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Question Number: 38
CaseA 52-year-old woman has a history of progressive ulnar deviation and PIPenlargement. She also has lumbosacral spine and hip, knee, and shoulder
joint involvement. Two years ago, she required right knee
replacement. Eight years ago, she had nephrolithiasis with several recurrentepisodes. The kidney stones have not been analyzed. She notes that there
are dark stains on her undergarments. She has a long history ofrosacea. One week ago, she began treatment for rosacea with minocycline.
Physical examination shows bilateral bluish-gray sclerae, mild ulnar deviationof the fingers, bilateral 2nd, 3rd, and 4th PIP enlargement, and a modifiedSchober test of 10 cm to 13 cm. Examination of the face shows erythema,
papules, and dilated blood vessels.
Radiographs of the lumbar sacral spine reveal loss of disc height, disc
calcification, very thick syndesmophytes, and bamboo spine (see figurebelow).
Figure
QuestionWhich of the following is the most likely diagnosis?
A. Rheumatoid arthritis
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B. Minocycline-induced hyperpigmentation
C. Alkaptonuria
D. Hyperuricemia and gout
E. Ankylosing Spondylitis
Correct Answer
C
Answer Rationale
Alkaptonuria is an autosomal recessive inborn error of metabolism. The
enzyme homogentisic acid oxidase is deficient resulting in large volume
excretion of
homogentisic acid (HGA) in the urine and sweat. Urine that contains HGA
will turn dark as the acid is oxidized to melanin-like product. This may result
in dark stains on clothing and undergarments. HGA may also accumulate oncartilage, tendons, sclerae, skin, and intima of large vessels. Nephrolithiasis
is a manifestation of the disease. Alkaptonuria resembles ankylosing
spondylitis involving the spine and large joints, however, it differs in that the
sacroiliac joints (SI joints) are spared. Minocycline has been associated with
hyperpigmentation resembling alkaptonuria, however, urine has normal HGA
measurement.
Question Number: 39
Case
A 56-year-old man has a maculopapular rash over the lower extremities thatcomes and goes. He notes some edema when he has the rash. He hasfatigue and some joint pain but no paresthesias. He has been losing weight.Review of other systems is unremarkable. He takes no medications.
Examination shows a normotensive man with palpable purpura and noabnormal neurological or musculoskeletal features. Findings on the
remainder of the general examination are normal.
Laboratory data include:
CBC: Normochromic anemia
BUN: 32 mg/dlCreatinine: 1.9 mg/dlAST: 86 U/L
ALT: 94 U/LAlkaline phosphatase: 110 U/L
Total bilirubin: 0.8 mg/dl
ESR: 98 mm/hrANA: 1:40 speckled
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dsDNA: negativeANCA: negative
RF: 450Anti-CCP antibody: negativeTotal protein: 8.8 gm/dlUrinalysis: 1+ protein, 2+ blood, no casts
Findings on chest radiograph are normal.
QuestionBased on the information above, which of the following is the most likelycause of the patients elevated rheumatoid factor?
A. Rheumatoid arthritis
B. Sjgrens syndrome
C. Lupus
D. Hepatitis-associated cryoglobulinemia
E. Wegeners granulomatosis
Correct Answer
D
Answer Rationale
The patient is a middle-aged man with palpable purpura, which is highly
suggestive of vasculitis. There is little in the history to suggest infection as atrigger, and there are no symptoms referable to the upper respiratory tract,
which are present in about 80% of patients with Wegeners
granulomatosis. He is not taking any medications that might trigger a
hypersensitivity vasculitis. The examination is unrevealing other than
showing purpura, and the laboratory studies show renal insufficiency, an
active urinary sediment, hepatitis, a low titer ANA with negative anti-dsDNA
antibodies, and markedly elevated rheumatoid factor. The anti-CCP antibody
is negative, arguing against RA, especially in light of the absence of joint
inflammation.
Causes of very high levels of rheumatoid factor include rheumatoid arthritis,
Sjgrens syndrome, and cryoglobulinemia. Cryoglobulinemia can be caused
by autoimmune diseases, chronic infections, and lymphoproliferative
disorders. Type I cryoglobulins are comprised of monoclonal
immunoglobulins, and are associated with lymphoproliferative disorders.
Type II cryoglobulins contain a monoclonal and polyclonal component, and
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can be seen with autoimmune diseases and infectious disease like hepatitis
C. Hepatitis C infection, as well as other infectious diseases, produce mixed
cryoglobulins, composed entirely of polyclonal immunoglobulins.
Rheumatoid arthritis is not likely, given the absence of joint findings and
negative anti-CCP antibody. Sjgrens syndrome is in the differential
diagnosis, but there is little in the clinical history to suggest its presence.
Results of SSA and SSB antibodies are not given. Wegeners granulomatosis
is not likely given the absence of symptoms referable to the upper
respiratory tract and the negative ANCA, which are seen in the majority of
patients with Wegeners granulomatosis. Lupus is also a possibility but the
patient does not appear to fulfill sufficient criteria to support the diagnosis.
Question Number: 40
CaseA 30-year-old man has gradually increasing right knee pain for the past 21
days. He is a long-distance runner who averages 30 miles a week. He saysthe pain began after a speed work out. The pain is on the lateral aspect ofthe knee. It resolves with rest to some degree, but any running causes it toreturn and causes him to limp for a day or 2. He can also bring on pain by
standing on his right leg and flexing his knee. The knee does not lock.
Physical examination shows tenderness over the lateral knee and lateral tibialcondyle. There is a creaking over the tender area with knee flexion and
extension. The knee is stable and there is no effusion.
