Igg4 Related Retroperitoneal Fibrosis C0035357
A condition causing inflammation and scarring behind the abdominal cavity. It may affect the ureters or cause back pain. Treatment typically involves corticosteroids or immunosuppressive medications.
Key facts at a glance
- ICD-10
- R69
- ICD-11
- MA00.0
- Body system
- other
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(other)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Hydronephrosis
(renal)Abdominal pain
(gastrointestinal)Flank pain
(musculoskeletal)Weight loss
(general)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Corticosteroid therapy
Systemic corticosteroids serve as first-line treatment to reduce retroperitoneal inflammation and fibrosis, potentially reversing ureteral obstruction. Initial high doses are followed by gradual taper.
Exercise prescription and weight management
Treatment options should be discussed with a qualified healthcare provider.
Ureteral stenting or percutaneous nephrostomy
Urgent intervention to relieve obstructive uropathy and preserve renal function when retroperitoneal fibrosis causes significant ureteral obstruction with hydronephrosis.
Additional treatment options
Steroid-sparing immunosuppressants
Maintenance therapy with agents such as mycophenolate mofetil, azathioprine, or methotrexate to maintain remission and reduce cumulative corticosteroid exposure.
medicationUreterolysis with intraperitoneal transposition
Surgical separation of ureters from inflammatory tissue with placement in intraperitoneal position. Considered for refractory cases or when tissue diagnosis is needed.
surgicalSerial imaging and renal function monitoring
Regular CT or MRI imaging combined with serum creatinine monitoring to assess treatment response and detect recurrent fibrosis or obstruction.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
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Frequently asked questions
Common questions about this topic.
What is Igg4 Related Retroperitoneal Fibrosis C0035357?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy