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:

  • Infections with P. jiroveci[1]
  • Alveolar hemorrhage[2]
  • End stage renal disease
  • Pulmonary failure

Prognosis

  • Prognosis is generally good for patients with Goodpasture syndrome who receive treatment.[3]
  • The 5 survival rate of patients with Goodpasture syndrome who receive treatment is approximately 80%.
  • Recent advances in pharmacotherapy and use of immunosuppressive and plasmapheresis have further improved the outcome of patients with Goodpasture syndrome[4].
  • Today, the prognosis of Goodpasture syndrome is heavily dependent on the time of diagnosis, the start of medication, and the level of serum creatinine.[5]

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 [6]

References

  1. 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.
  2. 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.
  3. Fernandes R, Freitas S, Cunha P, Alves G, Cotter J (2016). "Goodpasture's syndrome with absence of circulating anti-glomerular basement membrane antibodies: a case report". J Med Case Rep. 10 ( ): 205. doi:10.1186/s13256-016-0984-6. PMC 4962374. PMID 27459964.
  4. Shah MK, Hugghins SY (2002). "Characteristics and outcomes of patients with Goodpasture's syndrome". South Med J. 95 (12): 1411–8. PMID 12597309.
  5. 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.
  6. 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.

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