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
Pathophysiology
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References
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
References
Differentiating Retroperitoneal fibrosis from other Diseases
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References
Epidemiology and Demographics
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References
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:
- Chronic bilateral obstructive uropathy
- Chronic kidney failure
- Chronic unilateral obstructive uropathy
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
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
External links
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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