My Clinical Approach
Retroperitoneal fibrosis is rare but threatening to renal function. Management is multilevel: urgent kidney rescue through decompression + immunosuppressive therapy + surgical repair where needed.
According to EAU 2024 (ureteral obstruction), ACR criteria 2020 for IgG4-RD and ESH 2023:
- Early decompression with JJ stents or PCN in bilateral hydronephrosis.
- Contrast CT/MRI + serum IgG4 + ESR/CRP for diagnostic staging.
- CT-guided biopsy for histologic diagnosis and exclusion of malignancy.
- First-line corticosteroids (prednisolone 1 mg/kg taper).
- Tamoxifen / MMF / Rituximab in relapse or IgG4-RD.
- Ureterolysis with omental wrap laparoscopically/robotically in failure.
- Long-term follow-up with CT/MRI every 6-12 months + renal function.
What is retroperitoneal fibrosis
Retroperitoneal fibrosis (RPF — Ormond's disease) is characterized by accumulation of fibrous tissue that encases the descending abdominal aorta, IVC and ureters, without displacing the vessels. First described by Albarran (1905) and Ormond (1948).
Incidence: ~1:200,000-500,000 annually. Men:women = 3:1. Most common diagnostic age: 40-60 years. In 30% of cases there is systemic autoimmune disease (IgG4-RD, ANCA-associated vasculitis, rheumatoid arthritis).
Important: RPF does not displace the aorta anteriorly, a feature that distinguishes it from retroperitoneal lymphoma or sarcoma.
Causes (idiopathic vs secondary)
Idiopathic (2/3 — 70%)
- • IgG4-related disease (up to 50%)
- • Autoimmune inflammatory
- • Ormond's disease
- • Association with abdominal aortic aneurysms (inflammatory aneurysm)
Secondary (1/3 — 30%)
- • Drugs: methysergide, ergotamine, β-blockers, methyldopa, hydralazine, amiodarone
- • Malignancy: lymphoma, metastases (breast, prostate, lung), carcinoid
- • Infections: tuberculosis, actinomycosis, histoplasmosis
- • Radiotherapy of abdomen/pelvis
- • Surgery retroperitoneal / trauma
Symptoms & clinical presentation
Classic triad:
- Chronic dull flank / abdominal pain (95%) — non-specific, lasting weeks to months.
- Bilateral hydronephrosis (75%) — gradual ureteral obstruction.
- Elevated ESR/CRP/creatinine — laboratory findings.
Systemic
Fatigue, weight loss, low-grade fever, anorexia, night sweats.
From IVC compression
Lower limb edema, deep vein thrombosis (DVT), testicular weight (bilateral varicocele).
From acute kidney injury
Oliguria/anuria, hyperkalemia, nausea/vomiting, hypertension, fluid overload.
From arterial compression
Intermittent claudication, severe renovascular hypertension from renal artery stenosis (rare).
Diagnosis (CT/MRI, biopsy, IgG4)
Laboratory
ESR, CRP (elevated in 80%), creatinine/eGFR, urinalysis, serum IgG4 (>135 mg/dL suspicious), ANA, ANCA, rheumatoid factor — exclude systemic autoimmune disease.
Renal ultrasound
Bilateral hydronephrosis + possibly retroperitoneal mass. First line.
Contrast-enhanced abdominal CT
Diagnostic. Characteristic mass retroperitoneal that encases aorta/IVC/ureters, without displacing them. Difference from malignancy: RPF does not displace the aorta anteriorly.
Abdominal MRI
Better soft tissue resolution. On T2 sequences: active RPF = high signal (edema/inflammation); chronic inactive = low signal (fibrosis).
PET-CT (FDG)
Disease activity: increased FDG uptake in inflammatory areas. Treatment response monitoring. Differentiation from malignancy.
CT-guided biopsy
Confirmation: lymphoplasmacytic infiltrates, increased IgG4+ plasma cells (>10/HPF in IgG4-RD), storiform fibrosis. Exclusion of malignancy (lymphoma, sarcomas).
DMSA / scintigraphy
Renal function assessment before and after decompression.
Immediate decompression (emergency)
Sepsis or AKI = emergency. Decompression within 24 hours. Undiagnosed hyperkalemia >6.5 mmol/L → immediate hemodialysis.
JJ stent (first line)
Endoscopic placement under fluoroscopy. May fail in severe ureteral encasement by fibrosis. Exchange every 3-6 months until definitive treatment.
Percutaneous nephrostomy (PCN)
When endoscopic JJ stent placement fails. Immediate kidney decompression, monitoring of diuresis. Gradual transition to JJ stent when fibrosis is achieved.
Post-decompression care
IV fluids + electrolyte monitoring (often post-decompression diuresis), antibiotics (cephalosporin + aminoglycoside in sepsis), acidosis correction.
Medical therapy
1. Corticosteroids (first line)
Prednisolone 1 mg/kg/day × 4 weeks, taper to 5-10 mg/day maintenance for 6-12 months. Response 75%. Monitoring: ESR, CRP, CT mass every 3 months.
2. Tamoxifen
20 mg BID. Alternative in patients with contraindications to steroids or in combination. Response similar to low-dose corticosteroids (van Bommel 2009).
3. Mycophenolate Mofetil (MMF)
1 g BID, in combination with low-dose prednisolone. In relapse or resistance to corticosteroid monotherapy. Steroid-sparing for chronic immunosuppression.
