Rapidly Progressive Glomerulonephritis (RPGN)

Table of Contents

1. Overview
2. Epidemiology
3. Classification: Three Types of RPGN
4. Pathophysiology
5. Clinical Presentation
6. Diagnosis
7. Serological Workup
8. Treatment
9. Type-Specific Treatment
10. Prognosis
11. Key Research Papers
12. Featured Videos

1. Overview

RPGN is defined by the clinical-pathological combination of:

• Clinical: acute glomerulonephritis syndrome (hematuria with RBC casts, proteinuria, hypertension, oliguria) with rapid GFR decline over days to weeks
• Pathological: crescents filling Bowman's space, commonly in more than 50% of glomeruli

Crescents represent proliferation of parietal epithelial cells (PECs) of Bowman's capsule in response to fibrin leakage through disrupted glomerular capillary walls (necrosis/breaks in GBM). Monocytes and macrophages migrate into the Bowman's space, amplifying proliferation. Initially cellular crescents can evolve to fibrocellular then fibrous crescents (irreversible scarring) within days to weeks, which is why early diagnosis and treatment matter for kidney outcome.

RPGN is classified into three immunopathological types based on the pattern of immunofluorescence (IF) on kidney biopsy — the most important initial test:

• Type I: Linear IgG (anti-GBM disease / Goodpasture syndrome)
• Type II: Granular IgG (immune complex–mediated GN)
• Type III: Pauci-immune (ANCA-associated vasculitis)

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2. Epidemiology

RPGN is uncommon, and the share of each type differs between populations. In Wessex, UK (a stable population of 2.5 million), the incidence of biopsy-proven pauci-immune (Type III) RPGN was 4 per million per year over 10 years, and 73% of these patients were ANCA-positive (Hedger 2000). A 2024 meta-analysis of 47 studies put the incidence of anti-GBM disease (Type I) at 0.60 to 1.79 per million population per year, and found anti-GBM disease in 8.0% of RPGN cases (Kuang 2024). In a single-centre Chinese series of 528 biopsy-proven crescentic GN cases (mean age 37.6 years), 11.6% were Type I, 62.7% Type II (lupus nephritis 34.3%, IgA nephropathy 17.4%) and 25.8% Type III (Chen 2016). In a pooled cohort of 848 patients from seven European ANCA-vasculitis trials, the mean age at diagnosis was 58 years (Sánchez-Álamo 2023). Immune complex RPGN (Type II) occurs across all ages, depending on the underlying condition.

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3. Classification: Three Types of RPGN

Type I — Anti-GBM Disease (Goodpasture Syndrome)

Pathogenesis: Autoantibodies directed against the non-collagenous domain 1 of the alpha-3 chain of type IV collagen [α3(IV)NC1] — expressed in GBM and alveolar basement membrane. The disease is strongly associated with the HLA allele DRB1*15:01 (DR15) (McAdoo 2025). Spatial and temporal clustering of cases supports an environmental trigger such as infection in genetically susceptible people (Canney 2016; McAdoo 2017), and relapses are usually associated with ongoing exposure to lung irritants such as cigarette smoke or hydrocarbons (McAdoo 2025).

Classic Goodpasture syndrome = pulmonary hemorrhage + crescentic GN. Reviews describe concurrent alveolar hemorrhage in 40%–60% of patients (McAdoo 2017); a 2024 meta-analysis pooled the prevalence of lung hemorrhage at 32.6% (Kuang 2024). The remainder have kidney-limited disease.

IF: Linear IgG along the GBM (the "railroad track" pattern).

Type II — Immune Complex GN

Granular "lumpy-bumpy" IgG/IgM/IgA deposits on IF (distinguish from linear Type I and paucity of deposits in Type III).

