Clinical and serological association between sjogren syndrome and vasculitis

BackgroundSjögren Syndrome (SS) is a systemic autoimmune systemic disease which mainly affects the exocrine glands, presenting mainly as persistent eye and mouth dryness as a result of a functional impairment of the lacrimal and saliva glands. Cutaneous vasculitis is one of the most characteristic extraglandular manifestations, especially in patients with anti-Ro antibodies. Although vasculitis may affect a wide range of organs and systems, the clinical characteristics and the diversity of vasculitis associated with SS have not been thoroughly studied.ObjectivesTo describe the clinical and analytical characteristics of patients with SS and cutaneous, or systemic vasculitis, as well as their outcome.MethodsRetrospective, descriptive study, of patients with primary SS and vasculitis attended at a Tertiary Hospital. Collected data were analysed using the SPSS statistical package. Quantitative data were expressed as a mean +/- 2SD and qualitative data as a proportion. A p-value 0.05 was considered significant.ResultsFrom a total of 395 patients with primary SS, 53 patients presented with both SS and vasculitis (51, 96.2%, women). The mean age at diagnosis was 56.12 (±2.17) years. Globally, 84.9% of patients met the 2002 classification criteria; 90.6% the 2012 criteria, and 83.0% the 2016 criteria. Thirty-two (60.4%) patients presented with cutaneous vasculitis (mainly purpura), and 7 (13.2%) with systemic vasculitis with renal, neurological and/or lung involvement. Extraglandular manifestations were frequent. Out of the 53 patients, twenty-five (47.2%) presented pulmonary involvement; 43(81.1%) articular involvement (19 [35.8%] arthritis); 21(39.6%) Raynaud phenomenon, and 15.1% peripheral neuropathy. Six (11.3%) patients developed a lymphoma. All patients presented positive ANA; 69.8% anti-Ro60, 52.8% anti-La, 75.5% RF; 62.3% polyclonal hypergammaglobulinemia; 33.9% cryoglobulins, 58.5% elevated beta2-microglobulin levels, and 12 (22.65%) hypocomplementemia (4 decreased C3, and 8 decreased C4). Biopsy specimens showed in 8 (15.9%) cases urticaria vasculitis; in 1 (1.9%) PAN; in 2 (3.8%) Cryoglobulinaemic vasculitis, in 5 (9.4%) ANCA associated vasculitis (4 PAM-MPO, and 1 GPA-PR3); and in 1 SNC vasculitis. 64.2% of patients received glucocorticoids and 37.7% hydroxychloroquine. 5 (9.4%) patients received Rituximab. Vasculitis were significantly more frequent in patients with anti-Ro60 (p=0.005, OR2.5 IC95% 1.33-4.74); anti-La (p=0.009, OR 2.39, IC95% 1.29-4.43); RF (p<0.000, OR 3.78, OR 3.72 IC95%1.90-7.26); C4 hypocomplementemia (p<0.00, OR 6.42, IC 95% 2.65-15.56); cryoglobulins (p=0.016, OR 2.29, IC95% 1.21-4.33); hypergammaglobulinemia (p=0.004, OR 2.53, IC95% 1.36-4.71), arthritis (p= 0.001, OR 3.26, IC95% 1.72-6.19), lung fibrosis (p=0.010, OR 2.92, IC95% 1.38-6.17) and lymphoma (p=0.011, OR 4.05, IC95% 1.5-10.94).ConclusionIn our series, most patients with SS presented repetitive flares of cutaneous vasculitis as previously reported. Nonetheless, our patients showed a greater proportion of lung and articular manifestations as well as anti-La positivity.Five patients presented with ANCA associated vasculitis, which represents and exceedingly rare and severe manifestation in this context.Patients with SS may present a wide range of vasculitis, ranging from the most common leucocytoclastic vasculitis to the more severe systemic vasculitis, such as: AAV and cryoglobulinaemic vasculitis.The association of SS with vasculitis is more common in patients with anti-Ro, anti-La and/or RF, hypocomplementemia, cryoglobulins and hypergammaglobulinemia.References[1]Ramos-Casals, M., Anaya, J., García-Carrasco, M., Rosas, J., Bové, A., Claver, G., Diaz, L., Herrero,[2]C. and Font, J., 2004. Cutaneous Vasculitis in Primary Sjögren Syndrome. Medicine, 83(2), pp.96-106.Disclosure of InterestsNone declared