Lege Artis Medicinae

[Idiopathic retroperitoneal fibrosis (Morbus Ormond) associated with severe cardiomyopathy in a young man: a case report]

KOVÁCS Erzsébet, ZEHER Margit, SZÁNTÓ János, PÁSZTOR Éva, TÓTH László, PFLIEGLER György

JULY 20, 2012

Lege Artis Medicinae - 2012;22(06-07)

[INTRODUCTION - The cause of idiopathic retroperitoneal fibrosis is unknown, but probably involves a chronic, nonspecific, autoimmune inflammation. This rare disease mostly affects middle-aged or elderly people, it hardly ever occurs in young people. It causes obstruction of the urinary tract, postrenal insufficiency as well as functional disorder of vital organs in the retroperitoneum and its surrounding area. The diagnosis is based on diagnostic imaging and histological examinations. Surgical treatment of the urinary obstruction is only efficient in the long-term if combined with immunomodulatory treatment. CASE REPORT - A young, 34-year-old man who presented with oedema in his legs was diagnosed with retroperitoneal fibrosis. Autoimmune serology was negative. He needed temporary nephrostomy on the right side. Treatment with steroid and colchicine was initially successful. His severe cardiomyopathy improved gradually. No regression occurred with tamoxifen, so we started to administer azathioprine with methylprednisolone. The control abdominal CT showed stagnation of the process. CONCLUSIONS - Because of the rarity of this disease, no randomised controlled trials are available regarding its treatment. The aim of treatment is to eradicate the symptoms, solve the urinary obstruction, maintain renal function and prevent progression of the disease. Improvement may be achieved by corticosteroids, cyclophosphamide, azathioprine, leflunomide, mycophenolate mofetil and tamoxifen. To our knowledge, this case is unique as no similar case with severe cardiomyopathy and idiopathic retroperitoneal fibrosis has been reported in the literature previously.]

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