QuestionWhich of the following is the best course of action at this time?
A. Prescribe NSAID and avoid running
B. Inject the right knee with a depot-corticosteroid preparation
C. Inject the bursa with anserine
D. Apply an elastic bandage (ACE wrap) and have the patient use crutches for 4-6 weeks
Correct Answer
A
Answer Rationale
Iliotibial band syndrome is a common injury in long-distance runners that
usually develops with over-training and after vigorous running or hiking. The
treatment involves avoiding running and NSAID therapy. In severe cases,
physical therapy and local corticosteroid injection may be helpful.
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There is no role for intra-articular injection or avoiding weight bearing in this
condition.
An MRI would be done if one expects a meniscal tear or ligament
injury. These conditions are unlikely because the joint is stable and there is
no effusion, locking, or joint-line tenderness.
A bone scan would be indicated if one suspects a stress fracture.
Surgery is not indicated for this condition unless it is chronic and refractory
to all other therapies.
Question Number: 41
CaseA 6-year-old girl with polyarticular juvenile idiopathic arthritis has a negativerheumatoid factor and positive ANA.
Question
How often should she undergo slit-lamp examination to screen for chroniciritis?
A. Every three to four months for four years then every six months
B. Every six months for four years then every year
C. Every year for four years then every two years
D. When her eyes hurt
E. When she is moved to the front of the class because of difficulty seeing the blackboard
Correct Answer
A
Answer Rationale
Iritis in children is usually chronic and asymptomatic until extensive scarring
has occurred. Those at highest risk of developing iritis are children with
young onset oligoarticular disease and who are ANA positive. However,
polyarticular ANA positive disease also carries a significant risk of iritis,
although lower than the oligaorticular group, and should be screened at the
same intervals.
Question Number: 42
CaseA 38-year-old woman is undergoing evaluation of new-onset polyarthritis
involving the small joints of her hands. She also has had a recurrent
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urticarial eruption over her shins (see figure below) for the past 3-4days. Both symptoms developed over the past 6 months.
Review of systems is notable for the recent need to switch from contactlenses to traditional lenses because of persistent corneal irritation. Althoughher previous dental health was excellent, she has recently developed three
new caries. She takes no medications.
Physical examination shows mildly injected conjunctivae. Having just taken adrink of water, her oral mucosa is moist, but there is no salivary pool. There
are 3 recently restored dental caries, two at the gingival margin.There is nopalpable lymphadenopathy. Findings on pulmonary, cardiac, and abdominalexaminations are normal. Peripheral articular examination shows symmetricsynovitis affecting the wrists and MCP and PIP joints.
Laboratory data include:
C3: 123 mg/dlC4: 32 mg/dl
Figure
Question
Which of the following additional findings is most likely in this patient?
A. Hilar adenopathy
B. Cryoglobulins
C. Anti-SSA/Ro antibody
D. Anti-thyroglobulin antibody
E. Anti-neutrophil cytoplasmic antibody
Correct Answer
C
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Answer Rationale
The patient has secondary Sjgren syndrome in the setting of what is most
likely rheumatoid arthritis, given the symmetrical involvement of the small
joints of her hands. Sjgren syndrome may have a variety of cutaneous
manifestations, including urticarial eruptions. Sjgren syndrome patients with
anti-SSA/Ro antibody are more likely to have such extraglandular
manifestations of their disease. Dental caries at the gingival margin, as seen
in this patient, are characteristic in patients with oral sicca symptoms,
autoimmune or otherwise.
Hilar adenopathy is seen in the Lfgren syndrome, a female-predominant
presentation of sarcoidosis that is also marked by inflammatory polyarthritis
and erythema nodosum. Sarcoidosis may also present with sicca symptoms.
The knees and ankles are the most commonly affected joints. However,
erythema nodosum is characterized by erythematous, tender but
nonpruritic - subcutaneous nodules over the shins. Cryoglobulins may be
seen in rheumatoid vasculitis, but the cutaneous eruption is typically more
acral in distribution and is not typically pruritic. Anti-thyroglobulin antibody is
associated with autoimmune thyroid disease, but not rheumatoid arthritis or
Sjgren syndrome. Anti-neutrophil cytoplasmic antibodies are associated
with necrotizing vasculitis, including Wegener granulomatosis and Churg-
Strauss syndrome, but not rheumatoid arthritis or Sjgren syndrome.
Question Number: 43
CaseA 38-year-old man has polyarticular arthritis. Despite his relative youth and
absence of any history of joint trauma, he has widespread arthritis affectinghis axial skeleton, shoulders, and hips. He reports no visible articular swellingor erythema or palpable heat. He has otherwise been in excellent health andhas no family history of early-onset arthritis, either inflammatory or
mechanical.
Physical examination shows blue-black pigmentation of both sclerae (seeFigure 1).
Radiographs of his spine (see Figure 2) reveal calcification of the
intervertebral discs and disc space narrowing.
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Figure
Figure 1 Figure 2
QuestionWhich of the following tests is most likely to confirm the diagnosis?
A. COMP haplotype analysis
B. FRZB 2-marker single-nucleotide polymorphism (SNP) haplotype analysis
C. Targeted type II collagen polymorphism analysis
D. Plasma homogentisic acid assay
Correct Answer
D
Answer Rationale
The patient has alkaptonuria, an inborn error of metabolism resulting in
inability to metabolize homogentisic acid. Homogentisic acid is deposited in
the soft tissues and cartilage causing ochronosis, darkening of the sclerae,
pinnae, and other soft tissues, as well as premature osteoarthritis. The urine
will also darken if exposed to air.
