Date post: | 15-Jan-2015 |
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Prof.S.SUNDAR’S unitDr.G.Rengaraj.PG
History
Mrs. Kalyani, a 60 yr female came with c/o difficulty in swallowing – 2 years - more for solid foods - persistent -not progressive no h/o drooling of food or saliva no h/o nasal regurgitation of food no h/o hoarseness of voice no h/o vomiting h/o dryness of mouth – 2 yrs
History h/o dryness of both eyes – 2 yrs - asso. With gritty sensation in both eyes
- diff. in opening both eyes in morning
no h/o redness of eyes h/o pain in both knee joint – 6 weeks - more during activity, relieved by rest - no diurnal variation - no h/o any swelling in the joints - no h/o any other joint involvement
History no h/o any chronic drug intakeNot a known DM/HTNo h/o any surgery or irradiationNo h/o jaundiceNo positive family historyPM – attained menopause at 50 yrs of ageh/o cataract surgery done for both eyes 5 yrs
before
ExaminationO/E pt. conscious, oriented,afebrile no P/Ict./Cy./Cl./nodes/PE dry tongue + BP- 120/74 , P – 78/min CVS- S1 S2 + , no murmurs RS – NVBS + , no added sounds P/A – soft , no organomegaly CNS – NFND Both eyes – Normal
DD of sicca symptomsXerostomia Viral infectionsDrugs :
1.antihypertensive 2.parasympatholyticDMTraumaSjogren’s
Dry eyeInflammation: 1.chronic
conjunctivitis 2.chronic blepharitis 3. SJS 4. Pemphigoid 5. sjogren’s syn.Toxicity 1. Burns 2. drugs Misc. 1.trauma 2. hypovitaminosis A
INVESTIGATIONSInv. Hb- 10.0 Sugar – 133 PCV – 3O urea - 22 RBC – 3 millions/ml creatinine –
0.8 TC – 7000 Na – 138 DC – P67L30 E3 k - 4.6 ESR – 15 / 30 Bicarb - 22 Plt. – 2 lakhs/ ml Cl – 98 MCV - 92 MCH- 32 MCHC - 30
Inv.Urine r/e alb – nil sugar- nil deposits- 1-2 pus cells/hpf FBS – 78 , PPBS – 104CXR – NormalECG – Normal X-ray both knee – normal PS – Normocytic normochromic anemia +
Ophthal op. Rt. Eye Left eyeAS N NPupils irregular,sluggish RTL irregular,
RTL Lens pseudophakia
pseudophakiaRefr. 6/24 5/60 with PH no inc. in power 6/24SCHIRMERS TEST 2 mm 2 mm imp - sugg. of DRY EYE
INV.RF – POS.ANA - neg
Anti – SS-A - Positive Anti – SS-B - Negative
Biopsy Lower lip biopsy:Microscopic description:
skin lined by hyperkeratotic,hyperplastic squamous epithelium with underlying dermis showing inflammatory cell infiltrate,congested blood vessels, basal layers showing basilar vacuolisation & edema of the dermis
Impression: consistent with sjogren’s syndrome
Inv.Anti-HCV – neg.HIV – non-reactiveCT Chest – normalACLA – neg
Sjogren’s syndrome Revised International Classification Criteria for Sjögren's Syndrome I. Ocular symptoms: a positive response to at least one of the following
questions: Have you had daily, persistent, troublesome dry eyes for more than 3
months? Do you have a recurrent sensation of sand or gravel in the eyes? Do you use tear substitutes more than 3 times a day?
II. Oral symptoms: a positive response to at least one of the following questions:
Have you had a daily feeling of dry mouth for more than 3 months? Have you had recurrently or persistently swollen salivary glands as an
adult? Do you frequently drink liquids to aid in swallowing dry food?
criteriaIII. Ocular signs-that is, objective evidence of ocular
involvement defined as a positive result for at least one of the following two tests:
Schirmer's I test, performed without anaesthesia (</=5 mm in 5 minutes)
Rose bengal score or other ocular dye score (>/=4 according to van Bijsterveld's scoring system)
IV. Histopathology: In minor salivary glands (obtained through normal-appearing mucosa) focal lymphocytic sialoadenitis, evaluated by an expert histopathologist, with a focus score >/=1, defined as a number of lymphocytic foci (which are adjacent to normal-appearing mucous acini and contain more than 50 lymphocytes) per 4 mm2 of glandular tissue
CRITERIAV. Salivary gland involvement: objective evidence of
salivary gland involvement defined by a positive result for at least one of the following diagnostic tests:
Unstimulated whole salivary flow (</=1.5 ml in 15 minutes)
Parotid sialography showing the presence of diffuse sialectasias (punctate, cavitary or destructive pattern), without evidence of obstruction in the major ducts
Salivary scintigraphy showing delayed uptake, reduced concentration and/or delayed excretion of tracer
VI. Autoantibodies: presence in the serum of the
following autoantibodies:Antibodies to Ro(SSA) or La(SSB) antigens, or both
Criteria Revised Rules for Classification For primary SS In patients without any potentially associated disease, primary SS may be defined as follows: The presence of any 4 of the 6 items is indicative of primary SS, as long as either item IV
(Histopathology) or VI (Serology) is positive The presence of any 3 of the 4 objective criteria items (that is, items III, IV, V, VI) The classification tree procedure represents a valid alternative method for classification, although it
should be more properly used in clinical-epidemiological survey For secondary SS In patients with a potentially associated disease (for instance, another well defined connective
tissue disease), the presence of item I or item II plus any 2 from among items III, IV, and V may be considered as indicative of secondary SS
Exclusion criteria: Past head and neck radiation treatment Hepatitis C infection Acquired immunodeficiency disease (AIDS) Pre-existing lymphoma Sarcoidosis Graft versus host disease Use of anticholinergic drugs
Picture showing DRY,ERYTHEMATOUS ORAL MUCOSA of the ptThe pt. had h/o dry mouth daily for 2 yrs & frequently drinking liquids to aid in swallowing solid food – satisfying item 2 of the criteria
Sjogren’sIt is a chronic,slowly progressive autoimmune
disease characterised by lymphocytic infiltration of the exocrine glands.
