Retroperitoneal fibrosis

Información en español Title

Other Names:

Idiopathic retroperitoneal fibrosis; Ormond's disease; IgG4-related retroperitoneal fibrosis; Idiopathic retroperitoneal fibrosis; Ormond's disease; IgG4-related retroperitoneal fibrosis; Ormond disease See More

Categories:

Digestive Diseases; Kidney and Urinary Diseases

Summary Summary

Retroperitoneal fibrosis is a slowly progressive disorder in which the tubes that carry urine from the kidneys to the bladder (ureters) and other abdominal organs or vessels become blocked by a fibrous mass and inflammation in the back of the abdomen. The disorder may cause pain in the abdomen that worsens with time, pain or swelling of the legs, decreased urine output, and swelling of the scrotum in men.^[1]^[2] Risk factors for retroperitoneal fibrosis include asbestos exposure, smoking, tumor, infection, trauma, radiotherapy, surgery, and use of certain drugs.^[1]^[2]^[3]^[4]Treatment may include corticosteroids, tamoxifen, stents or surgery.^[1]

Last updated: 6/20/2016

Symptoms Symptoms

Retroperitoneal fibrosis is characterized by inflammation and excessive scar tissue that develops in the back of the abdominal cavity. It can occur at any age, but appears most often between the ages of 40 and 60.^[1]^[2] Men are twice as likely to develop the condition as women.^[1]

Early symptoms of retroperitoneal fibrosis may include:^[1]^[2]

Dull pain in the abdomen, back, or side that increases with time
Decreased circulation in the legs leading to pain, swelling, and discoloration
Impaired blood flow to the intestines leading to death (necrosis) of intestinal tissue, severe pain, and excessive bleeding (Hemorrhage)
Swelling of the scrotum in men

Late symptoms of retroperitoneal fibrosis may include:^[1]^[2]

Decreased urine output
Total lack of urine (anuria)
Nausea, vomiting, weight loss, itching, anemia, and changes in thinking caused by kidney failure and the resulting build-up of toxic chemicals in the blood.

Last updated: 6/20/2016

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms	Other Names	Learn More: HPO ID
30%-79% of people have these symptoms
Abdominal pain	Pain in stomach Stomach pain [ more ]	0002027
Anorexia		0002039
Anti-thyroid peroxidase antibody positivity		0025379
Deep dermal perivascular inflammatory infiltrate		0031191
Elevated C-reactive protein level		0011227
Elevated erythrocyte sedimentation rate	High ESR	0003565
Elevated serum creatinine	Elevated creatinine High blood creatinine level Increased creatinine Increased serum creatinine [ more ]	0003259
Fatigue	Tired Tiredness [ more ]	0012378
Flank pain		0030157
Hydronephrosis		0000126
Increased blood urea nitrogen		0003138
Large vessel vasculitis		0005310
Low back pain		0003419
Normocytic anemia		0001897
Unilateral renal hypoplasia	Small kidney on one side Underdeveloped kidney on one side [ more ]	0012583
Weight loss		0001824
5%-29% of people have these symptoms
Acute kidney injury		0001919
Antineutrophil antibody positivity		0003453
Constipation		0002019
Fever		0001945
Hashimoto thyroiditis		0000872
Membranous nephropathy		0012578
Nausea and vomiting		0002017
Pedal edema	Fluid accumulation in lower limbs Lower leg swelling [ more ]	0010741
Psoriasiform dermatitis		0003765
Rheumatoid arthritis		0001370
Rheumatoid factor positive		0002923
Smooth muscle antibody positivity		0003262
Systemic lupus erythematosus		0002725
Ureteropelvic junction obstruction		0000074
1%-4% of people have these symptoms
Acute tubular necrosis		0008682
Budd-Chiari syndrome		0002639
Dysuria	Painful or difficult urination	0100518
Hematuria	Blood in urine	0000790
Hydrocele testis		0000034
Impotence	Difficulty getting a full erection Difficulty getting an erection Erectile dysfunction [ more ]	0000802
Nephrotic syndrome		0000100
Renovascular hypertension		0100817
Retrograde ejaculation		0012877
Varicocele		0012871

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Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 8/1/2019

Do you have updated information on this disease? We want to hear from you.