The COMP haplotype, FRZB 2-marker SNP haplotype, and type II collagen
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polymorphisms have all been associated with premature osteoarthritis, an
effect that varies with the gender and ethnicity of the patient and the specific
joint studied, but will not cause the pigmentary changes seen in ochronosis.
Question Number: 44
CaseA 55-year-old woman has noted a sensation of grit in her eyes for the past 2years. She has been using artificial tears 2 or 3 times a day, particularly
while watching TV or working at a computer. Within the past year, she hasalso noted dryness of her mouth. She keeps a bottle of water at her bedside
and must drink water frequently to aid in swallowing dry food. She has notnoted any recent increase in dental caries. She does not have a rash, jointpain, or fatigue. She does not have clinical evidence of systemic lupuserythematosus, rheumatoid arthritis, or scleroderma.
Laboratory data include:
WBC: 5600/mm3
Hgb: 13.2 gm/dlPlatelet count: 253,000/ mm3ANA testing (multiplex assay): negative antinuclear antibodies, negative SS-A and SS-B antibodies
QuestionWhich of the following additional findings is an absolute requirement forestablishing the diagnosis of primary Sjgrens syndrome in this patient?
A. Schirmers I test with wetting of 7 mm in the right eye and 4 mm in the left eye.
B. Unstimulated salivary flow of 0.1 ml/min
C. Labial salivary gland focus score of 1
D. Absent salivary pool
E. Parotid sialography showing extensive sialectasis
Correct Answer
C
Answer Rationale
Although there have been several sets of diagnostic criteria for Sjgrens
syndrome over the past 40 years, the set that is currently accepted was
derived by an international consensus group in 2002. According to the 2002
International Consensus Criteria for Sjgrens Syndrome, the diagnosis of
primary Sjgrens syndrome requires sicca signs/symptoms in the presence
of either Sjgrens antibodies (SS-A and/or SS-B) or a positive labial gland
biopsy, defined by the presence of a focus score of 1 or more. With the
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exception of the absent salivary pool, the other tests (abnormal Schirmers,
unstimulated salivary flow, or parotid sialography) would provide objective
evidence of the ocular or oral component of the disease and at least one
would be required to establish a diagnosis of Sjgrens syndrome in concert
with the abnormal labial gland biopsy.
Question Number: 45
CaseA 34-year-old woman with a 1-year history of scleroderma is seen in yourpractice as a new patient after moving from another city. She has had
Raynauds phenomenon in her hands as well as skin tightening on the handsand face. She is currently taking lisinopril, 20 mg/day and ASA 325
mg/day. She is a nonsmoker and has no family history of autoimmunedisease. Recently, she has developed a persistent dry cough and she
becomes short of breath after walking one block.
Blood pressure is 135/70 mmHg. She has tightening of the skin on the faceand fingers with digital pitting but no cyanosis. Velcro crackles are present
at both lung bases and she has borderline tachycardia at 105 bpm. Lowerextremities are without edema. Findings on chest radiograph are
normal. Echocardiogram shows a normal ejection fraction and an estimatedPA pressure of 25 mmHg. She is referred to a pulmonary specialist who does
bronchoaveolar lavage, and the fluid from this procedure shows 4%neutrophils. Cultures and stains on the fluid are negative for infectiousagents.
Question
Which of the following medications should be initiated at this time?
A. Prednisone 60 mg/day
B. Cyclophosphamide 1-2 mg/kg po daily
C. Mycophenolate Mofetil 1000 mg twice daily
D. Rituximab 750 mg/m2 x 2 doses
E. Infliximab 3 mg/kg
Correct Answer
B
Answer Rationale
The use of cyclophosphamide in scleroderma lung disease is based on results
from the Scleroderma Lung Study that compared in a blinded fashion oral
cyclophosphamide to placebo. Data from this 1-year study showed a
statistically significant improvement in lung function parameters in patients
treated with cyclophosphamide. Improvements were also observed in
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dyspnea and in quality of life indices, and these improvements persisted in
the year following cessation of cyclophosphamide treatment. The patient in
this case has neutrophilia on BAL consistent with the presence of
alveolitis. Prednisone in high doses is relatively contraindicated in
scleroderma because of the risk of precipitating renal crisis. The use of
mycophenolate mofetil has been assessed only in limited retrospective
studies and data regarding utility of rituximab and infliximab are largely
limited to case reports. There have been some reports suggesting that TNF
blockade might induce a scleroderma-like syndrome.
Question Number: 47
Case
A 30-year-old man was given the diagnosis with of rheumatoid arthritis 2years ago after he was evaluated for intermittent episodes of migratory
painful polyarthritis involving the knees, ankles, and elbows. The initial
episode lasted two weeks and on recurrence, synovitis of both elbows andtenderness in the wrists and hands was noted. At that time, rheumatoidfactor was positive and anti-citrullinated peptide antibodies were
negative. Therapy was initiated with methotrexate up to 20 mg orally everyweek without improvement. Between episodes he has polyarthralgias. His
father died at age 36 years from a myocardial infarction. Findings onphysical examination are normal with the exception of small nodules on the
Achilles, olecranon, and tibial tubercle. His hands and foot films are normal.