4. Rituximab (anti-CD20)
1 g IV × 2 doses (2 weeks apart). Highly effective in IgG4-related disease (Khosroshahi 2013). Indication: failure or relapse on other therapies.
5. Methotrexate / Cyclophosphamide
Alternative immunosuppressives in difficult cases. Today often replaced by MMF/rituximab due to better safety profile.
Surgical management
Indications: failure of conservative therapy ≥6 months, re-obstruction after stent removal, multiple relapses, intolerable side effects of immunosuppressants.
Ureterolysis with omental wrap
Standard technique: removal of fibrous tissue around the ureters, intraperitoneal transposition, wrapping with omentum (as anti-fibrotic barrier). Laparoscopic or robotic approach preferred. Success 80-90%.
Ureteral substitution
In extensive ureteral damage: plasty with buccal mucosa, ileal interposition, renal autotransplantation or Boari flap.
Permanent nephrostomy
In patients unfit for surgery or failure of ureterolysis. Alternative: Resonance metallic stents (12-month duration) or renal autotransplantation for function preservation.
Frequently Asked Questions (FAQ)
What is retroperitoneal fibrosis?
Retroperitoneal fibrosis (Ormond's disease) is a rare chronic inflammatory disease characterized by deposition of fibrous tissue in the retroperitoneal space, which encases the aorta, inferior vena cava and ureters, causing progressive obstruction and hydronephrosis. Incidence ~1:200,000 annually, more common in men (3:1), ages 40-60.
What are the causes?
Idiopathic (2/3 of cases): autoimmune/IgG4-related disease. Secondary (1/3): drugs (methysergide, ergotamine, β-blockers, methyldopa), malignancy (lymphoma, metastasis), infection (tuberculosis, actinomycosis), radiotherapy, abdominal aortic aneurysms.
What are the symptoms?
Classic triad: chronic flank pain + bilateral hydronephrosis + elevated creatinine/ESR/CRP. Systemic: fatigue, weight loss, low-grade fever. In advanced disease: acute kidney injury (AKI) symptoms, lower limb edema from IVC compression, hypertension, anuria.
How is it diagnosed?
Contrast-enhanced CT/MRI of abdomen: retroperitoneal mass encasing the aorta, IVC and ureters, without displacing the vessels (differs from malignancy). PET-CT: disease activity. CT-guided biopsy: confirms diagnosis + excludes malignancy/IgG4. Serum IgG4, ESR, CRP, renal function.
How is acute obstruction managed?
Immediate decompression: endoscopic JJ stent placement or percutaneous nephrostomy (PCN) if endoscopic fails. In sepsis or AKI = emergency. Initially IV fluids + antibiotics + electrolyte monitoring for post-decompression diuresis.
What is the medical treatment?
First-line corticosteroids: prednisolone 1 mg/kg/day × 4 weeks, taper over 6-12 months. Tamoxifen 20 mg BID: alternative or in combination. Mycophenolate mofetil 1g BID: in relapse or resistance. Rituximab: in IgG4-related disease, 1g IV × 2 doses.
When is surgery indicated?
Ureterolysis with omental wrap: in failure of conservative therapy ≥6 months, in re-obstruction after stent removal, or relapse. Laparoscopic or robotic approach preferred. Success 80-90%.
What is the prognosis?
5-year survival 90% with idiopathic form. Renal function recovers in 70-80% with timely decompression. Relapse in 30% — long-term follow-up required with CT/MRI and renal function every 6 months, then annually. Secondary forms depend on underlying cause.
What is the relationship with IgG4-related disease?
Up to 50% of idiopathic cases belong to the spectrum of IgG4-related disease (IgG4-RD): systemic autoimmune disease with elevated serum IgG4 + lymphoplasmacytic infiltrates on biopsy. Often coexists with sialadenitis, type 1 pancreatitis, cholangitis. Rituximab highly effective.
Related topics
Suspected retroperitoneal fibrosis?
Contact us for full urologic and immunorheumatologic evaluation: CT/MRI, IgG4, biopsy and personalized plan — from urgent decompression to immunosuppressive therapy and ureterolysis.
Scientific literature
- EAU Guidelines on Urological Trauma & Ureteral Obstruction (2024) — uroweb.org
- Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet 2006;367(9506):241-51 — pubmed.ncbi.nlm.nih.gov
- van Bommel EFH et al. Tamoxifen vs prednisone for treatment of idiopathic retroperitoneal fibrosis. Eur Urol 2009;55(6):1448-55 — pubmed.ncbi.nlm.nih.gov
- Khosroshahi A et al. Rituximab therapy leads to rapid decline of serum IgG4 levels and prompt clinical improvement in IgG4-related disease. Medicine 2013;92(2):82-91 — pubmed.ncbi.nlm.nih.gov
- Fenaroli P et al. Mycophenolate mofetil and prednisone vs prednisone monotherapy for idiopathic retroperitoneal fibrosis: an RCT. Lancet Rheumatol 2021;3(7):e483-e491 — pubmed.ncbi.nlm.nih.gov
Medical Editorship
Dr. Marinos Vasilas, Urologist – Andrologist
Dr. Marinos Vasilas treats retroperitoneal fibrosis with modern multilevel approach: urgent endoscopic decompression, pharmacologic immunoregulation (corticosteroids/tamoxifen/MMF/rituximab) and surgical ureterolysis with omental wrap laparoscopically/robotically per EAU 2024.
View full profile
