IgG4-related retroperitoneal fibrosis
MONDO:0018848Mondo
Findings
No curated finding names IgG4-related retroperitoneal fibrosis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Retroperitoneal fibrosis (RPF) is characterized by the development of a fibrotic mass surrounding retroperitoneal structures, such as aorta, vena cava, ureters and psoas muscle.
Definition from the Mondo Disease Ontology (MONDO:0018848), read 2026-09-29. CC BY 4.0.
Features
43 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- PainHPOHP:0012531
- Very frequent (80% to 99% of cases)
- Abdominal painHPOHP:0002027
- Frequent (30% to 79% of cases)
- AnorexiaHPOHP:0002039
- Frequent (30% to 79% of cases)
- Anti-thyroid peroxidase antibody positivityHPOHP:0025379
- Frequent (30% to 79% of cases)
- Deep dermal perivascular inflammatory infiltrateHPOHP:0031191
- Frequent (30% to 79% of cases)
- Elevated circulating C-reactive protein concentrationHPOHP:0011227
- Frequent (30% to 79% of cases)
- Elevated circulating creatinine concentrationHPOHP:0003259
- Frequent (30% to 79% of cases)
- Elevated erythrocyte sedimentation rateHPOHP:0003565
- Frequent (30% to 79% of cases)
- FatigueHPOHP:0012378
- Frequent (30% to 79% of cases)
- Flank painHPOHP:0030157
- Frequent (30% to 79% of cases)
- HydronephrosisHPOHP:0000126
- Frequent (30% to 79% of cases)
- HypertensionHPOHP:0000822
- Frequent (30% to 79% of cases)
Show the remaining 31
- Increased blood urea nitrogenHPOHP:0003138
- Frequent (30% to 79% of cases)
- Large vessel vasculitisHPOHP:0005310
- Frequent (30% to 79% of cases)
- Low back painHPOHP:0003419
- Frequent (30% to 79% of cases)
- Normocytic anemiaHPOHP:0001897
- Frequent (30% to 79% of cases)
- Renal insufficiencyHPOHP:0000083
- Frequent (30% to 79% of cases)
- Unilateral renal hypoplasiaHPOHP:0012583
- Frequent (30% to 79% of cases)
Where it sits
- Narrower terms (1)
Other names
3 names
Resolves to: IgG4-related retroperitoneal fibrosis
- Also called
- idiopathic retroperitoneal fibrosisOrmond diseaseRetroperitoneal Fibrosis