QuestionWhich of the following tests is the most appropriate next step to establish the
diagnosis?A. Anti-citrullinated peptide antibody titer
B. Rapid plasma reagent test
C. Anti-endomysial tissue transglutaminase antibody titer
D. Fasting serum lipid profile
E. Anti-nuclear antibody titer
Correct Answer
D
Answer Rationale
Lipoprotein disorders result from defect in either the synthesis or catabolism
of lipoprotein. Familial hypercholesterolemia is an autosomal dominant
disorder caused by mutation in the gene encoding for low density lipoprotein
(LDL) receptor. Deficiency results in high concentration of LDL in connective
tissues and arterial beds causing xanthomas and atherosclerosis. In patients
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C4: LowRheumatoid factor: 298 U
CCP: < 10 U
Figure
QuestionWhich of the following is the most appropriate next step in management?
A. Add an anti-TNF agent
B. Increase methotrexate dose
C. Add anti- B cell therapy
D. Begin cyclophosphamide
E. Add aspirin
Correct Answer
D
Answer Rationale
This patient has a nailfold infarction and sensory symptoms. This should raise
concern about the possibility of the development of rheumatoid vasculitis
(RV). RV refers specifically to a protean, destructive inflammatory process
that is centered on the blood vessel wall itself and associated with substantial
morbidity. It may affect a wide range of blood vessel types, from medium
muscular arteries to somewhat smaller arterioles to postcapillary venules.
Within a given patient, clinical features of both medium- and small-vessel
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disease may be found. RV leads to necrosis, blood vessel occlusion, and
tissue ischemia in a manner that resembles other forms of systemic
vasculitis, particularly polyarteritis nodosa.
RV typically occurs in patients with long-standing, joint-destructive RA. In
one study, the mean duration between the diagnosis of RA and the onset of
vasculitic symptoms was 13.6 years. Presentations of RV within 5 years of
the RA diagnosis are very unusual. Patients with RV nearly always have
rheumatoid nodules and are typically strongly positive for rheumatoid
factor. RV usually develops at a time when the inflammatory arthritis is
"burned out," ie, when the erosive process that led to joint destruction has
become less active.
The areas of the body involved most commonly by RV are the skin, digits,
peripheral nerves, eyes, and heart. The most devastating form of RV is
characterized by a medium-sized vasculitis reminiscent of (and histologically
identical to) polyarteritis nodosa. Many of the clinical manifestations of RV
reflect this predilection for medium-sized vessels, even though purpura,
petechiae, and papules (manifestations of small-vessel involvement) may
also occur.
In one study of 32 patients with rheumatoid vasculitis, the presence of
cutaneous vasculitis, multifocal neuropathy,and depressed C4 levels were the
3 independent variables that best predicted mortality. This patient had all
three of these variables that suggests that the use of cyclophosphamide
would be appropriate for this patient.
Question Number: 49
CaseA 66-year-old man with a history of ongoing chronic alcohol abuse, comes to
the emergency department because of bilateral knee pain. Physicalexamination shows a thin man with stigmata of liver disease, poor hygiene,
several nonblanching ecchymoses on both upper and lower extremities (seepicture) and bilateral knee effusions. There are no skin abrasions or
ulcers. He is able to flex his knees to 95 degrees with discomfort; both
knees are warm to touch. Arthrocentesis of the right knee is performed andshows a hemorrhagic effusion. A nontraumatic tap of the left knee reveals ahemorrhagic effusion also. (Image 1)
Laboratory data include:
CBC: WBC 5,000/mm3, hgb11.6 gm/dl; platelet count 95,000/mm3
PT: 10.7 seconds
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PTT: 36 secondsBUN: 24 mg/dl
Creatinine: 1.4 mg/dlAST: 96 U/LALT: 45 U/L
Figure
Question
Which of the following is the most likely cause of the hemarthrosis?A. Thrombocytopenia
B. Pigmented villonodular synovitis
C. Decreased ascorbic acid
D. Circulating anticoagulant
Correct Answer
C
Answer RationaleVascular fragility due to vitamin C deficiency may lead to hemarthrosis and
subperiosteal bleeding. Scurvy is a clinical syndrome seen with ascorbic acid
deficiency largely due to impaired collagen synthesis with disordered
connective tissue. Symptoms (occurring as early as three months after
deficient intake) include ecchymoses, bleeding gums, petechiae,
hyperkeratosis, Sjgren-like syndrome, arthralgias, and impaired wound
healing. Generalized systemic symptoms are weakness, malaise, joint
swelling, arthralgias, edema, coiled hair, depression, neuropathy, and
vasomotor instability. In the United States, ascorbic acid deficiency occurs
mostly in severely malnourished individuals, drug and alcohol abusers, orthose living in poverty. Although as many as 80 percent of hospitalized
alcoholics have mild thrombocytopenia, in most the platelet function remains
normal. Decreased production of coagulation factors occurs in hepatic failure
but this patients PT and PTT are normal, which also eliminates the possibility
of a circulating anticoagulant as the cause of the hemarthrosis. Pigmented
villonodular synovitis or diffuse tenosynovial giant cell tumor is a benign
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neoplasm that typically presents as an intra-articular tumor expanding along
the synovial surface into the soft tissue. Clinical presentation of PVNS
usually involves the knee with pain, swelling, and dysfunction. Bloody
effusions are common. These tumors present at a younger age with most
patients younger than 40, monoarticular, and without systemic
manifestations.
Question Number: 50
CaseA 36-year-old man has a 14- month history of intermittent pain and stiffness
in the hands and feet. One year ago, he was evaluated for the persistentsymptoms in his hands and feet. He had Achilles tendon pain. Occasionally
he would have lumbar spine pain; however, activity helped improve thesymptoms.
Physical examination one year ago showed swelling of the right 2nd and 4thtoes and the left 3rd finger. A modified Schober test was 10 cm to 15 cm.
Laboratory data one year ago include:
ESR: 25 mm/hr
Treatment with indomethacin provided incomplete resolution of thesymptoms; however, he was able to resume some of his daily activities.
