Background/Purpose: Primary Sjögren’s Syndrome (pSS) is a chronic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands. Is the connective tissue disease that is most frequently associated with other autoimmune disorders and approximately 30% of these patients have another associated autoimmune condition. To date, no studies have evaluated the frequency of another autoimmune connective tissue disease development in patients with initial diagnosis of pSS. 
OBJECTIVES: To determine the frequency of patients who, following the diagnosis of pSS, developed another autoimmune rheumatic disease during their follow-up using the GESSAR database (Sjögren Syndrome Study Group of the Argentine Society of Rheumatology). To detail the connective tissue diseases that occurred most frequently in these patients. To describe the clinical, serological and salivary gland histology characteristics of these patients.

Methods: Descriptive, observational, cross-sectional study. We analyzed the data of patients diagnosed with pSS (American-European criteria 2002 / ACR-EULAR 2016), included in the GESSAR database. It was considered the development of another connective tissue autoimmune disease in those cases in which the diagnosis was added during follow-up.

Results: 681 patients were included, mean of following in years was 4.7 años (SD 4.94), 94.8% were women, mean age of 54 (+/- 14) and mean age at diagnosis of 50 (+/- 13) years. Of the total of patients, 30 (4.41%) developed another autoimmune rheumatic disease, according to the classification criteria for each disease, in its evolution. The median time to development was 4 (ICR 2-9). They were: Rheumatoid Arthritis (RA) 14 patients, scleroderma 9 patients, lupus (SLE) 5 patients, dermatomyositis 1 patient and RA plus SLE 1 patient. The mean age of this subgroup was 53 (+/- 14), with a mean age at diagnosis of pSS 48 (+/- 13), 91 % were women. Regarding the SICCA symptoms: 96% reported xerophthalmia, and 86.2% xerostomia to the diagnosis of pSS. With respect to the objective tests: 92% had positive Schirmer test, 88.24% Bengal Rose test or Lisamine Green or positive staining score, 81.2% positive sialometry, Ro + 82.1% and La + 33.33% of patients. Of the 30 patients, 14 had a minor salivary gland biopsy, 12 of them with positive results. Prior to the diagnosis of RA, 78% of the patients presented with arthralgia and arthritis, 12 had RF + and in 2 of the 14 patients the dose of ACPA was recorded and the result was positive. Prior to the diagnosis of scleroderma, 44% of the patients who developed this disease had raynaud’s phenomenon and 22.22% ANA with centromeric pattern, 1 patient with PHT and 3 patients with esophagitis due to reflux. 20% of patients who added SLE also presented raynaud’s phenomenon. 1 patient had anticardiolipin +, 100% reported arthralgia and 80% had arthritis.

Conclusion: Of all the patients analyzed, 4.4% developed another connective tissue disease during their follow-up. We consider the importance of recognizing this possibility in order to arrive at an early diagnosis. The presence of certain clinical and serological manifestations could be considered as suggestive of the subsequent development of another autoimmune rheumatic disease.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/primary-sjogrens-syndrome-and-development-of-another-connective-tissue-disease-during-follow-up/