DISEASE SCANNER

Global Incurable Diseases Tracker

Back to Globe
Nephrologic Disease

Goodpasture Syndrome (Anti-GBM Disease)

HIGH SEVERITY

A rare autoimmune disease where antibodies attack the basement membrane in lungs and kidneys, causing rapidly progressive glomerulonephritis and pulmonary hemorrhage. Medical emergency with high mortality if untreated.

Global Affected

20.0K

Countries

15

Symptoms

Pulmonary hemorrhage (coughing blood)
Dyspnea
Fatigue
Hematuria
Proteinuria
Rapid kidney function decline
Anemia
Chest pain

Treatment Options

Plasma exchange (daily initially)
High-dose corticosteroids
Cyclophosphamide
Rituximab
Supportive care (oxygen, transfusion)
Dialysis
Kidney transplantation

Risk Factors

1HLA-DRB1*1502 allele
2Smoking (lung involvement)
3Hydrocarbon exposure
4Infections
5Male sex
6Young adults and older adults (bimodal)

Diagnostic Methods

  • 1Anti-GBM antibody testing
  • 2Kidney biopsy (linear IgG deposition)
  • 3Chest X-ray
  • 4Urinalysis
  • 5Blood gas analysis

Prognosis

Without treatment, almost universally fatal. With prompt plasma exchange: 50-80% kidney recovery if creatinine <5.7 mg/dL at presentation. Lung recovery usually complete.

Prevention

  • Smoking cessation
  • Avoid hydrocarbon exposure
  • Early treatment of respiratory symptoms in at-risk individuals

Research Status

Medical emergency. Plasmapheresis plus immunosuppression (steroids, cyclophosphamide) standard. Rituximab emerging as alternative. Early treatment essential for kidney recovery.

Affected Countries

AustraliaBrazilCanadaChinaGermanySpainFranceUnited KingdomIndiaItalyJapanMexicoNigeriaUnited StatesSouth Africa

Sources

  • https://www.kidney.org/atoz/content/goodpasture
  • https://www.ncbi.nlm.nih.gov/books/NBK441848
  • https://rarediseases.org/rare-diseases/goodpasture-syndrome

Medical Disclaimer

This information is for educational purposes only. Always consult healthcare professionals for medical advice, diagnosis, and treatment.