Causes include:

• Post-infectious GN (streptococcal or staphylococcal infection; infective endocarditis)
• Lupus nephritis Class III/IV (diffuse proliferative)
• IgA nephropathy (crescentic variant)
• Membranoproliferative GN (MPGN)
• Cryoglobulinemic GN (hepatitis C-associated)
• Henoch-Schönlein purpura (IgA vasculitis)

Type III — Pauci-Immune ANCA-Associated GN

Pauci-immune = few or no immune deposits on IF (despite severe glomerular injury). Associated with antineutrophil cytoplasmic antibodies (ANCA):

• PR3-ANCA (c-ANCA): associated with Granulomatosis with Polyangiitis (GPA, formerly Wegener's); upper/lower airway granulomas + glomerulonephritis
• MPO-ANCA (p-ANCA): associated with Microscopic Polyangiitis (MPA); predominantly renal + pulmonary capillaritis; no granulomas
• Drug-induced AAV: hydralazine (MPO-ANCA), propylthiouracil, minocycline
• ANCA-negative disease: combined immunofluorescence and PR3/MPO assays detect ANCA in up to 90% of patients with active generalised GPA and MPA (Sinico 2014); in the Wessex series, 27% of pauci-immune RPGN cases tested ANCA-negative (Hedger 2000)

Double-positivity (anti-GBM + ANCA): circulating ANCA, usually against MPO, are found in 30%–40% of patients with anti-GBM disease (McAdoo 2025). In a European cohort of 37 double-positive patients, the disease combined features of both conditions (severe kidney disease and frequent lung hemorrhage, but an older age profile); no single-positive anti-GBM patient relapsed, whereas about half of surviving double-positive and ANCA-vasculitis patients had recurrent disease over a median 4.8 years. The authors describe early treatment as for anti-GBM disease, with long-term follow-up and consideration of maintenance immunosuppression as for ANCA vasculitis (McAdoo 2017, Kidney Int).

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4. Pathophysiology

The final common pathway in all three types is disruption of the glomerular capillary wall:

1. Necrosis/rupture of GBM: fibrin leakage into Bowman's space
2. Parietal epithelial cell (PEC) proliferation: PECs line Bowman's capsule and are progenitor cells; injury induces rapid proliferation
3. Monocyte/macrophage recruitment: driven by chemoattractants (MCP-1, IL-8, complement products); monocytes differentiate into macrophages within the crescent
4. Extracellular matrix deposition: fibronectin, collagen IV, tenascin; early cellular crescents → fibrocellular → fibrous (permanent)
5. Tubular atrophy: from reduced GFR, ischemia, and protein casts
6. Interstitial inflammation: CD4+ T-cell infiltration; drives tubular injury

In Type III AAV, ANCA activates primed neutrophils → neutrophil extracellular traps (NETs) → endothelial injury → fibrinoid necrosis; MPO and PR3 released from degranulating neutrophils propagate inflammation and GBM injury.

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5. Clinical Presentation

RPGN presents as a nephritic syndrome with rapid GFR decline:

• Hematuria: macroscopic (tea/cola-colored) or microscopic; dysmorphic RBCs and RBC casts point to a glomerular origin
• Proteinuria: usually below the nephrotic range; can be in the nephrotic range with underlying MPGN or lupus
• Oliguria: developing over days-weeks as GFR falls
• Hypertension: often marked; reflects RAAS activation and fluid retention
• Edema: periorbital, pedal
• Uremia: if rapid progression — nausea, vomiting, fatigue, confusion

Type-specific extra-renal features:

• Type I (anti-GBM/Goodpasture): Pulmonary hemorrhage in 40%–60% (McAdoo 2017): hemoptysis, dyspnea, alveolar infiltrates on CXR/CT (diffuse alveolar hemorrhage). It can be life-threatening but is highly treatment-responsive, with 90%–100% reaching remission with immunosuppression and plasma exchange (McAdoo 2025).
• Type III GPA (PR3-ANCA): Sinus disease (crusting, epistaxis, saddle-nose deformity), otitis media, subglottic stenosis, orbital pseudotumor, lung nodules/cavities, skin purpura
• Type III MPA (MPO-ANCA): Pulmonary capillaritis (hemoptysis, restrictive pattern), mononeuritis multiplex, constitutional symptoms
• Type II lupus: malar rash, photosensitivity, arthritis, serositis, CBC abnormalities
• Type II post-infectious: recent pharyngitis/skin infection; low complement (C3)

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6. Diagnosis

Kidney biopsy is the key test and is done urgently, because cellular crescents can evolve into irreversible fibrous crescents within days to weeks.