Indomethacin was continued for two months and then discontinued.Methotrexate was then prescribed for treatment.
He now has had progressive low back pain and stiffness for the past 4months. He describes morning stiffness that lasts for 2 hours. Two monthsago, he had iritis that was treated with topical corticosteroids.
Physical examination today shows modified Schober test of 10 cm to 13
cm. The 2nd and 4th toes are swollen, but there is no tenderness.
Radiograph of the sacroiliac joint (SI joints) reveals erosive changes on theiliac sides.
Laboratory data now include:
ESR: 25 mm/hr
QuestionWhich of the following is the best treatment option at this time?
A. Leflunomide
B. Cyclosporine
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C. Methotrexate
D. Azathioprine
E. TNF alpha antagonist
Correct Answer
E
Answer Rationale
Ankylosing spondylitis affects mainly the axial skeleton. The majority of
patients will have sacroiliac joint involvement. If a patient has peripheral
joint arthritis then sulfasalazine, cyclosporine, methotrexate or Arava may be
effective. However, axial involvement is best treated with TNF alpha
antagonist. Concomitant use of methotrexate with TNF alpha antagonist has
not been found to be superior to TNF alpha antagonist monotherapy for
treatment of axial manifestations.
Question Number: 51
CaseA 19-year-old African-American man has joint pain and a positive ANA (1:80
dilution). Approximately 3 years ago, he started having intermittentepisodes of severe bilateral knee, ankle, and hip pain. During these episodeshe has difficulty getting around. They occur 2 to 3 times a year, last for 2 to
3 days, and resolve spontaneously. He has not had any associatedsymptoms, such as fever, rash, swollen joints, or cardiopulmonaryproblems. NSAIDs, oxycodone, and morphine did not provide any
benefit. He is a smoker, nondrinker, and denies any illicit drug use. Heplays golf but has no other physical activities. He has not traveled outside
the USA. His mother has systemic lupus erythematosus. Findings onphysical examination are normal.
Laboratory data include:
Hgb: 11 gm/dl
Hct: 36%ENA: negative
Chemistry profile: normal
Urinalysis: normalPlain radiographs of chest and knees: normalMRI of the right knee: Infarct of the medial femoral condyle, small infarct ofthe anterior distal femoral metaphysis and multiple small areas of edema.
QuestionWhich of the following studies is most likely to establish the diagnosis?
A. Bone marrow biopsy
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B. Bone scan
C. Hemoglobin electrophoresis
D. Anti-native DNA antibodies
E. Antiphospholipid antibodies
Correct Answer
C
Answer Rationale
The clinical diagnosis of osteonecrosis is appropriately made in a
symptomatic patient when imaging findings are compatible with this disease
and other causes of pain and bony abnormalities are either unlikely or have
been excluded by appropriate testing as in this case. Although there are
many conditions that lead to avascular necrosis, the most likely cause in this
case is hemoglobinopathy. Clues to this are young age of onset, the painfulcrisis, his ethnicity, and lack of systemic symptoms. Hemoglobin
electrophoresis will help to determine the cause of the painful crisis
associated with bone infarct. Long-bone infarct occurs most commonly with
HgBSS (sickle cell disease) but also with HbSC, HbS alpha thal, and least
commonly in sickle cell trait.
Bone marrow biopsy would be indicated if there were clinical findings
suggestive of Gauchers disease. Gauchers disease is a hereditary
(autosomal recessive) disorder of glucocerebroside metabolism that results in
the accumulation of cerebroside-filled cells within the bone marrow. Thisprocess may lead to compression of the vasculature and subsequent
osteonecrosis (60% of cases). Patients with Gauchers disease usually have
hepatosplenomegaly, thrombocytopenia, and bleeding, which are not present
in the above case. Bone scan will help to confirm osteonecrosis and identify
other asymptomatic areas but would not provide the cause. Patients with
classic antiphospholipid antibody syndrome present with venous and/or
arterial thrombosis and not with isolated osteonecrosis. A native-DNA
antibody is not indicated as nothing in this patients history or examination
points to a systemic autoimmune process.
Question Number: 52
CaseA 58-year-old man has a 3-year history of progressive muscle weakness
involving his shoulder and pelvic girdles. He had some difficulty swallowingliquids. He has been treated for 3 months with prednisone 60 mg/day withno benefit.
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Physical examination shows 4/5 strength in shoulder abduction and hipflexion.
Previously, he has had CK levels that were 3 to 4 times the upper range ofnormal. Electrophysiologic studies revealed increased insertional activity,fibrillation, and positive waves. Motor units were of low amplitude and short
duration. A muscle biopsy showed some atrophic and triangular fibers witha few lymphocytes in and around muscle fibers. Centralized nuclei were seen
but no necrosis or regenerating cells were present. Vacuoles were seen insome fibers. Trichrome staining revealed rare ragged red change.
Histochemistry with ATPase staining revealed fiber type grouping, and Congored staining was positive in fibers with vacuoles.
QuestionIn addition to physical therapy for muscle strengthening, which of thefollowing management options is most appropriate at this time?
