Clinical features of primary Sjögren syndrome with purpura

Abstract

Objective: To study the clinical characteristics of patients with primary Sjögren syndrome(pSS) with purpura. Methods: A total of 101 patients with pSS were enrolled from January 2017 through January 2020 in Department of Dermatology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine. The patients were divided into 2 groups: pSS with purpura (purpura group, n=15) and without purpura (non-purpura group, n=86). The clinical presentations and laboratory data were compared between the 2 groups. Results: Of the 15 patients with manifestation of purpura (14.9%), 12(80%) were diagnosed as hypergammaglobulinemia purpura and the other 3(20%) were cryoglobulinemia purpura, thrombocytopenic purpura, and pigmented purpuric dermatosis, respectively. Four cases developed purpura prior to the glandular symptoms of dry mouth and eyes, and 11 cases had purpura after presentations of glandular symptoms. Com-paring with the non-purpura group, the pSS patients with purpura group had earlier onset age [(41.6±13.5) years vs. (51.7±11.4) years, P=0.003], higher incidence of lymphoma (2/15 vs. 0, P=0.011); higher serum levels of rheumatoid factor (RF) (P=0.002), erythrocyte sedimentation rate (ESR) (P=0.019), immunoglobulin (Ig) G (P<0.001), γ-globulin (P=0.001), free Ig light (κ) chains (P<0.001), free Ig heavy (λ) chains (P=0.015), and lower levels of compliment components C3 (P=0.011) and C4 (P=0.021). Conclusions: As a common and heterogeneous dermatological manifestation of pSS patients revealed in this study, the diagnosis of pSS should be considered in patients presenting purpura. The pSS patients with purpura with early onset and abnormal laboratory parameters have increased risk of lymphoma and demanded further monitoring.

Keywords

• |primary sj&#x000f6;gren syndrome|cutaneous manifestation|purpura|lymphoma