Clinical Characteristics of Distinct Subgroups of Patients with Primary Sjögren’s Syndrome, Classified according to Serological Profiles and the Presence of Cutaneous Vasculitis; a Comparative Study

This study aimed to determine the specific characteristics of extraglandular manifestations especially cutaneous involvement in patients with primary sjögren’s syndrome(pSS), focusing on the different clinical and histologic types of SS-associated vasculitis. A retrospective study was conducted and 402 patients (378 women and 24 men) with pSS were analyzed. Patients were categorized into three subgroups based on serological tests. These; 1) quadruple seropositive (positive for anti-Sjögren’s-syndrome-related antigen A antibodies (anti-SSA; anti-Ro) and anti-Sjögren’s-syndrome-related antigen B antibodies (anti-SSB; anti-La), rheumatoid factor (RF), and antinuclear antibody (ANA)), 2) double seropositive (positive for ANA and anti-SSA/Ro antibodies), 3) quadruple seronegative (negative for ANA, RF, anti-SSA/Ro and anti-SSB/La antibodies). The number of quadruple seropositive patients was 72 (18.6%), double seropositive 174 (43.2%), and quadruple seronegative was 85 (21.1%). The diagnosis age of quadruple seropositive pSS was 42.4±10.8, significantly younger than patients with double seropositive and quadruple seronegative pSS (p = 0.021, p=0.112). In terms of organ involvement, salivary gland enlargement, arthralgias, arthritis, Raynaud's phenomenon, lymphadenopathy, vasculitis-purpura, interstitial lung disease, neurological involvement, autoimmune thyroiditis, renal interstitial disease, anemia, leukopenia, hypergammaglobulinaemia and hypocomplementaemia were more common in quadruple seropositive pSS compared to quadruple seronegative (p