Background Primary Sjögren syndrome (pSS) is a systemic autoimmune disease affecting primarily the exocrine glandular system. More than half of the patients with 10 or more years of follow up show extra-glandular manifestations, which will be determinant for the prognosis. Several validated methods are available in order to assess activity in pSS, including the Sjogren's Syndrome Disease Activity Index (SSDAI) and the EULAR Sjogren's Syndrome Disease Activity Index ESSDAI).
Objectives The aim of our study was to evaluate disease activity in a representative cohort of patients followed in rheumatology departments.
Methods We conducted a multicenter descriptive transversal study of a cohort of Spanish pSS patients fulfilling European/American criteria. It is a randomised register of patients obtained from 33 spanish rheumatology departments. We used SSDAI and ESSDAI in every patient. Informed consent was obtained and local ethics committees approved the study. Variables were analysed by descriptive statistical methods, using means, medians, and rates, with their deviations and interquartile ranges (p25-p75).
Results A total of 437 patients were included. Median score for SSDAI was 1 (0-3, p25-p75) and for ESSDAI was 2 (0-4, p25-p75). More than 95% of patients did not show fever, night sweating or weight loss. Twenty percent of them manifested weakness. Less than 5% of patients suffered glandular inflammatory disease or lymphadenopathy. Thirty two percent referred joint pain with stiffness but only 3% had true sinovitis. Fourteen patients (3%) showed cutaneous involvement. Fourteen percent of patients developed pleural involvement. High resolution CT was performed in 89 patients and showed interstitial disease in less than 4% percent of them. Serum creatinine was elevated in 3% of the cases. Seven patients developed tubular acidosis and 2 patients glomerular involvement. Less than two percent of patients had central nervous system involvement and 3% had peripheral nervous system involvement. Twenty seven percent of patients developed some kind of cytopenia: anaemia 14%, lymphopenia 14%, neutropenia 7% and thrombocytopenia, 9%. Twenty three percent had monoclonal component and/or hypoclomplementemia and/or hypergammaglobulinemia and/or high Ig G levels. Four percent showed cryoglobulinemia and/or hypergammaglobulinemia and/or high Ig G levels and/or recent hypogammaglobulinemia or a recent IgG levels fall.
Conclusions pSS patients show low scores at the systemic damage indexes. Most affected domains were haematological, biological and articular. Considering parenchymatous and pleural involvement, lung is the organ most frequently involved.
Disclosure of Interest None declared