A case of familial Mediterranean fever and polyarteritis nodosa complicated by spontaneous perirenal and subcapsular hepatic hemorrhage requiring multiple arterial embolizations

Rheumatology International
Servet AkarNurullah Akkoc

Abstract

The association of familial Mediterranean fever (FMF) and polyarteritis nodosa (PAN) has been well established. These patients have been reported to have an overall better prognosis than other PAN patients. Herein we report a patient with FMF and PAN who died of sepsis following a severe course of recurrent bleeding episodes which required multiple embolization attempts. The 39-year-old Turkish male presented with abdominal pain of 1-month duration. He had been diagnosed with FMF at the age of 24. On admission, he had pallor with general ill appearance. Rebound tenderness was obtained in the right upper abdominal quadrant. He had mild anemia, leukocytosis, thrombocytosis, and hypoalbuminemia. On the 2nd day of his admission, he developed hypotension with a rapid decline in hemoglobin level. Abdominal angiography showed multiple aneurysms in the branches of renal arteries, superior mesenteric artery, and hepatic arterial system including left renal infarct, suggesting PAN. He was put on high-dose steroids and oral cyclophosphamide. Despite medical treatment, he developed intense abdominal pain, hypotension, tachycardia, and a rapid fall in hemoglobin on four occasions. Active bleeding sites were embolized in two different angiog...Continue Reading

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Jun 22, 2006·International Journal of Hematology·Tadashi MatsushitaTomoki Naoe
May 25, 2006·Clinical Rheumatology·Alexandra Balbir-GurmanYolanda Braun-Moscovici
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Related Concepts

Cyclophosphamide, (S)-Isomer
Polychemotherapy
Embolization, Therapeutic
Hemorrhage
Kidney Diseases
Liver Dysfunction
Urbason
Polyarteritis Nodosa
Familial Mediterranean Fever
Predonine

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