[Durgical](https://durgical.com/en/) › [DurgiPedia](https://durgical.com/en/durgipedia/) › [Conditions](https://durgical.com/en/conditions/) › Rapidly progressive glomerulonephritis (anti-GBM, ANCA)

[Conditions](https://durgical.com/en/conditions/) · [Nephrology and urology](https://durgical.com/en/conditions/)

# Rapidly progressive glomerulonephritis (anti-GBM, ANCA)

Rapidly progressive glomerulonephritis is severe kidney inflammation that destroys kidney function within days to weeks; it needs emergency treatment.

Updated 2026-11-01

## Structures involved

Tap one to see what it is and open it in 3D.

- [Kidneys](https://durgical.com/en/anatomy/kidney/)
- [Vessels inside the kidney](https://durgical.com/en/anatomy/intrarenal-arteries-and-veins/)
- [Segmental bronchi](https://durgical.com/en/anatomy/segmental-bronchus/)
- [Nasal cavity](https://durgical.com/en/anatomy/nasal-cavity/)
- [Nasal septum](https://durgical.com/en/anatomy/nasal-septum/)

## In the clinical exam

- [Nephrotic vs nephritic syndrome](https://durgical.com/en/clinical-exam/nephrotic-vs-nephritic-syndrome/)

## Why it happens

- [When leukocytes injure tissue: proteases, oxidants and NETs](https://durgical.com/en/general-pathology/when-leukocytes-injure-tissue-proteases-oxidants-and-nets/)
- [Patterns of necrosis: coagulative, liquefactive, caseous, fat, fibrinoid, gangrenous](https://durgical.com/en/general-pathology/patterns-of-necrosis-coagulative-liquefactive-caseous-fat-fibrinoid-gangrenous/)
- [Physiology of acute and chronic kidney failure](https://durgical.com/en/physiology/physiology-of-acute-and-chronic-kidney-failure/)
- [Glomerulus and the filtration barrier: podocytes, mesangium, JGA](https://durgical.com/en/histology/glomerulus-and-the-filtration-barrier-podocytes-mesangium-jga/)
- [Basement membrane](https://durgical.com/en/histology/basement-membrane/)

## What it is

A clinical syndrome of glomerulonephritis with a loss of kidney function over days to weeks, usually with crescents in most glomeruli on biopsy (crescentic glomerulonephritis). It is a medical emergency because untreated it leads to irreversible kidney failure. When it occurs with lung haemorrhage it forms a pulmonary-renal syndrome, such as Goodpasture (anti-GBM) disease or ANCA vasculitis.

## Who gets it

Uncommon, but important to recognise. ANCA-associated disease (granulomatosis with polyangiitis, microscopic polyangiitis) is the most frequent cause in adults, especially over 50. Anti-GBM disease has two peaks: young men, often with lung haemorrhage, and older women. In Latin America, lupus nephritis is a major immune-complex cause in young women.

## Causes

- Type I, anti-GBM: antibodies against type IV collagen of the glomerular basement membrane; with lung haemorrhage it is Goodpasture syndrome
- Type II, immune complex: lupus nephritis, IgA nephropathy or IgA vasculitis, post-infectious glomerulonephritis, cryoglobulinaemia (hepatitis C), endocarditis
- Type III, pauci-immune: ANCA vasculitis (granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis)
- Some patients have both anti-GBM and ANCA (double positive)

## Risk factors

- Smoking and inhaled hydrocarbons (lung haemorrhage in anti-GBM disease)
- Older age (ANCA vasculitis)
- Systemic lupus erythematosus
- Drugs linked to ANCA: hydralazine, propylthiouracil, levamisole-adulterated cocaine
- Genetic susceptibility (HLA types)

## Pathophysiology

Severe injury breaks the glomerular capillary walls, so fibrin and inflammatory cells leak into Bowman's space. Parietal epithelial cells and macrophages proliferate around the tuft and form crescents that compress it; without treatment, cellular crescents become fibrous and the nephron is lost. In anti-GBM disease the antibody binds linearly along the basement membrane, and a similar antigen in alveolar capillaries explains lung bleeding. In ANCA vasculitis, ANCA activate neutrophils that damage small vessels with few or no immune deposits.

## Classification

- Type I anti-GBM: linear IgG on immunofluorescence
- Type II immune complex: granular deposits
- Type III pauci-immune (ANCA): few or no deposits; anti-MPO or anti-PR3 antibodies

## Symptoms and signs

- Falling urine output and dark or cola-coloured urine
- Oedema and high blood pressure
- Fatigue, nausea and other symptoms of uraemia
- Coughing up blood and breathlessness (lung haemorrhage)
- Systemic symptoms in vasculitis: fever, weight loss, joint pain, purpura
- Chronic sinusitis, nosebleeds, ear disease (granulomatosis with polyangiitis); asthma and eosinophilia (eosinophilic form)

## Red flags

- Haemoptysis or falling oxygen saturation (diffuse alveolar haemorrhage)
- Oliguria or anuria
- Severe hyperkalaemia, acidosis or fluid overload needing dialysis
- Creatinine that rises day by day

## Diagnosis

**Clinical**
A nephritic picture (haematuria, hypertension, oedema) with rapidly worsening [kidney](https://durgical.com/en/anatomy/kidney/) function, plus a search for lung, upper airway, [skin](https://durgical.com/en/anatomy/skin/) and joint involvement.

