Goodpasture syndrome is characterized by the presence of auto-antibodies against the basement membrane of the lungs and kidneys, which causes inflammatory destruction. It was first described in 1919 and can involve either or both the kidneys and lungs. The antibodies specifically target the non-collagenous domains of the alpha-3 chain of collagen type 4, initiating inflammation in the basement membranes of the kidneys and lungs. Treatment involves plasmapheresis, corticosteroids, and immunosuppressive drugs like cyclophosphamide to remove the antibodies from circulation.

1. GOODPASTURE SYNDROME
GUIDE – DR. L. S. PATIL
PRESENTER – DR. DEEPAK R. CHINAGI

2. GOODPASTURE SYNDROME
• It is a diffuse pulmonary hemorrhage
syndrome characterized by presence of
circulating auto-antibodies against the
basement membrane of lungs and kidney.
• These antibodies initiate inflammatory
destruction of basement membrane of
glomerulus and alveoli.

3. • This disease was first described by Ernest
Goodpasture (American Pathologist) in 1919.
• Both Kidney and Lung involved : 60-80% of the
cases
• Only Kidney involved : 20-40% of the cases
• Only Lung involved : 10% of the cases
• It initially presents with general constituitional
symptoms like Fever, weight loss, malaise, joint
pains.
• Incidence : 0.5 to 1.8 per Million per year.
• Europe , Asia, Maori People of New Zealand.

4. Commonly Implicated Causative
Factors
• Exposure to Organic Solvents, Tobacco Smoke,
Influenza A, Cocaine, Metal Dust.
• Gene Mutations (HLA DR15)
• Bacteremia/Sepsis
• Therapy with Monoclonal Antibodies

5. • These antibodies are also termed as Anti-GBM
antibodies (Anti Glomerular Basement
Membrane antibodies)
• These antibodies are specifically directed against
NON-COLLGENOUS DOMAINS OF α3 CHAIN OF
COLLAGEN TYPE 4.
• These antibodies initiate inflammatory
destruction of the collagen type 4 chains, which
are widely distributed in the basement
membranes of the kidney and lungs.

6. Kidney manifestations
• Anti GBM antibodies initiate proliferative RPGN
(Rapidly Progressive GlomeruloNephritis) type of
kidney injury.
• Typically seen in young and adolescent males
• Age = 5 to 40 years
• Males are more affected (6 times more common
than females)
• Clinical Features : Hematuria, Subnephrotic
Proteinuria, Nephritic Urinary Sediment, Rapidly
Progressive Renal Failure, Hypertension

7. Lung Manifestations
• Intra-Alveolar Hemorrhage
• Focal Necrosis of Alveolar Walls
• Clinical Features : Hemptysis, Diffuse Bilateral
Infiltrates, Chest pain, Breathlessness, Cough

8. Laboratory Diagnosis
• Serology – Anti GBM Antibodies detection
(seen in 90% of the cases)
• Renal Biopsy – GOLD STANDARD
– Diffuse Proliferative GlomeruloNephritis
– Focal Necrosis
– Crescent >50% of the glomeruli
• Immunoflorescence – linear deposition
• ANCA – Most often Negative (70% of the
cases)
• Chest Radiograph – Bilateral Diffuse Infiltrates

9. Treatment
• Plasmpheresis : Daily or Alternate days
• Corticosteroids – Prednisolone
• Azathioprine
• Cyclophosphamide
• Rituximab
• 5 Year Survival >80%

10. THANK YOU