographic progression compared to the placebo-treated patients

(p=0.046). Methotrexate was effective in reducing the signs and symptoms

of undifferentiated arthritis.Question Number: 24

Case

A 52-year-old woman is referred for further evaluation of progressive fatigue,

dyspnea, and stiffness in her hands. She began to have Raynaud’s symptoms3 years ago and for the past year has had mild nonproductive cough with

increasing exertional dyspnea. For the past 6 months, her fingers have become progressively stiff with impaired grip function. She has not had any

fever, rash, mouth ulcers, dysphagia, or changes in bowel function. Physical

examination shows blood pressure 120/86 mmHg, heart rate 100 bpm, andnormal respirations and temperature. Sclerodactyly without terminal digital

ulcers is present in the upper extremities with mild induration present on the

forearms and face. Ocular and oral membranes are notable for pallor but areotherwise normal without evidence of ulceration or xerostomia. Bibasilar

crackles are present on chest auscultation but there is no audible pleural or

pericardial rub and the pulmonic second heart sound is not accentuated.Other than limitation in extension of the wrists and IP joints of her hands,

findings on joint examination are normal. Muscle strength testing is normal.


WBC: 6200/mm3Hct: 24% (MCV 76)

Hgb: 7.4 mg/dl

Platelet count: 720,000/mm3Creatinine: 1.6 mg/dl

CK: 380 IU/ml (nl<190)

AST: normalALT: normal





for alveolar volume on pulmonary function testing or abnormalities on

echocardiography suggestive of pulmonary hypertension such as RV dilation

or elevated estimated PA systolic pressure, right heart catheterization wouldnot be indicated.

Elevations in creatine kinase (CK) levels may occur in patients with systemic

sclerosis, either because of concomitant hypothyroidism or inflammatorymyopathy. If there is no clinical weakness in association with moderate mild

to moderate CK elevations, it is not necessary to obtain a muscle biopsy. If

there is demonstrable weakness in a pattern consistent with inflammatorymyopathy and EMG findings are consistent with a myopathy (as opposed to a

primary neuropathic process), it may still be appropriate to proceed with

treatment for inflammatory myopathy without obtaining a confirmatory

muscle biopsy in patients with established systemic sclerosis. Treatmentoptions other than corticosteroids, such as weekly methotrexate,

azathioprine, or IVIG, should be considered due to the increased risk of renal

crisis associated with corticosteroids use in patients with systemic sclerosis.

Question Number: 25

CaseA 32-year-old woman has a 2-month history of lower extremity violaceous

macules, generalized arthralgias, and asthenia.

Laboratory data include:HCV RNA: positive

C4: 10 mg/dl

Cryoglobulins: positiveUrinalysis: normal

Rheumatoid factor: 180 IU/ml

Skin biopsy of the lower extremity rash reveals leukocytoclastic vasculitis.

QuestionIn addition to prednisone, which of the following is the most appropriate

treatment?

A.

Peg-Interferon – RibavirinB.

PlasmapheresisC.

Cyclophosphamide

D.Rituximab

E.



Infliximab

Correct Answer

AAnswer RationaleMixed cryoglobulinemia is a systemic vasculitis associated with hepatitis C

infection. The mixed cryoglobulinemia syndrome is associated with purpura,

arthralgia, and asthenia. There may also be neurologic and renalinvolvement. Treatment of hepatitis C mixed cryoglobulinemia (HCV-MC)

may target the viral load or the B-cell arm of autoimmunity. Peg-

Interferon─Ribavirin is the first line therapy. For refractory HCV-associated cryoglobulinemia vasculitis or acute severe life-threatening organ

involvement, plasmapheresis, corticosteroids in combination with cytotoxic

agent, or Rituximab should be considered as treatment options.

Question Number: 26

Case

A 56-year-old woman has a 4-month progression of 2nd and 4th bilateral DIPdeformity. Pruritic nodules on the left 4th DIP joint and right pinna also

developed. She has a 2-month history of cough, dyspnea, weight loss, and

malaise.Bilateral radiographs of the hands reveal extensive erosive changes in the

bilateral 2nd and 4th DIP joints.

