Background Systemic sclerosis (SSc) and Sjögren's syndrome (SS) are autoimmune diseases, which can coexist with sicca syndrome due to accumulation of collagen or chronic inflammation of the exocrine glands (salivary and endocrine). The prevalence of secondary SS to SSc ranges from 4 to 60%.
Objectives To determine the prevalence of SS and sicca syndrome in Mexican patients with SSc.
Methods Cross-sectional study. Seventy seven patients with SSc were studied at a tertiary hospital. SS and sicca syndrome were investigated according to the 2002 revised international criteria for Sjögren's syndrome by the American European Consensus Group (AECG). All patients underwent minor salivary gland biopsy, and the results of fibrosis andinflammation were classified according to Tarpley's classification with a score ≥1. Anti-Ro/SSA and Anti-La/SSB antibodies were quantified in 34 patients with positive histological findings for SS. All patients with ocular symptoms were also performed Schirmer's I test to evaluate the ocular manifestations.
Results Infiltration characteristic of SSc, and 21 (27%) revealed SS lymphocytic infiltrate. Isolatedxerostomia was found in 17 (22%) patients, and isolated xerophthalmia in 5 (7%) patients.Five (6.5%) patients referred parotid glands swelling. Anti-Ro/SSA and/or anti-La/SSBautoantibodies were found in 28.5% (6/21) of patients with SS. 21 (27%) patients met the2002 revised criteria for classification for SS by the AECG, Schirmer's I test revealed dryeye severity level 2 in 4 (19%) patients, level 3 in 12 (57%) patients and level 4 in 5 (23.8%) patients.
Conclusions The prevalence of sicca syndrome was 71%, 44 per cent related to systemic sclerosis's collagen infiltration, and 27% due to Sjögren's syndrome's lymphocytic infiltrate. It is important to give a treatment to improve symptoms and considering the high frequency of non Hodgkin lymphoma in patients with SSc and SS.
Kobak S, Oksel F, Aksu K, Kabasakal Y. The frequency of sicca symptoms and Sjögren's syndrome in patients with systemic sclerosis. Int J Rheum Dis. 2013;16:88–92.
Baldini C, Mosca M, Della Rossa A, Pepe P, Notarstefano C, Ferro F, Luciano N, Talarico R, Tani C, Tavoni AG, Bombardieri S. Overlap of ACA-positive systemic sclerosis and Sjögren's syndrome: a distinct clinical entity with mild organ involvement but at high risk of lymphoma. Clin Exp Rheumatol. 2013;31:272–80.
Avouac J, Sordet C, Depinay C, et al. Systemic Sclerosis - Associated Sjögren's Syndrome and Relationship to the Limited Cutaneous Subtype. Arthritis Rheum. 2006; 54: 2243–2249.
Disclosure of Interest None declared
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