A. Taper the prednisone
B. Sart azathioprine after checking thiopurine methyltransferase (TPMT) activity and slowly taperprednisone
C. Start methotrexate and azathioprine after checking thiopurine methyltransferase (TPMT) activityand slowly taper prednisone
D. Add IVIg
E. Add methotrexate
Correct Answer
A
Answer Rationale
Muscle histology in patients with inclusion body myositis may look identical to
that from patients with polymyositis; that is, degenerating and regenerating
muscle fibers with lymphocytes infiltrating the muscle and inside muscle
fibers. Over time, these inflammatory changes tend to resolve. In addition,
Trichrome staining may show ragged red change within some fibers. This
change is indicative of mitochondrial abnormalities and would suggest a
mitochondrial myopathy. However, ragged red fibers are reported with aging
and a mitochondrial myopathy would not be associated with the
inflammatory changes seen in this patient. In addition, patients with
inclusion body myositis may have neuropathic abnormalities. On physicalexamination, these may manifest as distal or asymmetric muscle
weakness. In muscle tissue, these are indicated by finding triangular cells
and fiber type grouping. Amyloid, as demonstrated with Congo red staining,
is present in IBM muscle fibers and in some vacuoles.
Although it is appropriate to give a trial of immunosuppressive therapy to
patients with presumed inclusion body myositis, these agents should be
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discontinued when it is clear that the patient is not responding because
inclusion body myositis is refractory to these medications.
Question Number: 53
CaseA 46-year-old man has had hypertension and diabetes mellitus for 20 yearsand has now developed chronic kidney disease. Ten years ago, he had whatappears to have been his first attack of gout. Synovial fluid aspirated from
his right knee at the time of a second attack contained monosodium uratecrystals. Over the next few years, he had multiple attacks of gout and visible
tophi developed on his elbows.
Two years ago, when his serum creatinine level was 2.0 mg/dl, he wasprescribed allopurinol 100 mg. This treatment led to a drop in his serumurate level from 11.0 mg/dl to 9.0 mg/dl. One year ago, the dose ofallopurinol was increased to 300 mg and colchicine 0.5 mg every other day
was added.
The diabetes mellitus is managed with insulin, he is takinghydrochlorothiazide 12.5 mg/day and losartan 25 mg for blood pressure
control, and he takes ASA 81 mg daily for cardioprotection.
He has not had a gout flare in the past 9 months but the tophi remainunchanged. Currently blood pressure is 165/100 mmHg, creatinine level is
2.3 mg/dl, and serum urate is 6.9 mg/dl (normal urate level in men is listedas 3.6-8.5 mg/dl).
QuestionWhich of the following interventions is most appropriate at this time?
A. Discontinue colchicne
B. Switch losartan to lisinopril
C. Discontinue the ASA
D. Add probenecid 1 g BID
E. Increase allopurinol to 400 mg/day
Correct Answer
E
Answer Rationale
Although many laboratories list the upper limit of normal for serum urate
levels above 6.8 mg/dl, the true physiochemical upper limit of normal of
serum urate is 6.8 mg dl. Above this concentration, body fluids are
supersaturated with urate and crystals can form and deposit. When levels
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above 6.8 mg/dl are listed as normal, they are based on statistical norms for
a population and are misleading regarding the pathophysiology of the
gout. To eliminate attacks and shrink tophi, serum urate levels must be
maintained below 6.8 mg/dl and preferably below a target value of 6.0
mg/dl.
Renal dysfunction increases the effectiveness of a given dose of allopurinol
because it delays the excretion of its active metabolite from the
body. Accordingly, the dose of allopurinol that is effective is usually lower
than that required for patients with normal renal function. Regardless, the
appropriate management of hyperuricemia in gout patients is to use the
lowest dose of whatever urate-lowering agent is used to maintain the serum
urate below 6.0 mg/dl.
Cochicine-induced axonal neuromyopathy most commonly occurs when renal
dysfunction is present, but it is reversible not permanent.
Losartan is an ideal antihypertensive agent for gout patients because it has
modest uricosuric effects. Hypertension in diabetic patients should be
managed with both an ACE inhibitor (such as lisinopril) and an ARB (such as
losartan). Increasing the dose of hydrochlorothiazide would not improve
blood pressure control but would induce a diuresis and have a hyperuricemic
effect.
Probenecid is not an effective agent in patients with renal dysfunction.
Question Number: 54
Case
A 52-year-old woman has recently received the new diagnosis ofosteoarthritis in the knees, which was made by her primary carephysician. She is otherwise generally healthy. She was a very active runnerfor many years and participated in 5K races until about 2 years ago. Now
she just jogs recreationally because she is somewhat limited by pain in herknees. She does not recall that anyone in her family had arthritis, and she
wants to know if there is anything she can do to improve her prognosis.
She had a hysterectomy 3 years ago. Her current medications include anestrogen patch and calcium supplements. She does not have diabetesmellitus or hypertension. She has smoked about a pack of cigarettes daily
for many years.
On examination, blood pressure is 135/72 mmHg and heart rate is 72/min.
She weighs 145 pounds and her height is 5 feet 5 inches; her calculated BMIis 24.1. She has crepitance in both knees, but the joints have full range of
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motion. There are no effusions in the knees. She has normal quadricepsmass and strength bilaterally.
Radiograph of the knee is shown in figure below.
Figure
QuestionWhich of the following has most likely contributed to cartilage loss in this
patient?
A. BMI of 24.1
B. Jogging
C. Use of cigarettes
D. Use of the estrogen patch
Correct Answer
C
Answer Rationale
While weight can impact osteoarthritis of the knee, this woman has a normal
BMI, so loss of weight is not likely to have significant benefit. Several
studies have now discounted the role of jogging or distance running in
accelerating the progression of knee OA. Furthermore, strength of the
quadriceps muscles that can be developed by this kind of exercise appears to
be an important factor in protection of the knee joint. Recent data suggest
that smoking is a modifiable risk factor for cartilage loss, so quitting cigarette
use is something that she can do to improve her joint health. Estrogen
supplements may slow rather than accelerate cartilage loss, so discontinuing
the patch probably will not help her joints.