Radiograph of the chest reveal hilar lymph nodes.CT Scan of the chest reveal nodular pleural thickening, pleural effusions, andmediastinal and hilar lymphadenopathy

Biopsy of the 4th DIP nodule reveals villous, hypertrophic synovitis with

plasma cells and multinucleated giant cellsQuestion

Which of the following is the most likely diagnosis?

A.Rheumatoid arthritis

B.

Relapsing polychondritis

C.Seronegative spondyloarthropathy with peripheral joint involvement

D.

Multicentric reticulohistiocytosisE.

Psoriatic arthritis




Multicentric reticulohistiocytosis is characterized by severe destructive

arthritis and cutaneous papulonodular lesions. Histiologic analysis of the

cutaneous nodules reveals infiltration of multinucleated giant cells andhistiocytes of monocyte/macrophage origin. An associated malignancy has

been identified in 15-28% of patients, particularly those with mesothelioma,

melanoma, lymphoma, sarcoma, ovarian cancer, and leukemia. Other associations include hyperlipidemia, pregnancy, TB, and autoimmune

disease. This patient has an associated mesothelioma. Although the other

provided diagnoses could be considered in the differential diagnosis, the biopsy supports the diagnosis of multicentric reticulohistiocytosis

Question Number: 27

CaseA 67-year-old woman has new-onset shoulder pain and stiffness. The

symptoms began insidiously 2 months ago. She describes pain and stiffness

around her shoulders and thighs, especially worse in the morning on arising.She now has pain in these areas during the night which awakens her

routinely. Ibuprofen 400 mg qid provides only short-term relief. Four weeks

ago, she was examined by her internist who noted “normal findings.”Laboratory data at that time included:

CBC: Normal

ESR: 29 mm/hr CR-P: 0.9 URheumatoid factor: negative

ANA: 1: 40

AST: NormalALT: Normal

CK: Normal

Creatinine: NormalAlbumin: Normal

TSH: 2.1 U

Urinalysis: Normal

Past medical history is notable for hypertension for which she takes atenolol50 mg daily.

On physical examination now, pain restricts maximal abduction and rotation of the

shoulders. Cervical spine range of motion is complete. No synovitis is detected. Motor strength is normal in all extremities. There is no muscle tenderness. There is no cranial

tenderness and temporal arteries are easily palpated with normal pulsations.

QuestionWhich of the following is the most appropriate next step in management?



A.

Begin prednisone 15 mg/day

B.Begin amitriptyline 10 mg at bedtime

C.

Inject shoulders with intra-articular corticosteroidsD.

Discontinue atenolol

E.Begin pregabalin 75 mg/day

Correct Answer

AAnswer Rationale

The clinical presentation of this patient is most consistent with polymyalgia

rheumatica (PMR). The onset of bilateral, symmetric, shoulder and pelvic

girdle stiffness and nocturnal symptoms in an older patient, with the absenceof peripheral joint swelling, is most consistent with this diagnosis. Though

markers of inflammation such as ESR and C-RP are generally elevated, theycan be normal in about 20 percent of patients. These patients with PMR andnormal makers of inflammation have a similar clinical course to patients with

elevated markers. Their response to low to moderate doses of corticosteroids

is generally dramatic.

MRI and ultrasound studies showed that patients who have PMR with normalor high ESRs have similar inflammatory shoulder lesions. These imaging

studies may facilitate the proper diagnosis in patients with the typical

proximal symptoms of PMR who also have normal ESRs.Intra-articular corticosteroids may provide short-term pain relief, but would

not be recommended given the strong likelihood of symptom relapse.

Increasing the dose of ibuprofen would likely provide little additional benefitand there is no evidence that NSAIDs are effective in managing patients with

PMR.

There are no clinical features to suggest fibromyalgia or another soft tissue pain

syndrome, thus prescribing amitriptyline or pregabalin is not indicated. Beta-blockerssuch as atenolol do not cause these pain syndromes and so this medication should not be

discontinued.


