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__NOTOC__
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{{Goodpastures syndrome }}
{{Goodpasture syndrome }}
{{CMG}}{{APM}}{{AE}}{{KW}}
{{CMG}}{{APM}}; {{AE}}{{KW}}{{Akshun}}
==Overview==
==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.
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 ==
== Natural History ==
If left untreated, Goodpasture syndrome can progress to end stage renal disease and pulmonary failure.  
*The symptoms of Goodpasture syndrome usually develop in either 20-40 or 60-70 years of age.
*The symptoms start with [[low grade fever]], [[cough]], [[malaise]], and [[joint pain]].
*If left untreated, patients with Goodpasture syndrome have a progressive increase in the severity of symptoms due to [[autoantibody]] induced tissue damage.
*Over time, the [[autoantibody]] induced [[pulmonary]] and [[renal injury]] will aggravate and may progress to [[end stage renal disease]] and [[pulmonary failure]].


== Complications ==
== Complications ==
Possible complications of Goodpasture syndrome include:
Possible complications of Goodpasture syndrome include:<ref name="pmid25462583">{{cite journal| author=Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Pagliuca G et al.| title=Goodpasture's syndrome: a clinical update. | journal=Autoimmun Rev | year= 2015 | volume= 14 | issue= 3 | pages= 246-53 | pmid=25462583 | doi=10.1016/j.autrev.2014.11.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25462583  }}</ref><ref name="pmid27407468">{{cite journal| author=Panjwani AH, Deoskar RB, Falleiro J, Rajan KE| title=Goodpasture's Syndrome. | journal=Med J Armed Forces India | year= 2003 | volume= 59 | issue= 1 | pages= 77-9 | pmid=27407468 | doi=10.1016/S0377-1237(03)80119-3 | pmc=4925784 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27407468  }}</ref>
* Infections with P. jiroveci<ref name="pmid25462583">{{cite journal| author=Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Pagliuca G et al.| title=Goodpasture's syndrome: a clinical update. | journal=Autoimmun Rev | year= 2015 | volume= 14 | issue= 3 | pages= 246-53 | pmid=25462583 | doi=10.1016/j.autrev.2014.11.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25462583  }}</ref>
* Infections with [[Pneumocystis jirovecii pneumonia|P. jiroveci]]
* Alveolar hemorrhage<ref name="pmid27407468">{{cite journal| author=Panjwani AH, Deoskar RB, Falleiro J, Rajan KE| title=Goodpasture's Syndrome. | journal=Med J Armed Forces India | year= 2003 | volume= 59 | issue= 1 | pages= 77-9 | pmid=27407468 | doi=10.1016/S0377-1237(03)80119-3 | pmc=4925784 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27407468  }}</ref>
* [[Alveolar]] [[hemorrhage]]
* End stage renal disease
* [[End stage renal disease]]
* Pulmonary failure
* [[Pulmonary failure]]