Cause Cause

The cause of retroperitoneal fibrosis is unknown in many cases (idiopathic).^[1]^[2] Occasionally, it may occur with autoimmune disorders and some researchers suggest that the immune system may be involved.^[2] Some cases occur in association with other factors, including:^[1]^[2]^[3]^[4]

Asbestos exposure
Smoking
Neoplasms (tumor)
Infections
Trauma
Radiotherapy
Surgery
Use of certain drugs (including those that contain methysergide, a medication sometimes used to treat migraines)

Some cases of idiopathic retroperitoneal fibrosis (IgG4-related retroperitoneal fibrosis) are considered part of a group of conditions known as “Immunoglobulin G4-related disease (IgG4-RD)”.

Last updated: 6/21/2016

Treatment Treatment

There is no consensus on the best treatment for retroperitoneal fibrosis. The approach may depend on the disease's stage.^[1]^[3]

Corticosteroids are tried first. Dosing will be prescribed on a case by case basis, but doses often vary between 30 and 60 mg per day. Corticosteroids are then tapered slowly. Some people with retroperitoneal fibrosis may continue on low dose maintenance therapy for up to 2 years.^[5] If corticosteroid treatment doesn't work, a biopsy should be done to confirm the diagnosis.^[1]

Other medicines to suppress the immune system, such as mycophenolate mofetil, methotrexate, azathioprine, cyclophosphamide or tamoxifen can be prescribed alone or in combination with corticosteroids.^[1]

When medication does not work, surgery and stents (draining tubes) are considered. Stents (drainage tubes) placed in the ureter or in the renal pelvis may provide short-term relief of the symptoms until the condition is surgically treated.^[3] Surgery aims to remove the mass and/or free the ureters.

In cases of severe inflammation of the kidneys (severe hydronephrosis) the initial treatment is done with surgery or stents. However, many professionals currently recommend surgery as the first option for patients with obstructed ureters who are in good overall health, and not as a last resort treatment.^[6]^[7] Some newer surgical techniques with fewer complications have shown better results, such as robotic laparoscopy^[8]

Patients with advanced age or health conditions that prevent a surgery are treated only with medication.^[9]

Last updated: 8/6/2016

Prognosis Prognosis

The outlook for patients with retroperitoneal fibrosis is usually considered to be good. Renal recovery frequently is observed within the first 2 weeks, but patients should always be checked periodically since disease recurrence has been reported up to 10 years after finishing the treatment. Obstruction of the ureter may recur in up to half of patients who had surgery alone and in about 10% of those treated with both surgery and medication.^[10] Severe complications such as chronic renal failure, requiring kidney transplant, can arise in rare cases.^[1]^[3]^[10] Other, less frequent complications, may include:^[7]

Hypertension
Scrotal swelling
Inferior vena cava thrombosis.

Last updated: 6/21/2016

Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are related to Retroperitoneal fibrosis. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Urology Care Foundation
1000 Corporate Boulevard
Linthicum, MD 21090
Toll-free: 1–800–828–7866
Telephone: 410–689–3700
Fax: 410-689-3998
E-mail: info@urologycarefoundation.org
Website: http://www.urologyhealth.org/index.cfm

Social Networking Websites

Retroperitoneal Fibrosis World Support
E-mail: retroperitoneal_fibrosisworldsupport@yahoogroups.com
Website: https://groups.yahoo.com/neo/groups/retroperitoneal_fibrosisworldsupport/info

