Background:Cryoglobulinemic vasculitis (CV) is a serious complication of Sjögren’s syndrome (SS) and is closely associated with type II IgMk cryoglobulins. CV has been well documented in HCV patients without SS, and shares common features with CV in SS. So far, few studies have described the clinical picture of CV in HCV negative SS, but the number of studied patients was rather small and CV was not well defined. To better describe the clinical spectrum of CV in SS and explore the differences compared to HCV-related CV, a large cohort of well characterized patients is required.Objectives:To study the clinical phenotype of CV in HCV-negative SS patients, in a large cohort of well characterized patients, after applying stringent classification CV criteria and in comparison with the classical CV of HCV patients.Methods:From a total cohort of 1997 consecutive SS patients who fulfill the 2016 EULAR/ACR criteria and are followed up in 5 clinical centers ([Universities ofUdine,Pisa andAthens,Harokopio andIoannina, (UPAHI)], those who fulfil the 2011 classification criteria for CV were identified and compared with matched HCV-CV patients according to age and gender. Glandular, extra-glandular manifestations and serologic features were compared between the 2 CV groups. Statistical analysis for categorical variables was performed by Fisher exact or chi-square tests and for continuous variables with t test or Mann-Whitney accordingly.Results:Among the 1083 SS patients who have been evaluated for cryoglobulins, 114 (9,8%) were found positive. Seventy-one (6,5%) SS patients met the 2011 CV criteria while 44 patients presented with type II IgMk cryoglobulinemia without CV. Sixty nine of 71 (97%) SS related CV patients were females and 2 of 71 (3%) males. Forty eight of 71 (68%) had SS disease onset >35 and <65 years old while 14/71 (19,7%) and 9/71 (12,7%) had SS disease onset <35 and >65 years old respectively. The most common clinical manifestations of CV among SS patients were purpura (90%) followed by arthralgias (70%), fatigue (59%), Raynaud’s phenomenon (48%), lymphadenopathy (31%), peripheral neuropathy (22,3%), vasculitic ulcer (11,3%) and glomerulonephritis (11,3%). Interestingly, almost 50% of SS related CV patients developed lymphoma and displayed high frequency of strong predictors including purpura, low C4 complement (88,6%) and salivary gland enlargement (SGE). Compared to HCV-CV patients, SS-CV patients had higher frequency of sicca manifestations, SGE, fatigue, arthritis, Raynaud’s phenomenon, lymphadenopathy, type II IgMk cryoglobulins and lymphoma.Conclusion:The prevalence of cryoglobulinemia and CV among SS patients is about 10% and 6-7% respectively. SS-CV patients are mainly middle-aged females with purpura as the main clinical manifestation, and up to one half of them may develop lymphoma, which is rarer in HCV-CV. Compared to HCV-CV patients, SS patients with CV have more frequently sicca symptoms, SGE and type II IgMk cryoglobulins.Disclosure of Interests:Ourania Argyropoulou: None declared, Vasileios Pezoulas: None declared, Luca Quartuccio: None declared, Francesco Ferro: None declared, Saviana Gandolfo: None declared, Valentina Donati: None declared, Aliki Venetsanopoulou: None declared, Loukas Chatzis: None declared, Evangelia Zampeli: None declared, Maria Mavromati: None declared, Paraskevi Voulgari: None declared, Clio Mavragani: None declared, Chiara Baldini: None declared, Fotini Skopouli: None declared, Dimitris Fotiadis: None declared, Massimo Galli: None declared, Salvatore De Vita Consultant of: Roche, Human Genome Science, Glaxo Smith Kline and Novartis, Haralampos M. Moutsopoulos: None declared, Andreas Goules: None declared, Athanasios Tzioufas: None declared
Clinical spectrum and genetic variations of LMNA-related muscular dystrophies in a large cohort of Chinese patients
