Goodpasture syndrome natural history, complications and prognosis: Difference between revisions
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== Natural History == | == Natural History == | ||
*The symptoms of Goodpasture syndrome usually develop in either 20-40 or 60-70 years of age. | *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. | *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 | *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 | *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:<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> | 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 | * Infections with [[Pneumocystis jirovecii pneumonia|P. jiroveci]] | ||
* Alveolar hemorrhage | * Alveolar [[hemorrhage]] | ||
* End stage renal disease | * [[End stage renal disease]] | ||
* Pulmonary failure | * [[Pulmonary failure]] | ||
== Prognosis == | == Prognosis == | ||
* 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> | * 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%. | * The 5 survival rate of patients with Goodpasture syndrome who receive treatment is approximately 80%. | ||
* Recent advances in pharmacotherapy and use of | * 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> | * 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:'''<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> | '''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. | * Aggressive treatment with [[corticosteroids]], [[plasmapheresis]], and [[immunosuppressants]]. | ||
* Serum creatinine of less than 5.7 mg/dL | * [[Serum creatinine]] of less than 5.7 mg/dL | ||
'''The following are poor prognostic factors:''' | '''The following are poor prognostic factors:''' | ||
* Serum creatinine that is greater than 5.7 mg/dL | * [[Serum creatinine]] that is greater than 5.7 mg/dL | ||
* Patients who require long term dialysis | * Patients who require long term [[dialysis]] | ||
* Glomerular Filtration Rate (GFR) of less than 15 mL/min | * 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 | * [[ANCA]] and [[Anti-glomerular basement membrane antibody|anti-GBM antibodies]] present together | ||
==References== | ==References== | ||
Revision as of 14:50, 1 May 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
- 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
Possible complications of Goodpasture syndrome include:[1][2]
- Infections with P. jiroveci
- Alveolar hemorrhage
- 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 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]
- 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
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.
- ↑ 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.
- ↑ 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.