Goodpasture syndrome natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Natural History

If left untreated, Goodpasture syndrome can progress to end stage renal disease and pulmonary failure. Goodpasture syndrome

Complications

Possible complications of Goodpasture syndrome include:

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

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

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

  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. Shah MK, Hugghins SY (2002). "Characteristics and outcomes of patients with Goodpasture's syndrome". South Med J. 95 (12): 1411–8. PMID 12597309.
  4. 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.
  5. 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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