Organizations Providing General Support

American Association of Kidney Patients
3505 E. Frontage Rd., Suite 315
Tampa, FL 33607-1796
Toll-free: 800-749-2257
Telephone: 813-636-8100
Fax: 813-636-8122
E-mail: info@aakp.org
Website: http://www.aakp.org
American Autoimmune Related Diseases Association (AARDA)
22100 Gratiot Avenue
Eastpointe, MI 48021
Toll-free: 800-598-4668
Telephone: 586-776-3900
Fax: 586-776-3903
E-mail: aarda@aarda.org
Website: https://www.aarda.org/
American Kidney Fund, Inc.
6110 Executive Boulevard
Suite 1010
Rockville, MD 20852
Toll-free: 866-300-2900
Telephone: 301-881-3052
E-mail: helpline@kidneyfund.org
Website: http://www.kidneyfund.org
National Kidney Foundation
30 East 33rd Street
New York, NY 10016
Toll-free: 800-622-9010
Telephone: 212-889-2210
Fax: 212-689-9261
E-mail: info@kidney.org
Website: https://www.kidney.org/
The Kidney Foundation of Canada
700-15 Gervais Drive
Toronto Ontario M3C 1Y8
Canada
Toll-free: 800-387-4474
Telephone: 416-445-0373
Fax: 416-445-7440
E-mail: kidney@kidney.on.ca
Website: http://www.kidney.on.ca

Do you know of an organization? We want to hear from you.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

Genetics Home Reference contains information on Retroperitoneal fibrosis. This website is maintained by the National Library of Medicine.
MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
PubMed is a searchable database of medical literature and lists journal articles that discuss Retroperitoneal fibrosis. Click on the link to view a sample search on this topic.

GARD Answers GARD Answers

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Have a question? Contact a GARD Information Specialist.

References References

Sobol J. Retroperitoneal fibrosis. MedlinePlus. June 29, 2015; http://www.nlm.nih.gov/medlineplus/ency/article/000463.htm.
Retroperitoneal fibrosis. Genetics Home Reference (GHR). July 2013; https://ghr.nlm.nih.gov/condition/retroperitoneal-fibrosis.
Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet. 2006 Jan 21; 367(9506):241-51. https://www.ncbi.nlm.nih.gov/pubmed/?term=16427494.
Goldoni M, Bonini S, Urban ML, Palmisano A, De Palma G, Galletti E, Coggiola M, Buzio C, Mutti A, Vaglio A. Asbestos and smoking as risk factors for idiopathic retroperitoneal fibrosis: a case-control study. Ann Intern Med. 2014 Aug 5; 161(3):181-8. http://www.ncbi.nlm.nih.gov/pubmed/25089862.
Tzou M, Gazeley DJ, Mason PJ. Retroperitoneal fibrosis. Vasc Med. 2014 Oct; 19(5):407-14. https://www.ncbi.nlm.nih.gov/pubmed/?term=25161213.
Fernando A, Pattison J, Horsfield C, Bultitude M, D'Cruz D & O'Brien T. A lot of questions and a few answers in retroperitoneal fibrosis. BJU Int. Janury 23, 2015; http://www.ncbi.nlm.nih.gov/pubmed/25614102.
Biyani CS. Retroperitoneal Fibrosis. Medscape Reference. 2016; http://emedicine.medscape.com/article/458501-overview.
Keehn AY, Mufarrij PW & Stifelman MD. Robotic ureterolysis for relief of ureteral obstruction from retroperitoneal fibrosis. Urology. June, 2011; 77(6):1370-4. http://www.ncbi.nlm.nih.gov/pubmed/21296397.
Retroperitoneal fibrosis. MedlinePlus. 2013; http://www.nlm.nih.gov/medlineplus/ency/article/000463.htm.
Ezimora A, Faulkner,ML, Adebiyi O, Ogungbemile A, Marianna SV & Nzerue C. Retroperitoneal Fibrosis: A Rare Cause of Acute Renal Failure. Case Reports in Nephrology. 2012; 2012:http://www.hindawi.com/journals/crin/2012/645407/cta/.