• Light microscopy: crescents, often in more than 50% of glomeruli; may see necrotizing lesions (fibrinoid necrosis of capillary tufts); glomerular hypercellularity; interstitial inflammation
• Immunofluorescence (distinguishes the three types):
  • Type I: linear IgG along GBM (± IgM, C3 trace)
  • Type II: granular IgG/IgM/IgA/C3 deposits in mesangium and capillary walls (pattern depends on underlying cause)
  • Type III: few or no immune deposits (pauci-immune)
• Electron microscopy: confirms GBM rupture; subepithelial humps (post-infectious), subendothelial deposits (lupus/MPGN), mesangial deposits (IgAN), or no deposits (pauci-immune/anti-GBM)

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7. Serological Workup

Serology and biopsy are usually pursued at the same time, so that neither waits on the other; a 2025 review calls rapid and accurate diagnosis essential to guide therapy in anti-GBM disease (McAdoo 2025).

• ANCA: c-ANCA/PR3 (GPA) and p-ANCA/MPO (MPA) — when indirect immunofluorescence is combined with PR3- and MPO-specific assays, ANCA are detected in up to 90% of patients with active generalised GPA and MPA and in about 40% of patients with EGPA (Sinico & Radice, 2014)
• Anti-GBM antibodies: immunoassay for α3(IV)NC1; circulating antibody is found in more than 90% of classic anti-GBM disease (McAdoo 2025), and a 2024 meta-analysis pooled anti-GBM antibody prevalence at 88.8% (Kuang 2024); testing for both anti-GBM antibodies and ANCA identifies double-positive patients
• ANA + anti-dsDNA: lupus (Type II)
• Complement C3, C4, CH50: low in post-infectious GN, lupus, MPGN, cryoglobulinemia; normal in anti-GBM and ANCA-associated
• ASO titer, anti-DNase B: post-streptococcal GN
• Hepatitis B and C serology: MPGN, cryoglobulinemia
• HIV, syphilis serology
• Cryoglobulins (Type II if cryoglobulinemic)
• Blood cultures (endocarditis-associated GN)
• SPEP, free light chains (myeloma-associated)

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8. Treatment: General Approach

RPGN is treated as a nephrology emergency. A 2025 treatment review of anti-GBM disease states that where clinical suspicion is high and disease is immediately organ- or life-threatening, empirical treatment (for example glucocorticoids and/or plasma exchange) may be started while definitive tests are awaited (McAdoo 2025):

• Glucocorticoids: high-dose glucocorticoids are part of induction for anti-GBM disease and ANCA vasculitis (McAdoo 2025; Hellmich 2024); pulsed intravenous methylprednisolone is widely used, although the same review notes limited evidence for it in anti-GBM disease when the other components of therapy start promptly (McAdoo 2025)
• Supportive care: management of hyperkalemia, acidosis and fluid overload; intensive care when there is pulmonary hemorrhage; about half of patients with anti-GBM disease need acute dialysis at diagnosis (McAdoo 2025)
• Patients on dialysis: recovery of kidney function after treatment is possible — 34% of ANCA-vasculitis trial patients who first needed dialysis recovered (Sánchez-Álamo 2024). In anti-GBM disease, every patient in a UK series who needed immediate dialysis and had 100% crescents remained dialysis-dependent (Levy 2001), and a biopsy study saw no recovery with 100% crescents or more than 50% glomerulosclerosis (cited in McAdoo 2025)

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9. Type-Specific Treatment

Type I (Anti-GBM Disease)

