Background/Purpose: Aim of the study was to characterize the spectrum of presenting clinical features in a single-centre inception cohort of  patients  with primary Sjögren’s syndrome (SS) focusing in particular on the leading symptoms for the diagnosis, on the mean latency between the onset and the diagnosis and, on how the clinical presentation  may impact on the long term follow-up of the disease. 

Methods: Data were obtained from an inception cohort of patients with SS (AECG 2002) recruited from 2000. The following data were recorded: demographics, presenting symptoms leading to the diagnosis, mean latency from the onset of the disease to the diagnosis, patients’ laboratory assessment, histological data at the diagnosis and clinical features at last follow-up. Descriptive statistics were used to assess clinic-serological correlations. 

Results: Among 378 patients enrolled (368 F) 99.2% were Caucasians. Mean age (SD) of patients at diagnosis was 51(15) years, and mean latency between the onset of the disease and the diagnosis was 78 (71) months. At disease onset, four clinical subsets were recognizable. The vast majority of the patients presented either isolated sicca symptoms (127/378, 33.7%) or sicca symptoms associated with Raynaud’s phenomenon, artralgias/arthritis, and mild cytopenias (188/378, 49.7%). A third subset  (23/378, 6%) was characterized by the presence of more severe systemic manifestations, including: interstitial lung disease (8/23), serositis (5/23), polineuropathy (3/23), central nervous system involvement (3/23), myositis (1/23) skin rashes (1/23) and renal involvement (2/23). Finally, a fourth subset (40/378, 10.6%) was represented by patients presenting recurrent salivary gland enlargement, purpura , peripheral nervous involvement and/or other features traditionally associated with a higher risk for non-Hodking’s lymphoma (NHL) occurrence. Patients with isolated sicca symptoms were significantly older, presented less likely a positivity for anti-Ro/SSA, anti-La/SSB autoantibodies and Rheumatoid Factor and showed a lower mean focus score (FS) in the minor salivary gland biopsies. Patients presenting risk factors for lymphoma had a higher mean FS, and were more likely to present low C4 levels, cryoglobulins and monoclonal gammopathy. At last follow-up severe systemic manifestations requiring immunosuppressants were observed in additional 35 patients who had presented at the onset only mild signs of autoimmunity and in 9 patients who had also developed a NHL over the time. Overall, 24 patients developed a NHL; out of them, 21/24 had presented either salivary gland enlargement, purpura and/or peripheral nervous involvement since the diagnosis. 

Conclusion: In this study we described different clinical subsets in patients with SS at the diagnosis. The impact of the disease clinical presentation on the subsequent disease course seems to be not negligible thus highlighting the importance of a comprehensive disease assessment since the early phases of the disease.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-subsets-and-presenting-features-in-primary-sjogrens-syndrome-results-from-a-single-center-inception-cohort-study/