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Question Number: 55
CaseA 28-year-old woman with a history of systemic lupus erythematosus,manifesting in the past as pleurisy, arthralgia, malar rash, and prior secondtrimester fetal loss, now has a positive pregnancy test. Her last menstrual
period was eight weeks ago. She feels fatigued but has no othersymptoms. She takes hydroxychloroquine 400 mg once daily and a prenatal
multivitamin. On physical examination, blood pressure is 110/70 mmHg andpulse rate is 88 bpm. No rashes are present.
Laboratory data include:
ANA: 1:1280; homogeneous pattern
Urinalysis: trace protein; no RBC or WBC casts or cells/hpfSerum creatinine: 0.9 mg/dl
Hgb: 11.3 mg/dl
WBC: 3400/mm3Platelet count: 122,000/mm3APTT: 56 sec
Anticardiolipin antibodies: IgG 58 GPL units; IgM 11 MPL units
QuestionWhich of the following is the most appropriate additional treatment for this
patient at this time?
A. Aspirin 81 mg per day
B. Aspirin 325 mg per day
C. Heparin 10,000 IU sq bid and prednisone 60 mg orally daily
D. Heparin 10,000 IU sq bid and aspirin 81 mg per day
E. Prednisone 60 mg orally daily
Correct Answer
D
Answer Rationale
This patient is in her first trimester of pregnancy and has a history of
antiphospholipid syndrome with moderate titer anticardiolipin IgG antibody
positivity and prior fetal loss. Her risk for miscarriage is increased. Studies
comparing heparin and aspirin versus aspirin alone show superiority of
combination therapy. Prednisone use poses greater risk for corticosteroid
complications without improvement in outcome.
Question Number: 56
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CaseA 72-year-old woman has had a 3-month history of an erythematous rash
over her face, forearms, and upper torso. Serologic testing revealed thepresence of ANA (1:640, speckled pattern) and hydroxychloroquine 400mg/day was prescribed. She has had no joint swelling or muscle weaknessbut has had some mild dysphagia for liquids. Physical examination shows
normal blood pressure; there is a confluent erythematous papular rash overthe upper torso and sun-exposed areas of her arms extending onto the
dorsal aspect of the hands with periungual erythema and mild scaleformation over the dorsal PIP and MCP joints. Erythema is also present over
the sun-exposed areas of the face and eyelids. Oral and ocular membranesare normal and lungs are clear without audible rub or crackles. Joints arenormal without limitation or synovitis. Strength in the upper and lowerextremity muscle groups is normal.
Laboratory data include:
CBC: normalChemistry profile: normalUrinalysis: normal
CK: normalAldolase: normal
Barium swallow radiograph: no mass, abnormal peristalsis in upper
esophagusChest, abdominal and pelvic CT scans: normal
Question
Which of the following studies is most appropriate at this time?A. Biopsy of affected dermis
B. EMG-directed muscle biopsy of an extremity muscle
C. MRI of the shoulder and pelvic girdle muscles
D. Vaginal ultrasonography and Ca-125 antigen study
E. Serologic tests for anti-dsDNA and anti-SSA
Correct Answer
D
Answer Rationale
Erythematous rash occurring on the face, eyelids, neck, and upper torso
along with papular lesions on the dorsum of the hands with periungual
erythema are very characteristic of dermatomyositis. The presence of orbital
violaceous erythema (heliotrope rash) and erythematous papules over the
dorsum of the hands is sufficient to establish the diagnosis of
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dermatomyositis. Skin biopsy frequently reveals interface dermatitis similar
to that seen in lupus and, for patients with these classic cutaneous
manifestations, adds little information to guide management. For patients
with demonstrable muscle weakness or CK elevation in the context of these
characteristic skin lesions, muscle biopsy is also not required to establish the
diagnosis.
The myopathy of dermatomyositis may be quite indolent or not clinically
manifest (dermatomyositis cine myositis). Moreover, it is important to
recognize that significant motor weakness may occur in the absence of
demonstrable elevations in creatine kinase or aldolase levels. Dysphagia due
to inflammatory myopathy of the pharyngeal muscles and/or striated portion
of the upper esophagus may be the initial presenting symptom of motor
weakness in patients with dermatomyositis.
The development of dermatomyositis, particularly in elderly patients,
requires consideration of possible associated malignancy. As such, in patients
such as the one presented, appropriate studies to determine the presence of
underlying lung, gastrointestinal/abdominal, or pelvic malignancies should be
performed. The prevalence of ovarian cancers in female patients with
dermatomyositis is particularly increased relative to the expected occurrence
and ovarian tumors in the early stages may be difficult to detect on routine
CT scanning. Serum studies for ovarian tumor antigens (Ca125) and vaginal
ultrasonography should, therefore, be performed to look for ovarian tumors
in female patients with dermatomyositis who have not undergone
oopherectomy.
Initial treatment of dermatomyositis occurring in the context of malignancy is
directed toward treatment of the neoplasm. In the absence of malignancy (or
in patients with persistent dermatomyositis manifestations despite removal
or treatment of the tumor), the inflammatory myopathy usually responds
favorably to corticosteroids, methotrexate, or other immunosuppressive
medications such as azathioprine. The cutaneous manifestations of
dermatomyositis are not uncommonly refractory to treatment with
corticosteroids and conventional immunosuppressive therapies. Skin disease
as well as the myopathy (when refractory to corticosteroids, methotrexate,
and/or other immunosuppressives) often responds favorably to a course of
IVIG, usually at a dose of 2 gm/kg administered in divided doses over 2-4
days. Preparations stabilized with sugars such as sucrose carry a higher risk
for renal toxicity and are best administered slowly, not exceeding 0.5
gm/kg/day.
