Goodpasture syndrome
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Background
- Goodpasture syndrome (60%): diffuse pulmonary hemorrhage + acute glomerulonephritis.
- Goodpasture disease: glomerulonephritis alone (30%,) pulmonary sx alone (10%,)
- Anti-GBM disease is most precise term for both entities.
- Type II hypersensitivty 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
Differential Diagnosis
Hemoptysis
- Infectious
- Neoplastic
- Lung cancer
- Metastatic cancer
- Cardiovascular
- Pulmonary embolism
- Congestive heart failure
- Pulmonary hypertension
- AV malformation
- Mitral stenosis
- Alveolar hemorrhage syndromes
- Hematologic
- Uremia
- Platelet dysfunction (ASA, clopidogrel)
- Anticoagulant therapy
- Traumatic
- Foreign body aspiration
- Ruptured bronchus
- Inflammatory
- 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
Diagnosis
- Pulmonary
- Depends whether pt stable or not
- See hemoptysis
- Renal:
- UA, CBC, Chemistry, Albumin (often reduced in acute glomerulonephritis,) C3, C4, ASO, ANCA
Management
- See Hemoptysis
- See Glomerulonephritis
- Plasmapharesis shown to be beneficial [1]
- In severe cases may need:
- Rheumatology eval: Immunosuppressants
Disposition
Admit for:
- Decompensated disease
- Massive hemoptysis
- Acute glomerulonephritis
See Also
External Links
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
- ↑ Levy JB, Turner AN, Rees AJ, Pusey CD. Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression. Ann Intern Med. 2001 Jun 5. 134(11):1033-42