The Efficacy of Interferon-alpha in a Patient with Resistant Familial Mediterranean Fever Complicated by Polyarteritis Nodosa

Abstract

Familial Mediterranean fever (FMF) is a recurrent self-limiting polyserositis. Polyarteritis nodosa (PAN) complicating FMF is very rare. Here, we present a 17-year-old male patient with FMF who subsequently developed PAN 2 weeks after hepatitis A infection. This case was also complicated with perirenal haematoma, and right nephrectomy was performed. The clinical condition of the patient was improved after therapy with intravenous and oral corticosteroid and intravenous cyclophosphamide. However, the FMF attacks and vasculitic skin lesions again occurred while he was using colchicine plus immunosuppressive agents a few months later. Interferon-alpha therapy was administered and the attacks were resolved within 3 months. He has not experienced any other symptom during the follow-up period of 28 months. (Internal Medicine 43: 612-614, 2004)

Dysregulation of innate immunity: hereditary periodic fever syndromes

“…If colchicine is ineffective this is usually due to non‐compliance, only 5% of patients are truly colchicine resistant. Both failures and successes have been reported in patients on combined colchicine and interferon‐α therapy (Tunca et al , 1997, 2004; Calguneri et al , 2004a,b). In some patients colchicine therapy has effectively been combined with infliximab ( n = 8), etanercept ( n = 2) and anakinra ( n = 2) (Aldea et al , 2004; Metyas et al , 2004; Daysal et al , 2005; Chae et al , 2006; Ozgocmen et al , 2006; Sakallioglu et al , 2006; Yuksel et al , 2006; Belkhir et al , 2007; Mor et al , 2007).…”

“…In some patients colchicine therapy has effectively been combined with infliximab ( n = 8), etanercept ( n = 2) and anakinra ( n = 2) (Aldea et al , 2004; Metyas et al , 2004; Daysal et al , 2005; Chae et al , 2006; Ozgocmen et al , 2006; Sakallioglu et al , 2006; Yuksel et al , 2006; Belkhir et al , 2007; Mor et al , 2007). Persistent febrile myalgia caused by vasculitis responds to systemic corticosteroids (Calguneri et al , 2004a; Gdynia et al , 2006; Soylu et al , 2006; Mor et al , 2007).…”

Polyarteritis nodosa and Henoch–Schönlein purpura nephritis in a child with familial Mediterranean fever: a case report

“…In life-threatening cerebral vasculitis or rapidly deteriorating renal function, pulse administration of cyclophosphamide and corticosteroids should be favored. Cangüneri et al [24] presented a patient having colchicines, pulse methylprednisolone and cyclophosphamide, and redeveloped symptoms. They suggested that interferon may be a useful adjuvant for the treatment of resistant attacks in FMF patients with vasculitis.…”

Familial Mediterranean Fever

“…Tekrarlayan karın ağrısı atakları, tekrarlayan pankreatit, porfi ri ile karıştırılabilir. Plevral ataklar tekrarlayan pulmoner emboli, [65]. Daha önceki gözlemlere dayanılarak AAA amiloidozuna bağlı son dönem böbrek yetmezliği olan hastalarda sürekli ayaktan periton diyalizinin abdominal atakları artırdığı ileri sürüldü [66].…”