Introduction:Sjögren’s Syndrome (SS) is a systemic autoim-

mune disease characterised by lymphocytic infiltrationof exocrine glands resulting in xerostomia and keratoconjunctivitis sicca. Extra glandular involvement ofmuscles, blood vessels, lungs, kidneys may also occur.It may be primary or secondary, when associated withother connective tissue diseases. Antibodies toSSA(Ro) and SSB (La) are characteristically associ-ated with SS. There is a risk of progression to lym-phoid malignancy. As SS is rarely reported from ourcountry, we decided to study the clinical and immuno-logical features of our patients.

Original article J Indian Rheumatol Assoc 2003 : 11 : 63 - 65

PRIMARY SJÖGREN’S SYNDROME - CLINICAL AND IMMUNOLOGICALFEATURESR Porkodi*, S Rukmangatharajan*, P Kanakarani*, M Parthiban**, N Vasanthy***, RadhaMadavan +, C Rajendran Panchapakesa++.Assistant Professor of Rheumatology*, Special Grade Bio chemist**, Tutor In Immunology***, Additional Professor ofImmunology+, Professor of Rheumatology++, Department of Rheumatology , Madras Medical College, Chennai.

Abstract:Aim: To retrospectively analyse the clinical and immunological features of patients with primary Sjögren’sSyndrome.Material and Methods: This is a retrospective analysis of patients with primary Sjögren’s syndrome fromJanuary 1998 through June 2002. Clinical and investigational data including histology and immunologi-cal markers were retrieved. Immunological markers included RF, ANA and antibodies to SS A and B (Roand La)Results: Over this period, 36 patients (26 F 10 M) with primary Sjögren’s Syndrome were diagnosed. Themean age was 40.4 years. The common presenting symptoms were dry mouth (32; 88.9%), dry eyes (31;86.1% ), arthritis/arthralgia (28;77.8%) and parotid gland enlargement (12;33.3%). Rarer extraglandularfeatures were renal tubular acidosis, Raynaud’s phenomenon, peripheral neuritis and purpura. Lip bi-opsy was positive in 94.7%. Of the immunological markers, RF (52.8%), ANA in 77.3%, antibodies toSSA in 71.4% , SSB in 57% of patients.Conclusion: The clinical and laboratory features of primary Sjögren syndrome as seen in South India iscomparable to published report from Western countries.Key Words: Xerostomia, Xerophthalmia, Parotid swelling, Renal tubular acidosis.

Patients and Methods:This was a retrospective analysis of patients who

fulfilled the San Diego Criteria of SS seen at theDepartment of Rheumatology of the Madras MedicalCollege from January 1998 through June 2002. Xe-rophthalmia was documented by Schirmer’s test. It wasconsidered positive when the wetting of the 30 mmfilter paper inserted in the lower eye lid was less than 5mm in 5 minutes. Rose Bengal stain was used to iden-tify devitalised epithelium of the cornea and conjunc-tiva as punctate or filamentary keratitis under slit lamp.Tear break up time was done by instilling a drop offluorescein in the eye and noting the time between thelast blink and the appearance of dark, non fluorescentarea in the tear film. A rapid tear break up time indi-cated an abnormality.

Absence of salivary pool, with or withoutpresence of caries teeth and abnormal sialogram

Address for correspondenceR. PorkodiDept. of Rheumatology, Madras Medical College,Chennai - 600 003.

showing sialectasis was considered as evidence ofxerostomia. Biopsy of the minor salivary gland fromthe lower lip was carried out to assess forlymphocytic infiltration. A focus score of more than 2of the 4 evaluable lobes was considered as evidence ofinvolvement. A focus is defined as more than 50 lym-phocytes per 4 mm2. Sialogram and lip biopsy wereperformed by the dental surgeons at the MadrasDental College.

Data regarding extra glandular features like ar-thritis, myositis renal, gastro intestinal, neurological,pulmonary, vascular and cutaneous involvement wereretrieved.

Haemogram, total proteins and globulin level,serum electrolytes, renal parameters, liver enzymes wereevaluated. Plain radiographs of the chest and hands werecarried out in all cases. In one patient with renal os-teodystrophy, Xray of pelvis and lumbar spine weredone. Immunological tests included Rheumatoid Fac-tor (RF) by latex agglutination, Antinuclear antibody(ANA) by indirect immuno fluorescence and antibod-ies to SSA(Ro) and SSB (La) by commercially avail-able ELISA kit.

Results:Out of the 8000 patients who presented with

various rheumatic manifestations during the study pe-riod, 36 patients were detected to have SS. There were26 females and 10 males in a ratio of 7:3. The meanage at presentation was 40.4 years (range 13-60 years).The age and sex distribution is shown in Table 1. Themean duration of illness was 36.6 months (range 2months - 20 years).

The presenting symptoms were dry mouth anddry eyes in 20 (55.6%), polyarthritis in 8 (22.2%),parotid enlargement in 7(19.4%), hypokalemic peri-odic paralysis in 2 (5.5%), lacrimal gland enlargementand jaundice in 1 patient (2.8%) each. The more fre-

quent cumulative features were dry mouth in 32, dryeyes in 31, and arthritis/arthralgia in 28. Parotid glandenlargement was seen in 12. It was episodic in all, painfulin 6 and bilateral in 4. Lacrimal gland enlargement wasseen in a 13 year old girl.