A 73-year-old woman who has had deforming rheumatoid arthritis for the

past 20 years now has persistent neutropenia. She has been living in anassisted care facility for the past 3 years. Despite bilateral knee

arthroplasties, her mobility and ability to live independently have been

progressively impaired by arthritis of her ankles, feet, wrists, and hands. Pasttherapies have included parenteral gold, penicillamine, hydroxychloroquine,

methotrexate, and etanercept. At the time of admission to the facility, her

WBC was 3200/mm3 and her physician chose to maintain her on prednisone

7.5 mg qAM alone for her rheumatoid arthritis. Her only infection was aurinary tract infection 3 months ago treated with oral ciprofloxacin. Other



than the 10-day course of ciprofloxacin, she has not been on any new

medications for the past 12 months.

WBC: 1800/mm3 (25% neutrophils, 65% lymphocytes)Hgb: 10.3 gm/dl

Peripheral smear: paucity of neutrophils, occasional large lymphocyte with

pale blue cytoplasm and azurophilic granules

Flow cytometry of peripheral blood demonstrates a clonal population of

CD3+, CD8+, CD16+, CD57+ T cellsQuestion

Which of the following is the most appropriate initial therapy for this patient?A.

SplenectomyB.

Increased dose of prednisone

C.Infliximab

D.

MethotrexateE.

Filgastrim

Correct Answer DAnswer RationaleThis patient has large granular lymphocyte (LGL) syndrome resulting in an

autoimmune neutropenia. The LGL syndrome is a clonal disorder of cytotoxic

T-lymphocytes and has been classified as leukemia, even though it has anindolent clinical course. About 25% of patients with LGL syndrome have

rheumatoid arthritis. The demonstration of a clonal population of CD3+,

CD8+, CD16+, CD57+ T cells with flow cytometry of the peripheral bloodserved to establish the diagnosis of LGL syndrome.

Methotrexate is considered first line therapy for patients with RA and LGL

syndrome. Cyclosporine A may also be of benefit in patients with LGL

syndrome. There are no data to indicate that glucocorticoids have lasting

benefit in the therapy for these diseases. While splenectomy may result in an

immediate improvement in the absolute neutrophil count, the clinical

response is usually not lasting. Given the morbidity of a splenectomy, it is

usually reserved for patients with active infections that are resistant to

treatment or patients with chronic refractory leg ulcers. Myeloid colony-



stimulating factors, such as filgastrim, may be used in severe neutropenia

while a remission is being induced with immunosuppressive agents.Correct AnswerDAnswer Rationale

Intercurrent pregnancy in the context of active SLE poses significant

diagnostic and therapeutic challenges. Patients with a history of lupus

nephritis who become pregnant are at increased risk for hypertension and

the development of pre-eclampsia. The differentiation of pre-eclampsia from

lupus flare is often difficult diagnostically when edema, hypertension, and/or

azotemia develop during the late second or third trimester of pregnancy, but

the presence of active urine sediment and edema in the very early stages of

the pregnancy are more likely attributable to active lupus than to pre-

eclampsia.

The presence of active nephritis requires continued aggressive treatment

through the course of pregnancy, but the choice of needed

immunosuppressive therapy is limited to agents not known to cause

deleterious effects on the fetus. ACE inhibitors such as lisinopril are

teratogenic and must be discontinued in the context of pregnancy; labetalol

is an effective and safe alternative that can be supplemented with

vasodilators (such as hydralazine) as needed to manage blood pressure.

Mycophenolate has also been shown to be teratogenic and should be

discontinued at the onset of pregnancy. Corticosteroids may be continued

through the pregnancy in doses required to suppress disease activity, but

continued high doses may exacerbate edema and hypertension as well as

promote the development of gestational diabetes. Azathioprine has been

used through the course of pregnancy and has not been associated with fetal

malformation or birth defects. As such, corticosteroid-sparing therapy with

azathioprine to continue aggressive treatment of active SLE nephritis or

immune cytopenias is reasonable and may be necessary to manage SLE and

pregnancy. Antimalarials have not been shown to induce fetal abnormalities

and current recommendations are to continue antimalarial treatment in

patients with active SLE who become pregnant.Question Number: 35Case

A 35-year-old woman has had pain and swelling in her right foot for the past

3 weeks. Two months ago, she tore the medial meniscus and anterior

cruciate ligament in her right knee while skiing. These were repaired with

arthroscopic surgery. She was hospitalized on the third postoperative day for





scan and venous Doppler studies would not be indicated in this clinicalsetting.Question Number: 36Case

A 22-year-old college student has left knee swelling after playing soccer. He

does not recall specific trauma to the affected joint. He denies sexual

activity. Review of systems is notable for the lack of antecedent infectious

symptoms, visual difficulties, oral ulcers, back pain, diarrhea, or rash. His

home is in Connecticut, and deer frequent his back yard. On physical

examination, the left knee is large and swollen.Analysis of aspirated joint fluid reveals WBC 11,000/mm3 with predominant

neutrophils, no crystals, and negative bacterial cultures. Lyme titer is

markedly positive with a confirmatory Western blot test showing 10 positive

bands.

