Goodpasture syndrome

Background

• Goodpasture syndrome (60%): diffuse alveolar hemorrhage + acute glomerulonephritis
• Goodpasture disease: glomerulonephritis alone (30%,) pulmonary symptoms alone (10%,)
• Anti-GBM disease is most precise term for both entities
  • Type II hypersensitivity causing linear IG deposition in glomerular and alveolar BM
  • Triggered by:
    • Sepsis
    • URI
    • Inhalation injury
    • Smoking
    • Pulmonary edema

Clinical Features

• Constitutional symptoms prior to pulmonary/renal complications
• Pulmonary
  • Cough, dyspnea, chest pain, hemoptysis, respiratory failure or pulmonary hemorrhage
• Renal: glomerulonephritis
  • Hematuria, proteinuria, RBC casts
• Symptoms are often rapidly progressive

Differential Diagnosis

Hemoptysis

• Infectious
  • Bronchitis
  • Pneumonia
  • Lung abscess
  • Tuberculosis
  • Plague
  • Aspergilloma
  • Coccidioidomycosis
• Neoplastic
  • Lung cancer
  • Metastatic cancer
• Cardiovascular
  • Pulmonary embolism
  • Congestive heart failure
  • Pulmonary hypertension
  • AV malformation
• Mitral stenosis
• Alveolar hemorrhage syndromes
  • Goodpasture syndrome
  • Granulomatosis with polyangiitis (Wegener)
  • SLE
• Hematologic
  • Uremia
  • Platelet dysfunction (ASA, clopidogrel)
  • Anticoagulant therapy
• Traumatic
  • Foreign body aspiration
  • Ruptured bronchus
• Inflammatory
  • Bronchiectasis
  • Cystic fibrosis
• Miscellaneous
  • Cocaine inhalation (crack lung)
  • Catamenial pneumothorax
  • Goodpasture syndrome
  • Cystic fibrosis
  • Epistaxis
  • Blood-laced mucus from the sinus or nose area
  • Upper GI bleeding

Causes of Glomerulonephritis

• Poststreptococcal glomerulonephritis
• Hemolytic-uremic syndrome
• Henoch-Schonlein purpura
• IgA nephropathy
• Lupus nephritis
• Alport syndrome
• Goodpasture syndrome
• Paraneoplastic

Evaluation

• Pulmonary
  • Depends whether patient stable or not, massive hemoptysis or not
  • Consider: CXR vs CT chest w/contrast, CBC, coags, T&S, bronchoscopy
• Renal:
  • Urinalysis, CBC, Chemistry, Albumin (often reduced in acute glomerulonephritis,) C3, C4, ASO, ANCA

Management

• See Hemoptysis
  • Massive: intubate (ideally ETT >8.0), angio or bronch to identify and stop localized source, consider nebulized TXA
  • Correct coagulopathy
  • Methylprednisolone 1g + plasmapheresis
• See Glomerulonephritis, consult nephrology
• Plasmapheresis shown to be beneficial
• In severe cases may need:
  • Rheumatology eval: Immunosuppressants

Disposition

Admit for:

• Decompensated disease
• Massive hemoptysis
• Acute glomerulonephritis

References

• Chan AL, Louie S, Leslie KO, Juarez MM, Albertson TE. Cutting edge issues in Goodpasture's disease. Clin Rev Allergy Immunol. 2011 Oct. 41(2):151-62.
• Kathuria, P; Sanghera, P; Stevenson, FT; Sharma, S; Lederer, E; Lohr, JW; Talavera, F; Verrelli, M (21 May 2013). Batuman, C, ed. "Goodpasture Syndrome". Medscape Reference. WebMD. Retrieved 23 Jan 2016