Harun PERU, Necla BUYAN, Enver HASANOĞLU, Baran ÖNAL, Bakkaloğlu Sevcan EZGÜ, Kibriya FİDAN, Yılmaz Sebahat AĞLADIOĞLU

Ailevi Akdeniz Ateşi ve poliarteritis nodoza birlikteliği olan pediatrik bir olgu sunumu

Ailevi Akdeniz atesi (FMF), tekrarlayan ve sıklıkla kendini sınırlayan ates, seröz yüzeylerde inflamasyon ve bu nedenle olusan karın, göğüs ve eklem ağrısı ile seyreden otozomal resesif geçis gösteren bir hastalıktır. FMF’li hastalarda, poliarteritis nodoza (PAN) ve Henoch- Schönlein purpurası (HSP) gibi vaskülitik hastalıkların sıklığının arttığı bilinmektedir. Patogenez açık olmamakla birlikte immünkompleks mekanizma ile iliskili olabilir. Bu yazıda karın ağrısı ve ates sikayetiyle basvuran ve FMF tanısı ile kolsisin tedavisi baslandıktan sonra, izlemde kontrol altına alınamayan semptomları ve yüksek akut faz yanıtı nedeni ile eslik eden vaskülitik hastalık düsünülerek yapılan angiografikincelemede izole hepatik arter anevrizması tespit edilen 9 yasında erkek hasta sunulmaktadır. Bu hasta, FMF ve PAN’ın nadir olan birlikteliğinin yanı sıra, renal ve çölyak arterlerde tutulum olmaksızın izole hepatik arter tutulumunun olabileceği ve immun supresif tedaviye eklenen pulse siklofosfamid tedavisi ile alınan yanıt vurgulamak amacı ile sunulmaktadır.

Polyarteritis nodosa in a case of familial mediterranean fever

Familial Mediterranean fever (FMF) is an autosomal recessive disease, characterized by recurrent and self-limited attacks of fever, usually accompanied by polyserositis. Several vasculitic diseases such as Henoch-Schönlein purpura and polyarteritis nodosa (PAN) have been reported with increased frequency in patients with FMF. Although the pathogenesis is not clearly defined, it has been hypothesized that immune complexes may play a role in the association of the vasculitides and FMF. In this report we describe a 9-year-old boy admitted to our hospital with abdominal pain and fever. FMF was detected and colchicine treatment was given. Despite the treatment, his constitutional symptoms persisted. Due to the increased likelihood for the development of a vasculitic process in patients with FMF, angiography was performed because of a suspicion of PAN and angiography showed hepatic artery aneurysms. He responded well to pulse methylprednisolone and pulse cyclophosphamide therapy in addition to colchicine. We emphasize the rare association of FMF and PAN and insulated hepatic artery aneurysmal angiographic signs of PAN.

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