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Goodpasture Syndrome (Anti-GBM Disease, Pulmonary-Renal Syndrome)

A rare life-threatening autoimmune disease with autoantibodies against the alpha-3 chain of type IV collagen in the glomerular and alveolar basement membranes; presents as rapidly progressive glomerulonephritis with diffuse alveolar hemorrhage; emergent treatment with plasmapheresis, cyclophosphamide, and corticosteroids saves life and preserves kidney function.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

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What is Goodpasture Syndrome (Anti-GBM Disease, Pulmonary-Renal Syndrome)?

Goodpasture syndrome is a rare but devastating autoimmune disease characterized by IgG autoantibodies (predominantly IgG1 and IgG3 subclasses) directed against an epitope (Goodpasture epitope) on the non-collagenous-1 (NC1) domain of the alpha-3 chain of type IV collagen, which is preferentially expressed in glomerular and alveolar basement membranes. Antibody binding triggers complement activation and neutrophil-mediated injury, leading to crescentic glomerulonephritis and pulmonary capillaritis with alveolar hemorrhage.

Epidemiology: incidence 0.5–1 per million per year, bimodal age distribution (peaks in 20–30 years with male predominance and DAH, and in 60–70 years with female predominance and isolated renal disease); HLA-DRB1*15:01 (DR2) confers strongest genetic susceptibility (8-fold risk); environmental triggers include smoking, hydrocarbon exposure, infections (especially influenza A and COVID-19), and lithotripsy or membranous-membrane disease.

Clinical presentation: pulmonary involvement (40–60 percent, especially in smokers and those with hydrocarbon exposure) — hemoptysis, dyspnea, anemia, bilateral alveolar infiltrates on chest imaging, hypoxemia; renal involvement (80–90 percent) — rapidly progressive glomerulonephritis with hematuria (often microscopic), red blood cell casts, proteinuria (typically subnephrotic), rising creatinine over days to weeks, often presenting with established renal failure requiring dialysis; constitutional symptoms (fatigue, malaise, weight loss). Pulmonary-renal syndrome (concurrent DAH and RPGN) is the classic presentation. Atypical presentations include isolated renal disease (20 percent — older patients), isolated pulmonary disease (5 percent — rare). Differential includes ANCA-associated vasculitis (10–30 percent of anti-GBM patients are also ANCA-positive — double-positive disease, behaves more like AAV with relapses).

Symptoms

Hemoptysis (40–60 percent — especially in smokers)
Dyspnea and cough
Anemia (often progressive over hours to days from alveolar bleeding)
Bilateral pulmonary infiltrates on chest imaging
Hypoxemia
Hematuria (microscopic or gross), red blood cell casts on urinalysis
Proteinuria (typically subnephrotic, 1–3 g/day)
Acute kidney injury with rapidly rising creatinine
Anuria or oliguria in advanced disease
Constitutional symptoms: fatigue, malaise, weight loss, fever
Symptoms develop over days to weeks

Risk Factors

HLA-DRB1*15:01 (DR2) genetic predisposition (8-fold increased risk)
Smoking (strongly associated with pulmonary involvement and hemoptysis)
Hydrocarbon exposure (occupational solvents, fuels)
Recent infection: influenza A, COVID-19, other respiratory viruses
Lithotripsy for nephrolithiasis (mechanical injury to GBM)
Anti-Thy-1 nephritis history
Membranous nephropathy (rare association)
Bimodal age (20–30 years, 60–70 years)
Male sex (in younger group with pulmonary disease)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Hemoptysis with hematuria — pulmonary-renal syndrome emergency
  • Sudden severe shortness of breath with bloody sputum
  • Rapidly rising creatinine with hematuria — emergency nephrology referral
  • Anemia with bilateral lung infiltrates and respiratory symptoms
  • Known anti-GBM disease with new symptoms (rare relapse)
  • Recent influenza or COVID-19 with new pulmonary or renal symptoms
  • Smoker with hemoptysis (urgent evaluation)
  • Hydrocarbon exposure with pulmonary or renal symptoms

Treatment Methods

01
Emergent recognition critical — anti-GBM disease has very poor renal recovery if treatment delayed beyond 1–2 days of dialysis dependency
02
Diagnostic: serum anti-GBM antibody (ELISA — diagnostic), urinalysis (RBC casts, hematuria, proteinuria), comprehensive ANCA panel (10–30 percent are double-positive — anti-GBM + ANCA, treat as AAV-anti-GBM overlap), kidney biopsy (gold standard — crescentic glomerulonephritis with linear IgG along GBM on direct immunofluorescence is pathognomonic), HRCT chest, bronchoscopy with BAL (confirms DAH, rules out infection)
03
Plasma exchange (TPE) — cornerstone of treatment, daily exchanges of 1 plasma volume with 5 percent albumin replacement (use FFP if recent renal biopsy or active bleeding) for minimum 14 sessions or until anti-GBM titers undetectable; removes circulating autoantibodies
04
Oral cyclophosphamide 2 mg/kg/day (reduce dose for elderly and renal impairment) for 3 months; or IV cyclophosphamide pulses 15 mg/kg every 2–3 weeks; suppresses new antibody production
05
High-dose corticosteroids: IV methylprednisolone 1 g/day x 3 days, then oral prednisone 1 mg/kg/day taper over 6 months
06
Rituximab as alternative or adjunct if cyclophosphamide intolerance; emerging role especially in double-positive disease
07
Treat coexisting ANCA-associated vasculitis if double-positive: rituximab as preferred induction agent, requires longer-term immunosuppression
08
Supportive care: mechanical ventilation if respiratory failure, packed red cell transfusion for severe anemia, dialysis (hemodialysis or CRRT) if renal failure, smoking cessation, avoid hydrocarbon exposure
09
Maintenance therapy: not required after induction in classical anti-GBM (relapses very rare, < 5 percent); double-positive disease behaves like AAV and requires maintenance immunosuppression
10
Long-term follow-up: anti-GBM titers monthly until undetectable then every 3 months for 1 year, urinalysis, renal function, CBC; vigilance for cyclophosphamide complications (bladder cancer screening with cystoscopy if cumulative dose > 36 g, hematologic malignancy, infertility); kidney transplant evaluation if ESRD (anti-GBM should be undetectable for 6+ months pre-transplant; recurrence in transplanted kidney very rare)

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.