**Tests**
Urinalysis with dysmorphic red cells and red cell casts; daily creatinine and potassium; ANCA (MPO and PR3), anti-GBM antibodies, ANA and anti-dsDNA, complement C3 and C4, antistreptococcal antibodies, hepatitis serology and cryoglobulins. Urgent kidney biopsy confirms crescents and the type; treatment is not delayed while waiting for it when suspicion is high.

**Imaging**
Kidney ultrasound (normal-sized kidneys suggest an acute process); [chest](https://durgical.com/en/anatomy/region/chest/) X-ray or CT for alveolar haemorrhage, nodules or cavities; sinus CT when granulomatosis is suspected.

## Differential diagnosis

- Acute tubular necrosis and other causes of acute kidney injury
- Acute interstitial nephritis
- Thrombotic microangiopathy (haemolytic uraemic syndrome)
- Obstructive uropathy
- Pneumonia with sepsis and kidney injury, or leptospirosis (pulmonary-renal picture in the tropics)

## Goal of treatment

Stop immune injury as fast as possible to rescue kidney function and control lung bleeding, support the patient through kidney failure, and maintain remission while limiting treatment toxicity.

## Treatment

**Acute**
Emergency care: admission, control of airway and oxygenation in lung haemorrhage, and correction of potassium, acidosis and fluid status.

**Medical**
Induction with high-dose intravenous then oral glucocorticoids plus cyclophosphamide or rituximab; maintenance with rituximab, azathioprine or mycophenolate for ANCA vasculitis; treatment of any secondary cause.

**Procedure**
Plasma exchange to remove circulating antibodies, standard in anti-GBM disease and considered in selected severe cases.

**Procedure**
Dialysis for kidney failure; kidney transplantation later once the disease is quiescent.

**Follow-up**
Nursing care: strict intake and output, daily weight, potassium and creatinine, respiratory status, and infection prevention during immunosuppression, including prophylaxis against Pneumocystis.

## Complications

- Irreversible kidney failure requiring dialysis
- Diffuse alveolar haemorrhage and respiratory failure
- Serious infections from immunosuppression
- Relapse of vasculitis
- Cyclophosphamide toxicity: bladder injury, infertility, cancer

## Prognosis

Outcome depends on how early treatment begins. Patients who start with lower creatinine and fewer fibrous crescents often recover useful function; those already on dialysis at presentation in anti-GBM disease rarely recover. ANCA vasculitis responds in most patients but relapses are common.

## Test yourself

Think of the answer, then tap the question to see it.

## What biopsy finding defines rapidly progressive glomerulonephritis?

Crescents in most glomeruli (crescentic glomerulonephritis).

## What immunofluorescence pattern is seen in anti-GBM disease?

Linear IgG along the glomerular basement membrane.

## Which syndrome combines glomerulonephritis with lung haemorrhage from anti-GBM antibodies?

Goodpasture syndrome.

## Which antibodies are linked to granulomatosis with polyangiitis and to microscopic polyangiitis?

PR3-ANCA (c-ANCA) mainly in granulomatosis with polyangiitis; MPO-ANCA (p-ANCA) mainly in microscopic polyangiitis.

## Which treatment removes circulating anti-GBM antibodies?

Plasma exchange, together with glucocorticoids and cyclophosphamide.

[Add to my cards (5)](https://durgical.app/estudiar/?add=/en/conditions/rapidly-progressive-glomerulonephritis/)

## Related conditions

- [Granulomatosis with polyangiitis](https://durgical.com/en/conditions/granulomatosis-with-polyangiitis/)
- [Microscopic polyangiitis](https://durgical.com/en/conditions/microscopic-polyangiitis/)
- [Eosinophilic granulomatosis with polyangiitis (Churg–Strauss)](https://durgical.com/en/conditions/eosinophilic-granulomatosis-with-polyangiitis/)
- [Systemic lupus erythematosus](https://durgical.com/en/conditions/systemic-lupus-erythematosus/)
- [Lupus nephritis](https://durgical.com/en/conditions/lupus-nephritis/)
- [IgA nephropathy](https://durgical.com/en/conditions/iga-nephropathy/)
- [IgA vasculitis (Henoch–Schönlein purpura)](https://durgical.com/en/conditions/iga-vasculitis/)
- [Post-streptococcal glomerulonephritis](https://durgical.com/en/conditions/post-streptococcal-glomerulonephritis/)
- [Membranoproliferative glomerulonephritis](https://durgical.com/en/conditions/membranoproliferative-glomerulonephritis/)
- [Cryoglobulinemic vasculitis](https://durgical.com/en/conditions/cryoglobulinemic-vasculitis/)
- [Infective endocarditis](https://durgical.com/en/conditions/infective-endocarditis/)
- [Hemolytic uremic syndrome (HUS)](https://durgical.com/en/conditions/hemolytic-uremic-syndrome/)
- [Acute kidney injury](https://durgical.com/en/conditions/acute-kidney-injury/)
- [Chronic kidney disease](https://durgical.com/en/conditions/chronic-kidney-disease/)

Educational information, not medical advice. For any symptom, see a health professional.

Sources: [Glomerulonephritis: MedlinePlus Medical Encyclopedia](https://medlineplus.gov/ency/article/000484.htm) (MedlinePlus (U.S. National Library of Medicine), 2025); [Anti-glomerular basement membrane disease: MedlinePlus Medical Encyclopedia](https://medlineplus.gov/ency/article/000142.htm) (MedlinePlus (U.S. National Library of Medicine), 2025).

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Source: https://durgical.com/en/conditions/rapidly-progressive-glomerulonephritis/
License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/conditions/rapidly-progressive-glomerulonephritis/