Association between Secondary and Primary Sjögren’s Syndrome in a Large Collection of Lupus Families

Rachna Aggarwal,R. Hal Scofield,Biji T. Kurien,Kristi A. Koelsch,Juan-Manuel Anaya

doi:10.1155/2015/298506

Rachna Aggarwal, R. Hal Scofield + Show 3 more

Open Access

https://doi.org/10.1155/2015/298506

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Abstract

Objective. Systemic lupus erythematosus (SLE) and Sjögren's syndrome (SS) share clinical and immunogenetic features and may occur together. We undertook this study to determine the risk of primary SS among SLE-unaffected relatives of SLE patients and whether or not primary and secondary SS tended to occur in the same families. Methods. We collected clinical and serological data on 2694 SLE patients, 7390 SLE-unaffected relatives of the SLE patients, and 1470 matched controls. Results. Of the 2694 subjects with SLE, 548 had secondary SS, while 71 of their 7390 SLE-unaffected relatives had primary SS. None of the 1470 controls had SS as defined herein (p = 5 × 10−5 compared to SLE-unaffected relatives). Of the 71 SLE-unaffected relatives with primary SS, 18 (25.3%) had an SLE-affected family member with secondary SS, while only 530 of the 7319 (7.2%) SLE-unaffected relatives without SS did so (p = 1 × 10−8). Conclusion. Among families identified for the presence of SLE, primary and secondary SS tend to occur within the same families. These results highlight the commonalities between these two forms of SS, which in fact correspond to the same disease.

Highlights

Sjogren’s syndrome (SS) is a chronic autoimmune disease that characteristically affects salivary and lacrimal glands such that patients have severe dry eyes and dry mouth [1]
Putative Systemic lupus erythematosus (SLE) patients completed an extensive questionnaire, underwent an interview, and had medical records reviewed to ensure that all patients met the American College of Rheumatology SLE classification criteria [10, 16]
The majority of Sjogren’s patients have anti-Ro and about half of these same patients have anti-La antibodies. Both anti-Ro and anti-La occur in those with SLE with less frequency, but anti-La is associated with lack of renal and central nervous system disease [20]

Summary

Introduction

Sjogren’s syndrome (SS) is a chronic autoimmune disease that characteristically affects salivary and lacrimal glands such that patients have severe dry eyes and dry mouth [1]. Pathology in these glands consists of lymphocytic infiltration, commonly with germinal center formation [2]. The disease may involve numerous other organs, including the lungs, kidneys, joints, skin, peripheral nerves, and brain. The diagnosis of SS is based on the combination of symptoms (sicca symptoms) and the presence of the autoimmune characteristics: activation of T cells (confirmed in practice by a positive salivary gland biopsy) or B cells (i.e., presence of autoantibodies). SS may be the second only to rheumatoid arthritis in terms of prevalence of the inflammatory rheumatic illnesses [5]

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Conclusion