• Plasma exchange (plasmapheresis): removes circulating antibody; the 2025 treatment review describes daily exchanges continued until antibody levels are fully suppressed, typically 14 days, with antibody levels monitored during and after treatment; observational studies associate early plasma exchange with better outcomes (McAdoo 2025)
• Cyclophosphamide + prednisolone: to stop new antibody production; the same review describes oral cyclophosphamide 2–3 mg/kg/day for 2–3 months and prednisolone 1 mg/kg/day (maximum 60 mg), tapered to stop at 6 months (McAdoo 2025). In a UK series of 71 patients treated with plasma exchange, prednisolone and cyclophosphamide, 1-year kidney survival was 95% when creatinine at presentation was below 500 μmol/L (5.7 mg/dL) (Levy 2001)
• Rituximab: published experience in anti-GBM disease is limited; it is described as an option when cyclophosphamide is contraindicated, or as an adjunct in severe or refractory disease (McAdoo 2025)
• Relapse and transplantation: relapse is rare (under 3%), and maintenance immunosuppression is not routinely required except in double-positive patients; anti-GBM antibodies are kept undetectable for at least 6 months before kidney transplantation to prevent recurrence in the graft (McAdoo 2025)

Type II (Immune Complex GN)

Treatment is directed at the underlying cause:

• Post-infectious GN: treatment of the infection and supportive care; a 2008 review describes the long-term prognosis of post-streptococcal GN as excellent in children but significantly worse in elderly people (Rodriguez-Iturbe 2008)
• Lupus nephritis Class III/IV: glucocorticoids with cyclophosphamide (including the low-dose Euro-Lupus regimen) or mycophenolate mofetil. Two add-on drugs have phase 3 trial results: belimumab raised the renal response at 104 weeks (43% vs 32%; Furie 2020), and voclosporin raised complete renal response at 52 weeks (41% vs 23%; Rovin 2021) — see the Lupus Nephritis page
• IgA nephropathy with crescents: crescentic IgA nephropathy can follow a rapidly progressive course; its treatment is covered on the IgA Nephropathy page
• MPGN and C3 glomerulopathy: treatment of an underlying cause such as hepatitis C; in C3 glomerulopathy, the complement factor B inhibitor iptacopan reduced proteinuria by 35.1% relative to placebo at 6 months in the APPEAR-C3G trial of 74 adults (Kavanagh 2025)
• Cryoglobulinemia: antiviral therapy for hepatitis C; rituximab; plasma exchange in severe disease

Type III (ANCA-Associated Vasculitis)

Induction therapy — the 2022 EULAR update recommends high-dose glucocorticoids with either rituximab or cyclophosphamide for organ- or life-threatening disease (Hellmich 2024):

• Rituximab (375 mg/m² weekly for 4 weeks): in RAVE (197 ANCA-positive patients with GPA or MPA), rituximab was non-inferior to daily oral cyclophosphamide (64% vs 53% in remission without prednisone at 6 months) and more effective in relapsing disease (67% vs 42%) (Stone 2010). In RITUXVAS (44 patients with kidney involvement, median GFR 18 ml/min), a rituximab-based regimen was not superior to intravenous cyclophosphamide (sustained remission 76% vs 82%) (Jones 2010)
• Cyclophosphamide: in CYCLOPS (149 patients with kidney involvement), intravenous pulses of 15 mg/kg every 2–3 weeks induced remission as often as daily oral 2 mg/kg (88.1% vs 87.7% at 9 months), with a lower cumulative dose (median 8.2 g vs 15.9 g) and less leukopenia (de Groot 2009). Its recognised toxicities include bone-marrow suppression, urothelial (bladder) toxicity, infertility and long-term secondary malignancy (McAdoo 2025)

PEXIVAS trial (2020): In 704 patients with severe ANCA-associated vasculitis (eGFR <50 ml/min/1.73 m² or diffuse pulmonary hemorrhage), plasma exchange did not reduce the composite of death from any cause or end-stage kidney disease compared with no plasma exchange (28.4% vs. 31.0%; hazard ratio 0.86, P = 0.27). The same trial found a reduced-dose oral glucocorticoid regimen noninferior to the standard-dose regimen for that outcome, with fewer serious infections at 1 year. The 2022 EULAR update states that plasma exchange may be considered in patients with rapidly progressive glomerulonephritis (Hellmich 2024). Plasma exchange remains part of standard treatment for anti-GBM disease (see Type I above).