== Prognosis ==
== Prognosis ==
In the past the prognosis of Goodpasture syndrome was fatal.<ref name="pmid12597309">{{cite journal| author=Shah MK, Hugghins SY| title=Characteristics and outcomes of patients with Goodpasture's syndrome. | journal=South Med J | year= 2002 | volume= 95 | issue= 12 | pages= 1411-8 | pmid=12597309 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12597309  }}</ref> Today, the prognosis of Goodpasture syndrome is heavily dependent on the time of diagnosis, the start of medication, and the level of serum creatinine.<ref name="pmid24657897">{{cite journal| author=Moroni G, Ponticelli C| title=Rapidly progressive crescentic glomerulonephritis: Early treatment is a must. | journal=Autoimmun Rev | year= 2014 | volume= 13 | issue= 7 | pages= 723-9 | pmid=24657897 | doi=10.1016/j.autrev.2014.02.007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24657897  }}</ref>
* Prognosis is generally good for patients with Goodpasture syndrome who receive treatment.<ref name="pmid27459964">{{cite journal| author=Fernandes R, Freitas S, Cunha P, Alves G, Cotter J| title=Goodpasture's syndrome with absence of circulating anti-glomerular basement membrane antibodies: a case report. | journal=J Med Case Rep | year= 2016 | volume= 10 | issue=  | pages= 205 | pmid=27459964 | doi=10.1186/s13256-016-0984-6 | pmc=4962374 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27459964  }}</ref>
* The 5 survival rate of patients with Goodpasture syndrome who receive treatment is approximately 80%.
* Recent advances in [[pharmacotherapy]] and use of [[immunosuppressive]] agents and [[plasmapheresis]] have further improved the outcome of patients with Goodpasture syndrome<ref name="pmid12597309">{{cite journal| author=Shah MK, Hugghins SY| title=Characteristics and outcomes of patients with Goodpasture's syndrome. | journal=South Med J | year= 2002 | volume= 95 | issue= 12 | pages= 1411-8 | pmid=12597309 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12597309  }}</ref>.
* Today, the prognosis of Goodpasture syndrome is heavily dependent on the time of diagnosis, the start of medication, and the level of [[serum creatinine]].<ref name="pmid24657897">{{cite journal| author=Moroni G, Ponticelli C| title=Rapidly progressive crescentic glomerulonephritis: Early treatment is a must. | journal=Autoimmun Rev | year= 2014 | volume= 13 | issue= 7 | pages= 723-9 | pmid=24657897 | doi=10.1016/j.autrev.2014.02.007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24657897  }}</ref>


The following are favorable prognostic factors:
'''The following are favorable prognostic factors:'''<ref name="pmid15458448">{{cite journal| author=Levy JB, Hammad T, Coulthart A, Dougan T, Pusey CD| title=Clinical features and outcome of patients with both ANCA and anti-GBM antibodies. | journal=Kidney Int | year= 2004 | volume= 66 | issue= 4 | pages= 1535-40 | pmid=15458448 | doi=10.1111/j.1523-1755.2004.00917.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15458448  }}</ref>
* Aggressive treatment with corticosteroids, plasmapheresis, and immunosuppressants.  
 
* Serum creatinine of less than 5.7 mg/dL
* Aggressive treatment with [[corticosteroids]], [[plasmapheresis]], and [[immunosuppressants]].  
The following are poor prognostic factors:
* [[Serum creatinine]] of less than 5.7 mg/dL
* Serum creatinine that is greater than 5.7 mg/dL
'''The following are poor prognostic factors:'''
* Patients who require long term dialysis
* [[Serum creatinine]] that is greater than 5.7 mg/dL
* Glomerular Filtration Rate (GFR) of less than 15 mL/min
* Patients who require long term [[dialysis]]
* Glomerular Filtration Rate ([[Glomerular filtration rate|GFR]]) of less than 15 mL/min
* Advanced age
* Advanced age
* Low hemoglobin
* Low [[hemoglobin]]
* High white blood cell count
* High [[white blood cell count]]
* Crescent formation that have extended greater than 80% of glomeruli
* Crescent formation that have extended greater than 80% of [[glomeruli]]
* ANCA and anti-GBM antibodies present together <ref name="pmid15458448">{{cite journal| author=Levy JB, Hammad T, Coulthart A, Dougan T, Pusey CD| title=Clinical features and outcome of patients with both ANCA and anti-GBM antibodies. | journal=Kidney Int | year= 2004 | volume= 66 | issue= 4 | pages= 1535-40 | pmid=15458448 | doi=10.1111/j.1523-1755.2004.00917.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15458448  }}</ref>
* [[ANCA]] and [[Anti-glomerular basement membrane antibody|anti-GBM antibodies]] present together
 
==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==
==References==

Latest revision as of 14:10, 19 July 2018

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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] Akshun Kalia M.B.B.S.[4]

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

Complications

Possible complications of Goodpasture syndrome include:[1][2]

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 agents 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:[6]

The following are poor prognostic factors:

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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