Goodpasture syndrome: Difference between revisions
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==Background== | ==Background== | ||
*Goodpasture syndrome (60%): diffuse | *Goodpasture syndrome (60%): [[diffuse alveolar hemorrhage]] + acute [[glomerulonephritis]] | ||
*Goodpasture disease: glomerulonephritis alone (30%,) pulmonary symptoms alone (10%,) | *Goodpasture disease: [[glomerulonephritis]] alone (30%,) pulmonary symptoms alone (10%,) | ||
*Anti-GBM disease is most precise term for both entities | *Anti-GBM disease is most precise term for both entities | ||
**Type II | **Type II hypersensitivity causing linear IG deposition in glomerular and alveolar BM | ||
**Triggered by:Sepsis | **Triggered by: | ||
***[[Sepsis]] | |||
***[[URI]] | |||
***[[Inhalation injury]] | |||
***Smoking | |||
***[[Pulmonary edema]] | |||
==Clinical Features== | ==Clinical Features== | ||
*Constitutional symptoms prior to pulmonary/renal complications | *Constitutional symptoms prior to pulmonary/renal complications | ||
*Pulmonary | *Pulmonary | ||
**Cough, dyspnea, chest pain, hemoptysis, respiratory failure or pulmonary hemorrhage | **[[Cough]], [[dyspnea]], [[chest pain]], [[hemoptysis]], [[respiratory failure]] or [[diffuse alveolar hemorrhage|pulmonary hemorrhage]] | ||
*Renal | *Renal: [[glomerulonephritis]] | ||
** | **[[Hematuria]], [[proteinuria]], RBC casts | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
| Line 20: | Line 25: | ||
==Evaluation== | ==Evaluation== | ||
*Pulmonary | *Pulmonary | ||
**Depends whether patient stable or not | **Depends whether patient stable or not, massive [[hemoptysis]] or not | ||
** | **Consider: [[CXR]] vs CT chest w/contrast, CBC, coags, T&S, bronchoscopy | ||
*Renal: | *Renal: | ||
*[[Urinalysis]], CBC, Chemistry, Albumin (often reduced in acute glomerulonephritis,) C3, C4, ASO, ANCA | **[[Urinalysis]], CBC, Chemistry, Albumin (often reduced in acute glomerulonephritis,) C3, C4, ASO, ANCA | ||
==Management== | ==Management== | ||
*See [[Hemoptysis]] | *See [[Hemoptysis]] | ||
*See [[Glomerulonephritis]] | **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 <ref> 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 </ref> | *[[Plasmapheresis]] shown to be beneficial <ref> 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 </ref> | ||
*In severe cases may need: | *In severe cases may need: | ||
Revision as of 16:02, 12 October 2019
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
- Renal: glomerulonephritis
- Hematuria, proteinuria, RBC casts
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
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 [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