He is treated with doxycycline for 30 days. The follow-up examination shows

a persistent effusion and no abnormal neurological manifestations. The oral

antibiotic is continued for another 30 days. Intravenous ceftriaxone is

prescribed for 1 month after the extended course of oral doxycycline fails to

eradicate the effusion. Synovial fluid aspiration now shows persistent

inflammatory joint fluid with negative PCR for Borrelia burgdorferi.QuestionWhich of the following is most appropriate therapy at this time?A.

EtanerceptB.

Oral doxycycline for 30 daysC.

IV ceftriaxone for 30 daysD.

IV penicillin for 30 daysE.

Hydroxychloroquine

Correct AnswerEAnswer RationaleLyme arthritis is an inflammatory arthritis that primarily affects large joints,especially the knee, and is caused by infection with Borrelia burgdorferi,

which is a spirochete transmitted by Ixodid ticks. In most patients,

treatment with oral doxycycline for 30 to 60 days eradicates infection and the

effusion. In a small percentage of patients, a persistent effusion occurs

despite months of oral and intravenous antibiotic therapy. In these patients,

the appropriate work-up should include PCR testing of the synovial fluid for

Borrelia burgdorferi. If the PCR is positive, repeated courses of antibiotic



therapy should be considered. If the PCR is negative, antibiotics are not

recommended, and treatment with NSAIDs or hydroxychloroquine is

recommended. PCR testing is technically challenging and should thus be

performed in experienced laboratories.


A 65-year-old woman has swelling in the right elbow. Four months ago, she

accidentally bumped it against a wall. She noted immediate pain that

resolved within few days but not the swelling. She has no pain, morning

stiffness, or limited function. She has a past medical history of hypertension

with stage II chronic kidney disease (CKD), takes Lisinopril 10 mg daily, is a

nonsmoker and nondrinker, and has not traveled outside the USA. The

patient is alert, oriented, and in no distress. Physical examination shows

normal vital signs. With the exception of a nontender firm nodular mass on

the right olecranon area, findings on the rest of the physical examination are

normal.Laboratory data include:

Creatinine: 2.0 mg/dl (creatinine a year ago was 1.6 mg/dl)

CBC: Hgb 11 gm/dl; Hct 34% with normal indices

Calcium: 11 mg/dl

Phosphate: 3.5 mg/dl

Parathyroid hormone (PTH): 65 pg/ml

PTH rp: 1.1 pmol/L (normal < 1.5 pmol/L)

25 OH Vit D: 50 nmol/L

1,25 OH Vit D: 65 micromol/L

Angiotensin-converting enzyme: 80 U/LUrinalysis: normal

24-hr urinary calcium: 350 mg

Chest (Image 1) and elbow (Image 2) radiographs are shown.





FigureFigure 1Figure 2QuestionWhich of the following is the most likely cause of these findings in thispatient?A.

Hyperparathyroidism secondary to CKDB.

Ectopic production of PTHC.

Primary hyperparathyroidismD.

Increased production of 1,25 Vit DE.

Myositis ossificans

Correct AnswerDAnswer Rationale



Mild to severe hypercalcemia and/or hypercalciuria occurs in 10% of patients

with sarcoidosis. The cause of this disordered regulation of calcium is due to

endogenous production of active metabolite of Vitamin D by macrophages in

the sarcoid granuloma. This extra renal synthesis of 1,25 OH Vit D is not

subject to the normal physiologic regulatory influences. This patient has CKD

Date post:	09-Apr-2018
Category:	Documents
Upload:	vijay-singh
View:	240 times
Download:	0 times

Rheumatology Conference

Documents