Multiple visceral hematomas in a child with familial Mediterranean fever: answer

Excerpt

This patient with high-grade fever and multiple hematomas was initially suggested to have a bleeding disorder or an infectious disease. Normal bleeding test results, negative culture findings and serological tests for various bacterial or viral infectious agents excluded these possibilities. The most common etiology of spontaneous renal and hepatic hemorrhage is benign or malignant neoplasms of kidney and liver. Concurrent occurrence of multiple hematomas in both kidneys and liver was hardly suggestive of malignancy. Moreover, radiological and hematological findings did not reveal any malign process. Vascular diseases including arteriovenous malformation, aneurysm, infarction and vasculitis, such as polyarteritis nodosa (PAN) and Wegener’s granulomatosis, were the next most common diseases leading to hemorrhage. Especially if bilateral hemorrhage is detected, clinicians must be aware of vasculitis. Because of the clinical presentation and multiple intraabdominal hematomas, our patient was suggested to have vascular disease, and following investigations revealed that: serum complement 3 (C3) level was slightly decreased, complement 4 (C4) level was normal. Antinuclear antibody, anti DNA, p- and c-anti-neutrophil cytoplasmic antibodies (ANCAs), lupus anticoagulants were absent. Electromyography demonstrated myopathic changes, but muscle biopsy failed to show any evidence of vasculitis or another pathology. Renal selective angiography showed multiple aneurysms in bilateral renal arteries, leading to the diagnosis of PAN (Fig. 1), since small aneurysms involving the renal, celiac, or mesenteric arteries are characteristic of PAN [1].

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