Methods Case Report: A 17-year old Turkish boy was admitted to our clinic with complaints of weight loss, fatigue and abdominal pain. He had been on colchicine therapy for 6 years with the diagnosis of FMF. On January 2000, he was diagnosed as having acute hepatitis A infection in another centre. Two weeks later he was hospitalised due to severe abdominal pain and abrupt decrease in his haemoglobin values. A right renal subcapsular hematoma was demonstrated by computerised tomography (CT). During follow-up haemoglobin values progressively declined and the hematoma showed enlargement in control CT. Therefore right nephrectomy was performed. After the patient was stabilised he was discharged. However he was again hospitalised with similar complaints 4 days later. This time subcapsular hematoma was demonstrated in the left kidney and he was referred to our hospital in March 2000. In physical examination, blood pressure was 160/125 mmHg. Laboratory evaluation demonstrated anaemia and elevated erythrocyte sedimentation rate and C-reactive protein. Serological tests for anti-hepatitis B surface antigen, anti-hepatitis A IgG and IgM were positive.
Nephrectomy slides were re-evaluated in our pathology department. Patchy and segmental areas of renal infarct were demonstrated. No amyloid deposits could be noted in the histopathology specimens. Renal angiography demonstrated 2 microaneurisms on intertubular arteries. Together with these findings the diagnosis of PAN was made and the patients was treated with intravenous corticosteroids, intravenous cyclophosphomide and oral corticosteroids together with antihypertensive therapy. He is still being followed-up by our clinic in good health.
Conclusion Perirenal hematoma, which is a rare complication of isolated PAN, have been reported in approximately half of the patients with FMF who developed PAN. The exact pathogenetic relationship of FMF and PAN also is not clear. Immune-related mechanisms have been demonstrated in FMF. It has been proposed previously that infection-related antigenic stimuli could trigger the development of vasculitides. In the present case an usual response to hepatitis A infection and/or previous hepatitis B infection might have been among the triggers of vasculitic process. Additional reports demonstrating direct causal relationship between infectious agents and vasculitic syndromes in patients with FMF will help us to clarify the relationship between the two disorders.
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