Idiopathiás retroperitonealis fibrosis (Ormond-kór) társulása súlyos cardiomyopathiával: Fiatal férfi betegünk esetbemutatása

Translated title of the contribution: Idiopathic retroperitoneal fibrosis (morbus Ormond) associated with severe cardiomyopathy in a young man: A case report

Erzsébet Kovács, M. Zeher, J. Szántó, Éva Pásztor, L. Tóth, György Pfliegler

Research output: Contribution to journalArticle

Abstract

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.

Original languageHungarian
Pages (from-to)417-422
Number of pages6
JournalLege Artis Medicinae
Volume22
Issue number6-7
Publication statusPublished - 2012

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Retroperitoneal Fibrosis
Cardiomyopathies
leflunomide
Azathioprine
Tamoxifen
Mycophenolic Acid
Therapeutics
Methylprednisolone
Colchicine
Serology
Diagnostic Imaging
Rare Diseases
Urinary Tract
Cyclophosphamide
Disease Progression
Edema
Leg
Adrenal Cortex Hormones
Randomized Controlled Trials
Steroids

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Idiopathiás retroperitonealis fibrosis (Ormond-kór) társulása súlyos cardiomyopathiával : Fiatal férfi betegünk esetbemutatása. / Kovács, Erzsébet; Zeher, M.; Szántó, J.; Pásztor, Éva; Tóth, L.; Pfliegler, György.

In: Lege Artis Medicinae, Vol. 22, No. 6-7, 2012, p. 417-422.

Research output: Contribution to journalArticle

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AB - 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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