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Diabetes mellitus is a much less likely cause of underlying peripheral
neuropathy, given the low-normal fasting glucose level. There is no
association between folic acid deficiency and carpal tunnel syndrome.
Acromegaly, diagnosed by the detection of an elevated insulin-like growth
factor I level, is also unlikely to be the cause of the worsening peripheral
neuropathic symptoms given his slight build and fine features, as opposed to
the more coarse features and broad hands expected in an individual with the
onset of acromegaly after puberty.
Question Number: 58
CaseA 55-year-old woman with diabetic glomerulosclerosis started hemodialysis 3
years ago. One year ago, swelling of her feet, lower legs, and handsdeveloped. Within a few weeks, this swelling progressed to a woody
induration, leading to flexion contractures of her fingers. There was also
progression of the induration to the forearms, upper legs, and thighs.Currently the affected skin has a brawny induration with areas of a peaudorange appearance as well as deep furrowing and loss of skin appendages.Skin biopsy shows diffuse dermal fibroplasia with spindle cells extending intothe subcutaneous tissues. There are thick collagen bundles separated by
large clefts in the dermis. Increased mucin is present.
QuestionWhich of the following additional findings is most likely in this patient?
A. Monoclonal gammopathy
B. Peripheral eosinophilia
C. Normal sedimentation rate and C-reactive protein
D. Raynauds phenomenon
E. Myocardial fibrosis
Correct Answer
E
Answer Rationale
Nephrogenic systemic fibrosis (NSF), formerly known as nephrogenic
fibrosing dermopathy, has been observed exclusively in patients with renal
insufficiency, most but not all of whom have undergone dialysis for renal
failure. The disease was initially thought to be confined to the skin. However,
recent reports have documented involvement of the liver, myocardium,
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lungs, diaphragm, and skeletal muscle. Increased ESR and C-reactive protein
level are commonly present. Paraprotein is a feature of scleromyxedema but
is not seen in NSF. The disease has been linked recently to the use of
gadolinium-based contrast agents for magnetic resonance imaging.
Question Number: 59
CaseA 72-year-old woman has had right knee pain and stiffness for the past six
months when arising from sitting. The knee pain worsens when she climbsstairs. She has had no trauma. She has a history of gastroesophageal refluxdisease and hypertension for which she takes omeprazole and enalapril. Onphysical examination, her weight is 110 pounds. Blood pressure is 130/80
mmHg. Right knee crepitus, medial joint tenderness, and a fluid wave arepresent. Radiograph of the right knee shows mild joint space
narrowing. She is treated with three weekly intra-articular injections of hylanG-F 20.
Three days after her last injection, she has severe worsening of pain andswelling of the right knee. Arthrocentesis of the right knee is performed andreveals 55 cc of cloudy yellow fluid.
Synovial fluid analysis is performed and reveals the following results:
WBC: 74,000/ mm3, differential 85% neutrophils,15% lymphocytes
RBC: 100/mm3Crystal analysis: no crystals observedGram stain: negative
Culture: pending
QuestionWhich of the following is the most likely diagnosis?
A. Osteonecrosis
B. Pseudogout
C. Pseudoseptic reaction
D. Rheumatoid arthritis
E. Septic joint
Correct Answer
C
Answer Rationale
This patient has a pseudoseptic reaction to injection with a hyaluronate. This
type of reaction may occur in 11% of injections with hyaluronate and is most
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commonly noted with hylan G-F 20. This reaction usually occurs in the first
week after the injection. Nucleated cell counts may exceed 100,000/mm3
and are predominantly neutrophils. Iatrogenic septic joint after intra-
articular injection has been estimated to occur once in 2,000 to once in
15,000 injections. Some cases of crystal-proven pseudogout have been
reported to occur after injection of hylan G-F 20. However, this patients
synovial fluid did not contain any crystals. Osteonecrosis is not a
complication of hyaluronate injection and is not associated with highly
inflammatory synovial fluid. This patient does not meet criteria for a
diagnosis of RA.
Question Number: 60
CaseA 14-year-old girl has a 6-week history of unexplained low-grade fever,fatigue, and joint pain. Family history reveals that her 4-year-old brother
has a history of perirectal abcesses, two hospitalizations for staphylococcalpneumonia, and a chronic cervical lymphadenitis for which culture grewSerratia species.
Question
Which of the following is the most likely diagnosis in the sister?
A. Inflammatory bowel disease
B. Common variable immune deficiency
C. Endocardial elastosis
D. Systemic lupus erythematosus
E. Chronic EBV syndrome
Correct Answer
D
Answer Rationale
The x-linked form of CGD usually presents with perirectal abcess and
difficulties in clearing staph infection and infection caused by other catylase-
positive organisms. Having a brother with these symptoms should raise the
index of suspicion of SLE. More commonly, this is discoid lupus, but it canalso be systemic. In the absence of that diagnosis, IBD and CVID could also
cause similar symptoms and appropriate evaluation should be undertaken.
Inflammatory bowel disease may present with fever, fatigue, and arthralgia,
but the family history points more toward SLE. Common variable immune
deficiency (CVID) may also have similar features, but the age of onset and
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types of infections in the brother are not those usually found with CVID. The
carriers of CGD are also at higher risk for endocardial fibroelastosis, but the
symptoms would more likely be shortness of breath and cardiac failure. The
patient had no adenopathy or rash suggestive of EVB, and duration is too
long for this to be likely.