Middle-aged women are primarily affected, but it can occur in all ages,including childhood
F:M = 9:1Prevalence – 0.5 to 1.0 %30 % of pts with autoimmune rheumatic disease
suffers from secondary sjogrens syn.Approx. 1/3rd of pts present with systemic
manifestations
Pathogenesis Autoimmune disease Both T cell lymphocytic infiltration of exocrine
glands & B cell lymphocytic hyperreactivity.Infiltrating lymphocytes provide apoptotic
signals to the epithelial cells.The serum contains autoantibodies directed
against non-organ-specific antigens such as immunoglobulins(RF) & extractable nuclear and cytoplasmic antigens ( Ro/SS-A, La/SS-B )
HLA-B8, DR3 & DRw52 are prevalent in pts with primary sjogren’s.
Clinical manifestationsMajority of pts have symptoms related to
diminished lacrimal & salivary gland functionIn most pts the primary syndrome runs a
slow & benign coursePredominant Glandular manifestations: xerostomia keratoconjunctivitis sicca other less frequent glandular
manifestations: xerotrachea,atrophic gastritis,subclinical
pancreatitis
Clinical.Extra-glandular manifestations are seen in one-third of pts:
low-grade fever raynaud’s phenomenon myalgia arthralgiaMost pts with primary sjogrens experience atleast
one episode of nonerosive arthritisrenal involvement – interstitial nephritis clinically
manifesting as hypoasthenuriaSN hearing loss was found in one-half of pts with
sjogrensVasculitis – purpura, recurrent urticaria, skin ulcers ILD
Clinical.A small but significant pts. Develop
malignant lymphoma. The features sugg. The development of lymphoma are:
persistent parotid enlargement purpura leukopenia cryoglobulinemiaMost lymphomas are extra-nodal marginal
zone B cell lymphomas & are usually detected incidentally during evaluation of labial biopsy
DiagnosisThe diagnosis of primary sjogren’s syndrome is
obtained if 1)the patient presents with eye and/or mouth
dryness, 2)the eye tests disclose keratoconjunctivitis sicca, 3)the mouth evaluation reveals classic
manifestations of the syndrome, and 4) the pt serum reacts with Ro/SS-A and/or La/SS-B
autoantigensLabial biopsy is needed when the diagnosis is
uncertain or to rule out other conditionsHCV infection should be ruled out since apart from
SEROLOGIC TESTS the remainder of the clinico-pathologic picture is almost identical to that of sjogren’s
DD of sjogren’s HIV infection & sicca syndrome
sjogren’s syn.
sarcoidosis
Age predominantly young males
predominantly in middle-aged women
invariable
Auto-antibodies - Presence of autoantibodies to Ro/SS-A and/or La/SS-B
-
Histology lymphoid infiltrates of salivary glands by CD8+ lymphocytes
Lymphoid infiltrates of salivary glands by CD4+ lymphocytes
granulomas in salivary glands
Genetics Associated with HLA-DR5
Associated with HLA-DR3 & DRw52
unknown
Serology Positive tests for HIV
Neg. tests Neg. tests
HIV & Sjogren’sImmunologic & rheumatologic disorders are common in
HIV ptsDrug allergies are the most significant allergic
reactions in HIV + , more common as the disease progresses(upto 65% for TMP/SMX)
SLE & RA are ameliorated by the concomitant HIV inf.The one autoimmune disease that may occur with
increased frequency in HIV inf. Is A VARIANT of PRIMARY SJOGREN’S
This syn. Consists of parotid gland enlargement,dry eyes & dry mouth
These pts donot have anti-SS-A or anti-SS-BLess common with increased use of ARVThe term DIFFUSE INFILTRATIVE LYMPHOCYTOSIS
SYNDROME(DILS) is used to describe this entity
Treatment Glandular manifestations:Dry eyes:1.Artificial tears without preservatives 2.avoidance of drugs that decrease
lacrimal & salivary secretion such as diuretics, antihypertensive drugs,anticholinergics & antidepressants.
3. systemic stimulation with oral pilocarpine 5 mg TID,oral cevimeline 30 mg TID
4. Severe dry eyes: NL duct occlusion ,soft contact lenses, corneal transplantation
Treatment Xerostomia:Lubrication – water( the best replacement)Local stimulation- flavoured lozenges or gumSystemic stimulation – as for dry eyesTopical application of fluorideOral candidiasis – topical nystatin or
clotrimazole lozengesParotid enlargement : Treat superinfection- antibiotics,analgesicsHard, persistent – r/o lymphoma
Treatment Extraglandular manifestations:Arthritis: Hydroxychloroquine 200-400 mg/day Raynaud’s : cold protection with gloves &
T.Nifedipine 10 mg TDSRTA : Bicarbonate replacementLymphoma : CHOP + anti-CD20Systemic vasculitis: glucocorticoids 1 mg/kg
per day
Secondary sjogren’sWhen it presents in association with other
autoimmune rheumatic diseasesThe common autoimmune diseases associated
with sjogren’s are: 1. Rheumatoid arthritis 2. SLE 3. Scleroderma 4. MCTD 5. Primary biliary cirrhosis 6. vasculitis 7. chronic active hepatitis