Maintenance therapy:

• Rituximab: in MAINRITSAN (115 patients in complete remission after cyclophosphamide), rituximab 500 mg on days 0 and 14 and at months 6, 12 and 18 was compared with daily azathioprine; major relapse by month 28 occurred in 5% vs 29%, with similar rates of severe adverse events (Guillevin 2014). The 2022 EULAR update recommends rituximab for maintenance in GPA/MPA (Hellmich 2024)
• Azathioprine: in CYCAZAREM (144 patients in remission after at least 3 months of oral cyclophosphamide), switching to azathioprine 2 mg/kg/day did not increase relapse compared with continued cyclophosphamide over 18 months (15.5% vs 13.7%); relapse was less frequent in MPA than in GPA (Jayne 2003). EULAR lists azathioprine and methotrexate as alternatives to biologics (Hellmich 2024)

Avacopan (C5a receptor antagonist): approved by the US FDA in October 2021 as an adjunctive treatment for adults with severe active ANCA-associated vasculitis (GPA and MPA), in combination with standard therapy including glucocorticoids. The approval rested mainly on the ADVOCATE trial (Jayne et al., NEJM 2021, PubMed PMID: 33596356), whose published report was retracted by the journal on 29 June 2026 (retraction notice PMID 42377355). The European Medicines Agency concluded in June 2026 that the trial was conducted in breach of good clinical practice (GCP) principles and that the study data provided at the time of assessment were “incorrect and misleading”; the EU marketing authorisation was revoked on 4 August 2026 (EMA announcement, 26 June 2026). The FDA’s Drugs@FDA record (NDA 214487) still listed the 2021 approval as of October 2026; whether the FDA has taken any action since the retraction could not be confirmed.

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10. Prognosis

Prognosis depends on the type, kidney function at presentation, and how much of the kidney is already scarred:

• Type I (Anti-GBM): in the UK series of 71 treated patients, 1-year kidney survival was 95% with creatinine below 500 μmol/L (5.7 mg/dL) at presentation, 82% with higher creatinine not needing immediate dialysis, and 8% in those who were dialysis-dependent at presentation (Levy 2001). A 2024 meta-analysis pooled 1-year patient and kidney survival at 76.2% and 30.2% (Kuang 2024)
• Type III (ANCA-AAV): among 848 patients from seven European trials, the cumulative incidence of end-stage kidney disease was 17% at 5 years and 22% at 10 years (Sánchez-Álamo 2024). Relapse is common: about half of surviving patients with ANCA vasculitis had recurrent disease over a median 4.8 years in one European cohort (McAdoo 2017, Kidney Int)
• Type II: varies by underlying cause; in the Chinese crescentic GN series, 5-year kidney survival was 17.6% for Type I, 70.1% for Type II and 44.3% for Type III (Chen 2016)

Factors linked to a poor kidney outcome include oligoanuria, a high proportion of glomerular crescents and dialysis-dependent kidney failure at presentation in anti-GBM disease (McAdoo 2017), and fewer normal glomeruli and more glomerulosclerosis on biopsy in ANCA vasculitis (Sánchez-Álamo 2024).

