Goodpasture syndrome: Difference between revisions

Goodpasture syndrome
Synonyms	Goodpasture’s disease, antiglomerular basement antibody disease, anti-GBM disease
Pronounce
Field	Nephrology, Pulmonology, Rheumatology
Symptoms	Hemoptysis, cough, shortness of breath, hematuria
Complications	Lung hemorrhage, kidney failure
Onset	Typically young adults or elderly individuals
Duration	Chronic (manageable with treatment)
Types
Causes	Autoantibodies against basement membrane antigens
Risks	Genetic predisposition, environmental factors (e.g., smoking, exposure to hydrocarbons)
Diagnosis	Kidney biopsy, detection of anti-GBM antibodies, chest X-ray
Differential diagnosis	Granulomatosis with polyangiitis, systemic lupus erythematosus, other forms of glomerulonephritis
Prevention	Avoidance of risk factors (e.g., smoking cessation)
Treatment	Immunosuppressive therapy, plasmapheresis
Medication	Corticosteroids, cyclophosphamide
Prognosis	Variable, depends on early diagnosis and treatment
Frequency	Rare (~1 per million annually)
Deaths	Rare with prompt treatment

Latest revision as of 03:54, 27 March 2025

Rare autoimmune disease

Antibody structure relevant in autoimmune diseases like Goodpasture syndrome

Goodpasture syndrome (GPS), also known as anti-glomerular basement membrane disease, is a rare autoimmune disease characterized by the presence of antibodies that target the basement membrane in the lungs and kidneys. This immune attack leads to serious complications including pulmonary hemorrhage and rapidly progressive kidney failure.

Signs and Symptoms[edit]

Typical clinical features include:

Hemoptysis (coughing up blood)
Shortness of breath
Fatigue
Pallor
Hematuria (blood in urine)
Edema due to renal impairment

Pathophysiology[edit]

Goodpasture syndrome occurs when the immune system mistakenly produces antibodies directed against collagen type IV, a key structural component of the basement membranes in pulmonary alveoli and renal glomeruli. This antibody attack results in inflammation and damage, leading to bleeding within the lungs and impaired kidney function.

Diagnosis[edit]

Diagnosis typically involves:

Detection of anti-glomerular basement membrane (anti-GBM) antibodies in blood tests
Kidney biopsy showing crescentic glomerulonephritis
Chest imaging (X-ray or CT scan) showing signs of pulmonary hemorrhage

Treatment[edit]

Treatment strategies include:

Plasmapheresis to remove circulating anti-GBM antibodies
Immunosuppressive medications, including corticosteroids and cyclophosphamide, to reduce antibody production and inflammation
Supportive therapy for kidney and lung function

Prognosis[edit]

The prognosis varies widely depending on how quickly the diagnosis is made and treatment initiated. Early intervention can lead to good outcomes, whereas delayed diagnosis or severe presentation increases the risk of irreversible kidney damage or respiratory failure.

Prevention[edit]

Primary preventive measures include reducing exposure to known risk factors, notably smoking and occupational exposure to hydrocarbons or solvents.

Epidemiology[edit]

Goodpasture syndrome is rare, with an estimated incidence of approximately 1 case per million individuals annually. It predominantly affects young adults and older individuals.

External links[edit]

GBM antibodies: immunofluorescence image

‹ The template below (Hypersensitivity and autoimmune diseases) is being considered for merging with Allergic conditions. See templates for discussion to help reach a consensus. ›

