Familial Mediterranean fever (FMF) and Sjögren's syndrome: A case responding to azathioprine treatment

Sjögren sendromu (SS), kronik, otoimmün, sistemik ekzokrinopatidir ve ekstarglandüler bulgularhastalığın seyrinde görülebilir. Bazı hastalarda nadirende olsa vaskülitik cilt lezyonlarıgörülebilmektedir. Ailevi Akdeniz ateşi (AAA), ailevi otoinflamatuvar bir hastalık olup daha çokgenç yaştaki bireylerde tekrarlayan, kendi kendini sınırlayan ataklarla karakterize ve tedavisindedaha çok kolşisin ile yanıt alınan bir hastalıktır. Fakat bazı vakalarda kolşisine direnç olmaktadır.AAA ve SS birlikteği nadir olup özellikle dirençli-AAA tedavisinde azatioprin (AZA) kullanımıda henüz tartışmalıdır. Biz burada AAA ve vaskülitin eşlik ettiği SSlu AZA ile tedavi edilenkolşisin dirençli hastada ortaya çıkan anlamlı yanıtı sunmak istedik.

Ailevi Akdeniz Ateşi (AAA) ve Sjögren Sendromu (SS): Azatioprin tedavisine yanıt veren bir olgu

Sjögren s syndrome (SS) is a chronic, autoimmune, systemic exocrinopathy and extraglandularmanifestations can be seen in the course of the disease. Vasculitic skin lesions can rarely be seenin patients. Familial Mediterranean fever (FMF) is a familial, recurrent, autoinflammatory diseasewhich is seen mostly in young people and characterized by self-limiting attacks responding tocolchicine treatment. However, resistance to colchicine might be seen in some cases. Coexistenceof FMF and SS is rare and the use of azathioprine (AZA) for the treatment of resistant FMF is yetcontroversial. Herein, we aim to share the post-treatment positive findings of a colchicine-resistantpatient diagnosed with FMF and SS comorbiding vasculitis treated with AZA.

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