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Key Research Papers

1. Jennette JC. Rapidly progressive crescentic glomerulonephritis. Kidney Int. 2003;63(3):1164–1177. — PubMed PMID: 12631105 · doi:10.1046/j.1523-1755.2003.00843.x
2. Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis (RAVE). N Engl J Med. 2010;363(3):221–232. — PubMed PMID: 20647199 · doi:10.1056/NEJMoa0909905
3. Jones RB, Tervaert JWC, Hauser T, et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis (RITUXVAS). N Engl J Med. 2010;363(3):211–220. — PubMed PMID: 20647198 · doi:10.1056/NEJMoa0909169
4. Walsh M, Merkel PA, Peh CA, et al. Plasma Exchange and Glucocorticoids in Severe ANCA-Associated Vasculitis (PEXIVAS). N Engl J Med. 2020;382(7):622–631. — PubMed PMID: 32053298 · doi:10.1056/NEJMoa1803537
5. Guillevin L, Pagnoux C, Karras A, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis (MAINRITSAN). N Engl J Med. 2014;371(19):1771–1780. — PubMed PMID: 25372085 · doi:10.1056/NEJMoa1404231
6. Jayne DRW, Merkel PA, Schall TJ, Bekker P, for the ADVOCATE Study Group. Avacopan for the Treatment of ANCA-Associated Vasculitis. N Engl J Med. 2021;384(7):599–609. — PubMed PMID: 33596356 · doi:10.1056/NEJMoa2023386 — Retracted (retraction notice: PubMed PMID: 42377355)
7. Levy JB, Turner AN, Rees AJ, Pusey CD. Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression. Ann Intern Med. 2001;134(11):1033–1042. — PubMed PMID: 11388816 · doi:10.7326/0003-4819-134-11-200106050-00009
8. Hudson BG, Tryggvason K, Sundaramoorthy M, Neilson EG. Alport's syndrome, Goodpasture's syndrome, and type IV collagen. N Engl J Med. 2003;348(25):2543–2556. — PubMed PMID: 12815141 · doi:10.1056/NEJMra022296
9. Pusey CD. Anti-glomerular basement membrane disease. Kidney Int. 2003;64(4):1535–1550. — PubMed PMID: 12969182 · doi:10.1046/j.1523-1755.2003.00241.x
10. Bomback AS, Appel GB. Pathogenesis of the C3 glomerulopathies and reclassification of MPGN. Nat Rev Nephrol. 2012;8(11):634–642. — PubMed PMID: 23026947 · doi:10.1038/nrneph.2012.213
11. Sinico RA, Radice A. Antineutrophil cytoplasmic antibodies (ANCA) testing: detection methods and clinical application. Clin Exp Rheumatol. 2014;32(3 Suppl 82):S112–117. — PubMed PMID: 24854381
12. McAdoo SP, Pusey CD. Anti-Glomerular Basement Membrane Disease. Clin J Am Soc Nephrol. 2017;12(7):1162–1172. — PubMed PMID: 28515156 · doi:10.2215/CJN.01380217
13. Hellmich B, Sanchez-Alamo B, Schirmer JH, et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis. 2024;83(1):30–47. — PubMed PMID: 36927642 · doi:10.1136/ard-2022-223764
14. McAdoo SP, Pusey CD. Anti-glomerular basement membrane disease—treatment standard. Nephrol Dial Transplant. 2025;41(1):42–54. — PubMed PMID: 40973182 · doi:10.1093/ndt/gfaf190
15. McAdoo SP, Tanna A, Hrušková Z, et al. Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival, frequency of relapse, and outcomes compared to single-seropositive patients. Kidney Int. 2017;92(3):693–702. — PubMed PMID: 28506760 · doi:10.1016/j.kint.2017.03.014
16. Kuang H, Jiang N, Jia XY, et al. Epidemiology, clinical features, risk factors, and outcomes in anti-glomerular basement membrane disease: a systematic review and meta-analysis. Autoimmun Rev. 2024;23(4):103531. — PubMed PMID: 38493958 · doi:10.1016/j.autrev.2024.103531