Of the extrarticular manifestations, distal renaltubular acidosis was encountered in 4 which led to peri-odic paralysis in 3 and renal osteodystrophy in one.Raynaud’s phenomena and peripheral neuropathy wereseen in 2 each and purpura, dysphagia, autoimmune hepa-titis and autoimmune thyroiditis were seen in one each.

Majority had raised ESR with a mean ESR of66.2 (10-160 mm/1st hour). Hypergammaglobulinaemia was seen in 89.5%, positive Schirmer’s test in90.5%, rapid tear break up in 85.7% and a positiveminor salivary gland biopsy in 94.7%. Sialogram wasabnormal in 50% of cases. The result of immunologicalinvestigations are compared with one Indian study andone Western series (Table 2)

Table 1 : Age and Sex Distribution of 36 patients.

Age group Female Male Total (Years)11-20 1 - 121-30 7 - 731-40 6 5 1141-50 8 2 1051-60 3 3 7

26 10 36

Table 2 : Immunological markers in 36 patients with SScompared with another Indian study and one series from WestFigures are in percentage.

Immunological Present Misra et al3 Tzioufas et al5

markers studyRF 52.8 60 61ANA 77.3 65 92Anti SSA ab. 71.4 66 57Anti SSB abs. 57.1 62 38RF – Rheumatoid factor, ANA – Antinuclear antibodies, abs- anti-bodies

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Primary Sjögren’s Syndrome - Clinical and Immunological Features

Discussion:Primary SS is an autoimmune disease primarily

affecting females in the fourth and fifth decades of lifeas was seen in our study also. The youngest patientreported in literature was 3 years old2 and in our studythe youngest patient was 13 years old.

Primary Sjögren can present initially with pro-tean manifestations to various specialists like theopthalmologist, dentists, nephrologist, neurologist orgastroenterologist . The initial manifestation can benon-specific and it may take more than 10 years fromthe initial symptom to the full blown development of thesyndrome. Some of our patients had presented withparotid enlargement, gritty eyes several years beforethe diagnosis was made.

Dry mouth, dry eyes and arthritis/arthralgia arethe 3 common presenting features, both at onset andcumulative feature as reported from Lucknow, NorthIndia3 and abroad1. Arthralgia or arthritis like that ob-served in rheumatoid arthritis was the next commonclinical feature. Unlike RA, there was no evidence oferosions.

Of the extraarticular manifestations, Raynaud’sphenomena is the most common skin manifestation seenin 35% of patients in the Western5 countries. We hadfewer patients with Raynaud’s, which is probably dueto the hot climate present throughout the year. Vascu-litis has been reported in 5% of patients with SS6. Thisincludes small vessel leucocytoclastic vasculitis andmedium vessel necrotising vasculitis. In our study,peripheral sensory neuritis due to small vessel vasculi-tis was noted in 5.5% of patients. Palpable purpurawas seen in 2.8%, in contrast to 13.6% in Lucknow3.None of our patient had pulmonary or CNS manifes-tations. Distal RTA may be silent or lead to renal stones,nephrocalcinosis and compromised renal function.4

Hypergammaglobulinemia is due to polyclonalactivation of B cells . The frequency of ANA, RF ,antibodies to SSA (Ro) and SSB (La) were similar tothat reported from Lucknow3 but were in variance fromthe Western series. This variability could be due to tech-nical reasons. As histology was confirmatory in 94.7%of patients, it is more sensitive that antibodies to Ro/La(71-77%). In the proposed modification of the Euro-pean Criteria, a positive biopsy or auto antibodies toSSA/SSB is obligatory 6.

In conclusion, SS is a rarity in our referral cen-tre. The common manifestations were dry eyes, drymouth and arthritis/arthralgia. Notably, periodic paraly-sis due to distal RTA occurred in a minority of patients.An abnormal histology of the salivary gland was moresensitive than antibodies to SSA / SSB in the diagnosisof SS.

References:1. Fox RI, Saito I. Criteria for diagnosis of Sjögren’s Syn-

drome. Rheumatic Disease Clinics of North America 1994;20:2:391-407.

2. Siamopoulou-Mauridou A, Drosos AA, AndonopoulosAP. Sjögren’s Syndrome in childhood: report of twocases. Eur J Pediatr 1989; 8:523-4.

3. Misra R, Hissaria P, Tandon V, Aggarwal A, Krishnani N,Dabadghao S. Primary Sjögrens syndrome : Rarity in In-dia. J Assoc Phys India 2003; 51: 859-62.

4. Moutsopoulos HM, Cledes J, Skopouli FN, Elisaf M,Youinou P. Nephrocalcinosis in Sjögren’s Syndrome IntMed 1991; 230:187-91.

5. Tzioufas AG, Youinou P and Moutsopoulos HM Sjögren’sSyndrome in Oxford Text Book of Rheumatology 2nd ed,P.J. Maddison et al (eds) Oxford University Press 1998; p1301-1317.

6. Brun JG, Madland TM, Gjesdal CB, Bertelsen LT. Sjögren’sSyndrome in an out patient clinic. Classification of pa-tients according to the preliminary European Criteria andthe proposed modified European Criteria: Rheumatology2002; 41:301-304.

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