Goodpasture’s Syndrome



  • Goodpasture’s Syndrome was First Described by Ernest Goodpasture in 1919
    • Initial Case Involved a Multisystem Disease Which Occurred in an 18 y/o Male (Following an Influenza Epidemic): however, questions exist as to whether the reported case actually reflected a true case of Goodpasture’s Syndrome
      • Diffuse Alveolar Hemorrhage
      • Glomerulonephritis
      • Pleuritis
      • Splenic Infarctions
      • Vasculitis of Small Intestine

Epidemiologic Features

  • Incidence: 1 case per million per year
  • Bimodal Age Peaks
    • Peak from Age 20-30: male predominance in this group
    • Peake from Age 60-70 y/o: female predominance in this age group
  • Race: more common in caucasians than in blacks

Risk Factors

  • Genetic Factors
    • Increased Frequency of HLA-DR15 (Previously Known as HLA-DR2), DRB103, and DRB104
    • Decreased Frequency of DRB101 and DRB107
    • Strong Association with the DRB11501 Allele: however, this allele is present in up to 33% of caucasian populations
    • Lesser Association with the DRB11502 Allele
  • Alemtuzumab (Campath, MabCampath, Campath-1H, Lemtrada) (see Alemtuzumab, [[Alemtuzumab]])
    • Physiology: lymphocyte-depletion therapy
  • Cocaine Exposure (see Cocaine, [[Cocaine]])
    • Epidemiology: xxx
  • Influenza A2 (and Other Respiratory, Particularly Viral) Infections (see Influenza Virus, [[Influenza Virus]])
    • Epidemiology: xxx
  • Metallic Dust Exposure
    • Epidemiology: increased risk in auto mechanics
  • Penicillamine (see Penicillamine, [[Penicillamine]]))
    • Epidemiology: case reports of drug-induced Goodpasture’s syndrome
  • Tobacco Exposure (see Tobacco, [[Tobacco]])
    • Epidemiology
      • Tobacco Smoke Exposure Increases the Risk of Diffuse Alveolar Hemorrhage (DAH): 100% of smokers with Goodpasture’s develop DAH, compared to only 20% of non-smokers with Goodpasture’s
      • Tobacco Increases the Risk of Recurrence of DAH in Cases with Prior Remission
    • Physiology: tobacco probably acts to increase alveolar-capillary membrane permeability
  • Volatile Hydrocarbon Exposure
    • Agents
      • Pesticides
      • Petroleum Products
      • Toluene
      • Turpentine




Complete Blood Count (CBC) (see Complete Blood Count, [[Complete Blood Count]])

Pulmonary Function Tests (PFT’s) (see Pulmonary Function Tests, [[Pulmonary Function Tests]])

Bronchoscopy (see Bronchoalveolar Lavage, [[Bronchoalveolar Lavage]])

Chest X-Ray (see Chest X-Ray, [[Chest X-Ray]])

Chest CT (see Chest Computed Tomography, [[Chest Computed Tomography]])

High-Resolution Chest CT (see High-Resolution Chest Computed Tomography, [[High-Resolution Chest Computed Tomography]])

Anti-Nuclear Antibody (ANA) (see Anti-Nuclear Antibody, [[Anti-Nuclear Antibody]])

Rheumatoid Factor (RF) (see Rheumatoid Factor, [[Rheumatoid Factor]])


Anti-Neutrophil Cytoplasmic Antibody (ANCA) (see Anti-Neutrophil Cytoplasmic Antibody, [[Anti-Neutrophil Cytoplasmic Antibody]])

Serum Complement (see Serum Complement, [[Serum Complement]])

Anti-Glomerular Basement Membrane Antibody (Anti-GBM) (see Anti-Glomerular Basement Membrane Antibody, [[Anti-Glomerular Basement Membrane Antibody]])

Serum Immunoglobulin A (IgA) Level (see Serum Immunoglobulin A, [[Serum Immunoglobulin A]])

Erythrocyte Sedimentation Rate (ESR) (see Erythrocyte Sedimentation Rate, [[Erythrocyte Sedimentation Rate]])

Urinalysis (see Urinalysis, [[Urinalysis]])

Clinical Manifestations

General Comments

Gastrointestinal Manifestations

Anorexia (see Anorexia, [[Anorexia]])

Hepatomegaly (see Hepatomegaly, [[Hepatomegaly]])

Nausea/Vomiting (see xxxx, [[xxxx]])

Hematologic Manifestations

Anemia (see xxxx, [[xxxx]])

Splenomegaly (see xxxx, [[xxxx]])

Otolaryngologic Manifestations

Autoimmune Inner Ear Disease (AIED)

Pulmonary Manifestations

Acute Respiratory Distress Syndrome (ARDS) (see Acute Respiratory Distress Syndrome, [[Acute Respiratory Distress Syndrome]])

Diffuse Alveolar Hemorrhage (DAH) (see Diffuse Alveolar Hemorrhage, [[Diffuse Alveolar Hemorrhage]])

Renal Manifestations

Rapidly Progressive Glomerulonephritis (see xxxx, [[xxxx]]) with/without Acute Kidney Injury (AKI)/Chronic Kidney Disease (CKD) (see Acute Kidney Injury, [[Acute Kidney Injury]] and Chronic Kidney Disease, [[Chronic Kidney Disease]])

Hypertension (see Hypertension, [[Hypertension]])

Other Manifestations


Plasmapheresis (see Plasmapheresis, [[Plasmapheresis]])

Corticosteroids and Cyclophosphamide (Cytoxan) (see Corticosteroids, [[Corticosteroids]] and Cyclophosphamide, [[Cyclophosphamide]]))

Other Therapies

Pneumocystis Jirovecii Prophylaxis (see Pneumocystis Jirovecii, [[Pneumocystis Jirovecii]])

Renal Transplantation (see Renal Transplant, [[Renal Transplant]])

Parameters to Monitor