17. Canney M, O'Hara PV, McEvoy CM, et al. Spatial and temporal clustering of anti-glomerular basement membrane disease. Clin J Am Soc Nephrol. 2016;11(8):1392–1399. — PubMed PMID: 27401523 · doi:10.2215/CJN.13591215
18. Hedger N, Stevens J, Drey N, et al. Incidence and outcome of pauci-immune rapidly progressive glomerulonephritis in Wessex, UK: a 10-year retrospective study. Nephrol Dial Transplant. 2000;15(10):1593–1599. — PubMed PMID: 11007827 · doi:10.1093/ndt/15.10.1593
19. Chen S, Tang Z, Xiang H, et al. Etiology and outcome of crescentic glomerulonephritis from a single center in China: a 10-year review. Am J Kidney Dis. 2016;67(3):376–383. — PubMed PMID: 26388283 · doi:10.1053/j.ajkd.2015.07.034
20. Sánchez-Álamo B, Moi L, Bajema I, et al. Long-term outcome of kidney function in patients with ANCA-associated vasculitis. Nephrol Dial Transplant. 2024;39(9):1483–1493. — PubMed PMID: 38268409 · doi:10.1093/ndt/gfae018
21. Sánchez Álamo B, Moi L, Bajema I, et al. Long-term outcomes and prognostic factors for survival of patients with ANCA-associated vasculitis. Nephrol Dial Transplant. 2023;38(7):1655–1665. — PubMed PMID: 36617233 · doi:10.1093/ndt/gfac320
22. de Groot K, Harper L, Jayne DR, et al. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis (CYCLOPS). Ann Intern Med. 2009;150(10):670–680. — PubMed PMID: 19451574 · doi:10.7326/0003-4819-150-10-200905190-00004
23. Jayne D, Rasmussen N, Andrassy K, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies (CYCAZAREM). N Engl J Med. 2003;349(1):36–44. — PubMed PMID: 12840090 · doi:10.1056/NEJMoa020286
24. Rodriguez-Iturbe B, Musser JM. The current state of poststreptococcal glomerulonephritis. J Am Soc Nephrol. 2008;19(10):1855–1864. — PubMed PMID: 18667731 · doi:10.1681/ASN.2008010092
25. Furie R, Rovin BH, Houssiau F, et al. Two-year, randomized, controlled trial of belimumab in lupus nephritis (BLISS-LN). N Engl J Med. 2020;383(12):1117–1128. — PubMed PMID: 32937045 · doi:10.1056/NEJMoa2001180
26. Rovin BH, Teng YKO, Ginzler EM, et al. Efficacy and safety of voclosporin versus placebo for lupus nephritis (AURORA 1): a double-blind, randomised, multicentre, placebo-controlled, phase 3 trial. Lancet. 2021;397(10289):2070–2080. — PubMed PMID: 33971155 · doi:10.1016/S0140-6736(21)00578-X
27. Kavanagh D, Bomback AS, Vivarelli M, et al. Oral iptacopan therapy in patients with C3 glomerulopathy (APPEAR-C3G): a randomised, double-blind, parallel group, multicentre, placebo-controlled, phase 3 study. Lancet. 2025;406(10512):1587–1598. — PubMed PMID: 41016405 · doi:10.1016/S0140-6736(25)01148-1

PubMed Topic Searches

1. PubMed: Rapidly progressive glomerulonephritis crescentic treatment
2. PubMed: ANCA vasculitis rituximab RAVE RITUXVAS
3. PubMed: Anti-GBM disease Goodpasture syndrome plasmapheresis
4. PubMed: PEXIVAS plasma exchange ANCA vasculitis
5. PubMed: Avacopan ANCA vasculitis ADVOCATE trial
6. PubMed: Lupus nephritis crescentic glomerulonephritis
7. PubMed: MAINRITSAN rituximab vasculitis maintenance

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Connections

• Nephrology & Hepatology
• Glomerulonephritis
• Acute Kidney Injury
• IgA Nephropathy
• Nephrotic Syndrome
• Lupus Nephritis
• Chronic Kidney Disease
• Pulmonary Hemorrhage
• Vasculitis
• Systemic Lupus Erythematosus
• Kidney Function Tests
• Urinalysis
• Creatinine

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