Goodpasture syndrome natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2]Associate Editor(s)-in-Chief: Krzysztof Wierzbicki M.D. [3]
Overview
If left untreated, Goodpasture syndrome can progress to end stage renal disease and pulmonary failure. Complications of Goodpasture syndrome include, infections, alveolar hemorrhage, end stage renal disease, and pulmonary failure. The prognosis of Goodpasture syndrome is variable, as it depends upon the diagnosis, start of treatment and the level of serum creatinine.
Natural History
If left untreated, Goodpasture syndrome can progress to end stage renal disease and pulmonary failure.
Complications
Possible complications of Goodpasture syndrome include:
Prognosis
In the past the prognosis of Goodpasture syndrome was fatal.[3] Today, the prognosis of Goodpasture syndrome is heavily dependent on the time of diagnosis, the start of medication, and the level of serum creatinine.[4]
The following are favorable prognostic factors:
- Aggressive treatment with corticosteroids, plasmapheresis, and immunosuppressants.
- Serum creatinine of less than 5.7 mg/dL
The following are poor prognostic factors:
- Serum creatinine that is greater than 5.7 mg/dL
- Patients who require long term dialysis
- Glomerular Filtration Rate (GFR) of less than 15 mL/min
- Advanced age
- Low hemoglobin
- High white blood cell count
- Crescent formation that have extended greater than 80% of glomeruli
- ANCA and anti-GBM antibodies present together [5]
Natural History, Complications and Prognosis
Complications
- Chronic kidney disease
- Rapidly progressive glomerulonephritis
- Lung failure
- Severe pulmonary hemorrhage
Prognosis
In the 1970s, Goodpasture’s syndrome was most often fatal, but due to advances in diagnosis and treatment deaths are less common now. Death from lung hemorrhage may occur before the diagnosis has been made or in the initial stages of treatment before it has been properly controlled. With treatment, however, the patient can usually recover completely from lung damage. Kidneys, though, are less able to repair themselves and patients with kidney damage must often resort of a life on dialysis or kidney transplantation. Even with the best management there is still a significant mortality from renal failure, particularly if the patient is otherwise in poor health. It must also be remembered that the immunosuppressive treatment many patients are put on increases their risk of infection with a number of serious or fatal diseases.
References
- ↑ Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Pagliuca G; et al. (2015). "Goodpasture's syndrome: a clinical update". Autoimmun Rev. 14 (3): 246–53. doi:10.1016/j.autrev.2014.11.006. PMID 25462583.
- ↑ Panjwani AH, Deoskar RB, Falleiro J, Rajan KE (2003). "Goodpasture's Syndrome". Med J Armed Forces India. 59 (1): 77–9. doi:10.1016/S0377-1237(03)80119-3. PMC 4925784. PMID 27407468.
- ↑ Shah MK, Hugghins SY (2002). "Characteristics and outcomes of patients with Goodpasture's syndrome". South Med J. 95 (12): 1411–8. PMID 12597309.
- ↑ Moroni G, Ponticelli C (2014). "Rapidly progressive crescentic glomerulonephritis: Early treatment is a must". Autoimmun Rev. 13 (7): 723–9. doi:10.1016/j.autrev.2014.02.007. PMID 24657897.
- ↑ Levy JB, Hammad T, Coulthart A, Dougan T, Pusey CD (2004). "Clinical features and outcome of patients with both ANCA and anti-GBM antibodies". Kidney Int. 66 (4): 1535–40. doi:10.1111/j.1523-1755.2004.00917.x. PMID 15458448.