[Vasculopathy in Sjögren's syndrome].
Sjögren's syndrome is a systemic autoimmune disease with a predominant involvement of exocrine glands leading to sicca symptoms. Extraglandular involvement occurs in about 40% of patients with skin, musculoskeletal, neurological and organ manifestations. Systemic vasculitic manifestations of Sjögren's syndrome can be assumed in approximately 5%-10% of patients. Leukocytoclastic or cryoglobulinemic vasculitis represent classic vasculitic manifestations of Sjögren's syndrome. In the pathogenesis of vasculitis, B-cell-driven autoimmune processes play a major role by producing autoantibodies against the Ro/SS-A and La/SS-B antigens and cryoglobulins. In patients with Sjögren's syndrome, manifestation of vasculitis, non-Hodgkin's lymphoma and glomerulonephritis, as well as positive cryoglobulins and decreased levels of complement factors, are considered negative prognostic markers. Various immunosuppressive strategies, usually in co-medication with glucocorticoids, are used for the treatment of vasculitis in Sjögren's syndrome. For refractory and severe manifestations, a B-cell-targeted therapy with Rituximab should be also considered.
Feist
E
E
Klinik für Rheumatologie und Klinische Immunologie, Charité-Universitätsmedizin Berlin, Charitéplatz 1, Berlin, Germany. eugen.feist@charite.de
Hermann
K-G A
KG
Dankof
A
A
D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D012859:Sjogren's Syndrome / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D014657:Vasculitis / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*
D007166:Immunosuppressive Agents