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Retroperitoneal fibrosis

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Steven C. Campbell, M.D., Ph.D.

Synonyms and keywords: Ormond’s disease; fibrosis, inflammatory perianeurysmal; fibrosis, perianeurysmal inflammatory; inflammatory fibrosis, perianeurysmal; inflammatory perianeurysmal fibrosis; Ormond disease; perianuerysmal fibrosis, inflammatory; perianeurysmal inflammatory fibrosis; periaortitis, chronic

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Retroperitoneal fibrosis or Ormond’s disease is a disease featuring the proliferation of fibrous tissue in the retroperitoneum, the compartment of the body containing the kidneys, aorta, renal tract and various other structures. It may present with lower back pain, renal failure, hypertension, deep vein thrombosis and other obstructive symptoms.

References

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Pathophysiology

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Causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Causes

Its association with various immune-related conditions and response to immunosuppression have led to speculations as to the autoimmune etiology of idiopathic RPF. One-third of the cases are secondary to malignancy, medication (methysergide, hydralazine, beta blockers), aortic aneurysm or certain infections.

Life Threatening Causes

Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated.

Common Causes

Causes by Organ System

Cardiovascular No underlying causes
Chemical/Poisoning No underlying causes
Dental No underlying causes
Dermatologic No underlying causes
Drug Side Effect Bromocriptine, Ergotamine tartrate, Rotigotine
Ear Nose Throat No underlying causes
Endocrine No underlying causes
Environmental No underlying causes
Gastroenterologic No underlying causes
Genetic No underlying causes
Hematologic No underlying causes
Iatrogenic No underlying causes
Infectious Disease No underlying causes
Musculoskeletal/Orthopedic No underlying causes
Neurologic No underlying causes
Nutritional/Metabolic No underlying causes
Obstetric/Gynecologic No underlying causes
Oncologic No underlying causes
Ophthalmologic No underlying causes
Overdose/Toxicity No underlying causes
Psychiatric No underlying causes
Pulmonary No underlying causes
Renal/Electrolyte No underlying causes
Rheumatology/Immunology/Allergy No underlying causes
Sexual No underlying causes
Trauma No underlying causes
Urologic No underlying causes
Miscellaneous No underlying causes

Causes in Alphabetical Order

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Differentiating Retroperitoneal fibrosis from other Diseases

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Epidemiology and Demographics

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Natural History, Complications and Prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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Overview

Natural History

Complications

The disorder may lead to:

Prognosis

Prognosis depends on the extent of the fibrosis and the amount of damage to the kidneys. The kidney damage may be temporary or permanent.

References

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Diagnosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | CT | MRI | Other Imaging Findings | Other Diagnostic Studies

Treatment

Treatment

Medical Therapy | Surgery | Primary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case Studies

Case #1
External links
  • van Bommel EF. Retroperitoneal fibrosis. Neth J Med 2002;60:231-42. PMID 12365466.
  • Ormond JK. Bilateral ureteral obstruction due to envelopment and compression by an inflammatory retroperitoneal process. J Urol 1948;59:1072-79.
  • Ormond JK. Idiopathic retroperitoneal fibrosis: a discussion of the etiology. J Urol 1965;94:385-90.

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