Goodpasture syndrome natural history, complications and prognosis: Difference between revisions

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Latest revision as of 14:10, 19 July 2018

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]

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.

Template:WH Template:WS

[pmid25462583-1] 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.

[pmid27407468-2] 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.

[pmid27459964-3] 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.

[pmid12597309-4] Shah MK, Hugghins SY (2002). "Characteristics and outcomes of patients with Goodpasture's syndrome". South Med J. 95 (12): 1411–8. PMID 12597309.

[pmid24657897-5] 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.

[pmid15458448-6] 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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@@ Line 1: / Line 1: @@
 __NOTOC__
-{{Goodpastures syndrome }}
+{{Goodpasture syndrome }}
-{{CMG}}{{APM}}{{AE}}{{KW}}
+{{CMG}}{{APM}}; {{AE}}{{KW}}{{Akshun}}
 ==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 ==
-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 ==
-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 ==
-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]].
+* [[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
+* [[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
-* 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
-The current 5 year survival rate of Goodpasture syndrome is greater than 80% and patients requiring long-term renal dialysis less than 30% in advent to earlier diagnosis and aggressive treatment regimen. <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 overview section should include the disease name in the first sentence.
-* The goal is to summarize the entire page in several sentences. This section can be the same as the natural history, complications and prognosis segment on the overview page.
-* To see an example, click [[Pericarditis natural history#Overview|here]].
-===Template===
-*'''First Sentences:'''
-:If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3]. Common complications of [disease name] include [complication 1], [complication 2], and [complication 3]. Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
-:OR
-:Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary.  However, the prognosis is generally regarded as poor/good/excellent.
-*'''Examples:'''
-:Example 1: If left untreated, 20% to 30% of patients with IgA nephropathy may progress to develop ESRD. Common complications of IgA nephropathy include pro-thrombotic states, such as stroke and myocardial infarction. Prognosis is generally good, and the 5-year mortality rate of patients with IgA nephropathy is approximately 5%.
-*'''Additional Sentences:'''
-:[Disease/malignancy] is associated with a 5 year survival rate of [#]%.
-:The presence of metastasis is associated with a particularly poor prognosis among patients with [disease/malignancy]. The 5 year event free survival rate is less than [#]%.
-:The [Subtype of disease or malignancy] is associated with the most favorable prognosis.
-:The prognosis varies with the [characteristic] of tumor: [subtype of disease/malignancy] have the most favorable prognosis.
-*'''Examples:'''
-:Example 1: Rhabdomyosarcoma is associated with a 5 year survival rate of 72%.
-:Example 2: The presence of metastasis is associated with a particularly poor prognosis among patients with rhabdomyosarcoma. The 5 year event free survival rate is less than 30%.
-:Example 3: The embryonal subtype of rhabdomyosarcoma is associated with the most favorable prognosis.
-:Example 4: The prognosis varies with the location of tumor: orbital and genitourinary tract rhabdomyosarcomas have the most favorable prognosis.
-==Preferred Template Statements==
-*If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
-*Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
-*Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
-*Depending on the extent of the [tumor/disease progression/etc.] at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor/good/excellent.
-===Additional Sentences===
-*The presence of [characteristic of disease] is associated with a particularly [good/poor] prognosis among patients with [disease/malignancy].
-*[Subtype of disease/malignancy] is associated with the most favorable prognosis.
-*The prognosis varies with the [characteristic] of tumor; [subtype of disease/malignancy] have the most favorable prognosis.
-==Natural History==
-* The natural history of disease details how the disease progresses without treatment.
-* Here are a few template sentences you can use: "The symptoms of (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___. The symptoms of (disease name) typically develop ___ years after exposure to ___. Without treatment, the patient will develop symptoms of ___, which will/ may eventually lead to ___.
-==Complications==
-* Using lists can be helpful for describing this section.
-* You can use these template sentences;
-**"Complications that can develop as a result of (disease name) are ___ (describe in list form)".
-**"Complications that can develop as a result of the treatment of (disease name) are ___ (describe in list form).
-** Next to each complication, provide a brief one sentence description detailing the complication.
-* For an example of the complications section in a natural history, complications and prognosis page, click [[Pericarditis natural history#complications|here]].
-==Prognosis==
-* This section should detail the prognosis of the disease, both treated and untreated.
-* Here are some template sentences; "The prognosis of (disease name) is poor/good with treatment. Without treatment, (disease name) will result in ___. (Disease name) is associated with a 1/5/10 year mortality of __ among patient with ______ (for example high grade lesions). The presence of ___ is associated with a particularly poor prognosis among patients with (disease name).
-* For an example of a prognosis section within a natural history, complications and prognosis page, click [[Pericarditis natural history#prognosis|here]].
-==References==
-* References should be cited for the material that you have put on your page. Type in <nowiki>{{reflist|2}}</nowiki>.This will generate your references in small font, in two columns, with links to the original article and abstract.
-* For information on how to add references into your page, click [[Help:Adding References to Articles|here]].
 ==References==