Goodpasture’s Syndrome (Anti-GBM Disease)

Goodpasture’s syndrome—also called anti–glomerular basement membrane (anti-GBM) disease—is a rare, rapidly progressive autoimmune illness in which antibodies target a collagen protein in the kidney glomerular basement membrane and the lung alveolar basement membrane. The result can be sudden kidney failure and life-threatening lung haemorrhage. Early recognition and urgent treatment are critical.

What’s Happening in the Body?

This is a classic type II hypersensitivity process: IgG autoantibodies bind linearly along the basement membrane of glomeruli and alveoli, triggering inflammation and tissue damage. Some patients are “double-positive,” with both anti-GBM antibodies and ANCA (anti-neutrophil cytoplasmic antibodies); these cases may have features of small-vessel vasculitis.

Who Is Affected & Triggers

Very rare: roughly ~1 case per million people per year, with peaks in young adults and again in later adulthood.
Smoking, recent lung infections, and exposure to hydrocarbons or metal fumes can precipitate lung haemorrhage in susceptible people.
Certain genetic backgrounds (e.g., HLA-DR15) are associated with increased risk.

Signs & Symptoms

Lung involvement

Dry cough, breathlessness, chest discomfort
Haemoptysis (coughing up blood) ranging from streaks to massive bleeding
Low oxygen levels; diffuse opacities on chest imaging

Kidney involvement

Dark or bloody urine (haematuria), proteinuria
Rapid rise in urea/creatinine with oliguria; ankle swelling, hypertension
Fatigue, anorexia, nausea as renal failure develops

Red flags: new haemoptysis, rapidly worsening breathlessness, or sudden kidney dysfunction warrant immediate medical assessment.

How the Diagnosis Is Made

Serologic testing: circulating anti-GBM antibodies (ELISA/reflective assays); check ANCA as well.
Urinalysis & blood tests: haematuria, red cell casts, declining kidney function, anaemia.
Imaging & bronchoscopy: diffuse alveolar haemorrhage pattern; bronchoalveolar lavage may show increasingly bloody aliquots.
Kidney biopsy: confirms crescentic glomerulonephritis with linear IgG deposition on immunofluorescence—often the diagnostic gold standard.

Treatment (Urgent)

Prompt, coordinated therapy aims to remove pathogenic antibodies, halt new antibody formation, and support organs:

Plasma exchange (PLEX) daily or on alternate days—typically for 2–3 weeks or until anti-GBM antibodies are undetectable.
High-dose corticosteroids (e.g., IV methylprednisolone pulses followed by oral taper).
Cytotoxic/biologic immunosuppression—commonly cyclophosphamide; rituximab is considered in selected scenarios or when cyclophosphamide is unsuitable.
Supportive care: oxygen/ventilation for severe haemorrhage, blood transfusion as needed, and dialysis for renal failure.
Smoking cessation and treatment of any concurrent lung infection to reduce recurrent bleeding.

Prognosis

Lung recovery is often excellent once bleeding is controlled.
Kidney outcome depends on severity at presentation (need for dialysis, degree of crescent formation). Some patients remain dialysis-dependent.
Transplantation is feasible after disease quiescence; most centres require anti-GBM antibodies to be negative for at least 6 months to minimise recurrence risk.

Prevention & Follow-Up

Strict avoidance of smoking and pulmonary irritants.
Regular monitoring of kidney function and anti-GBM titres during and after therapy.
Vaccination, infection risk counselling, and bone-health support while on immunosuppression.

Authoritative Resource

For a patient-friendly overview, see the National Institute of Diabetes and Digestive and Kidney Diseases: NIDDK: Goodpasture’s Syndrome.

This article is for general information only and is not a substitute for professional medical advice. Seek urgent care for coughing up blood or sudden kidney problems.

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