The Association of Familial Mediterranean Fever and Polyarteritis Nodosa: A Case Report

Abstract

Introduction: Familial Mediterranean fever (FMF) is the most common type of periodic fever syndromes. It is an autosomal recessive disorder characterized by acute, self-limited episodes of fever and polyserositis recurring at irregular intervals. Vasculitis has been frequently reported in patients with familial Mediterranean fever. The association of FMF and polyarteritis nodosa has been well established. Clinical presentation of polyarteritis nodosa in patients with familial Mediterranean fever has certain characteristics and it may be a feature of FMF itself. Herein, we report on a case of familial Mediterranean fever accompanied by polyarteritis nodosa with hepatic, renal and gastroinestinal involvement. Case Presentation: A ten-year-old Iranian boy was referred to our department with history of recurrent abdominal pain followed by fever, chills, arthralgia and scrotal edema. He suffered from hematuria and gastrointestinal bleeding. His physical exam revealed fever (axillary temperature: 38.7°C), hypertension (150/90 mmHg), hepatomegaly (liver span: 13 cm), orchitis and subcutaneous painful nodules of both legs and arthritis of both shoulders and right ankle. Conclusions: Mutations in MEFV gene provide a basis for the development of PAN both by forming a pro-inflammatory state and by resulting exaggerated response to infection.