Background/Purpose: Primary Sjögren’s Syndrome (pSS) is a systemic autoimmune disease which is mostly characterized by the presence of xerophthalmia and xerostomia, caused by the lymphoplasmacytic cell infiltration of the lacrimal and salivary glands. This pathological change can also affect other organs and produce various extraglandular manifestations (EGM), which may precede the typical sicca symptoms. The diversity of clinical presentations may partially explain the delay in diagnosis and determine the prognosis of the disease.

The purpose of this study was to describe the frequency of EGM as initial presentation in a cohort of patients with pSS and to evaluate the delay in diagnosis among patients with EGM versus sicca as initial symptoms. A secondary objective was to correlate disease onset form with age, sex, disease activity, accumulated damage, and other concomitant EGM.

Methods: Multicenter, observational, analytical, cross-sectional study. Consecutive patients ≥ 18 years of age with a diagnosis of pSS were included. Demographic and disease characteristics and clinimetric indices were recorded. EGM were defined by the EULAR-SS disease activity index (ESSDAI). The delay time from the EGM presentation to the diagnosis of the disease, and the time since the pSS diagnosis to the EGM presentation were considered.

Results: One-hundred eleven patients were included, 93% women, mean age 48.7 years (SD 13.2). 97.3% of the patients presented sicca symptoms (being the initial manifestation of the disease in 47% of them) and 90.9% had at least one EGM (being the initial symptom in 53% of the cases). The most frequent initial EGM was joint involvement (70.3%), followed by biological (32.6%) and pulmonary involvement (23.7%). No significant difference was observed in the time from the first manifestation and the diagnosis between the group that started with sicca (92 months [CI95 39.3-134.9] and that with EGM at onset (44.2 months [CI 95 29.2-59.2]); p = 0.063. Patients with EGM as initial symptom were younger at diagnosis compared to those who started with sicca symptoms (46.3 vs 51.4 years, p = 0.042). Mean ESSDAI score was higher in the group with EGM as initial symptom (1.2 vs 0.9; p = 0.002), while mean Sjögren’s Syndrome Disease Activity Index (SSDI) score was higher those with initial sicca symptoms (1.3 vs 0.9; p = 0.023). No significant differences were observed in other disease characteristics, or in the distribution of different EGM between the groups with sicca or EGM onset.

Conclusion: More than half of the patients with pSS presented an extraglandular involvement at disease onset. There were no differences in the time from the first manifestation to the diagnosis between the groups with sicca or extranglandular onset.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/extraglandular-manifestations-as-initial-presentation-in-a-cohort-of-patients-with-primary-sjogrens-syndrome/