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@@ Line 1: / Line 1: @@
-{{redirect|Goodpasture}}
 {{short description|Rare autoimmune disease}}
 {{Infobox medical condition (new)
@@ Line 5: / Line 4: @@
 | synonyms        = Goodpasture’s disease, antiglomerular basement antibody disease, anti-GBM disease
 | image           = Crescentic glomerulonephritis - high mag.jpg
-| caption         = [[Micrograph]] of a [[crescentic glomerulonephritis]] that was shown to be antiglomerular basement membrane disease, [[PAS stain]]
+| caption         = [[Micrograph]] showing [[crescentic glomerulonephritis]], characteristic of Goodpasture syndrome ([[PAS stain]])
 | pronounce       =
-| field           =
+| field           = Nephrology, Pulmonology, Rheumatology
-| symptoms        =
+| symptoms        = Hemoptysis, cough, shortness of breath, hematuria
-| complications   =
+| complications   = Lung hemorrhage, kidney failure
-| onset           =
+| onset           = Typically young adults or elderly individuals
-| duration        =
+| duration        = Chronic (manageable with treatment)
 | types           =
-| causes          =
+| causes          = Autoantibodies against basement membrane antigens
-| risks           =
+| risks           = Genetic predisposition, environmental factors (e.g., smoking, exposure to hydrocarbons)
-| diagnosis       =
+| diagnosis       = Kidney biopsy, detection of anti-GBM antibodies, chest X-ray
-| differential    =
+| differential    = Granulomatosis with polyangiitis, systemic lupus erythematosus, other forms of glomerulonephritis
-| prevention      =
+| prevention      = Avoidance of risk factors (e.g., smoking cessation)
-| treatment       =
+| treatment       = Immunosuppressive therapy, plasmapheresis
-| medication      =
+| medication      = Corticosteroids, cyclophosphamide
-| prognosis       =
+| prognosis       = Variable, depends on early diagnosis and treatment
-| frequency       =
+| frequency       = Rare (~1 per million annually)
-| deaths          =
+| deaths          = Rare with prompt treatment
 }}
-[[File:Antibody.JPG|thumb|antibody image]]
+[[File:Antibody.JPG|thumb|Antibody structure relevant in autoimmune diseases like Goodpasture syndrome]]
-'''Goodpasture syndrome (GPS)''', also known as '''anti-glomerular basement membrane disease''',  is a [[rare disease|rare]] [[autoimmune disease]] in which antibodies attack the [[basement membrane]] in lungs and kidneys, leading to bleeding from the lungs and [[kidney failure]].<ref>{{cite journal|last=Thibaud|first=V.|last2=Rioux-Leclercq|first2=N.|last3=Vigneau|first3=C.|last4=Morice|first4=S.|date=December 2019|title=Recurrence of Goodpasture syndrome without circulating anti-glomerular basement membrane antibodies after kidney transplant, a case report|url=https://bmcnephrol.biomedcentral.com/articles/10.1186/s12882-018-1197-6|journal=BMC Nephrology|language=en|volume=20|issue=1|pages=6|doi=10.1186/s12882-018-1197-6|issn=1471-2369|pmc=6323659|pmid=30621605}}</ref> It is thought to attack the alpha-3 subunit of [[type IV collagen]], which has therefore been referred to as Goodpasture's antigen.<ref>{{Cite web | url=http://ghr.nlm.nih.gov/gene/COL4A3 | title=COL4A3 gene}}</ref> Goodpasture syndrome may quickly result in permanent lung and kidney damage, often leading to death. It is treated with [[immunosuppressant drugs|medications that suppress the immune system]] such as [[corticosteroid]]s and [[cyclophosphamide]], and with [[plasmapheresis]], in which the [[antibodies]] are removed from the blood.
+'''Goodpasture syndrome (GPS)''', also known as '''anti-glomerular basement membrane disease''', is a [[rare disease|rare]] [[autoimmune disease]] characterized by the presence of antibodies that target the [[basement membrane]] in the lungs and kidneys. This immune attack leads to serious complications including pulmonary hemorrhage and rapidly progressive [[kidney failure]].
-The disease was first described by an American pathologist [[Ernest Goodpasture]] of [[Vanderbilt University]] in 1919 and was later named in his honor.<ref>{{cite journal | author=Goodpasture EW | title=The significance of certain pulmonary lesions in relation to the etiology of influenza | journal=Am J Med Sci | year=1919 | volume=158 | pages=863–870 | doi=10.1097/00000441-191911000-00012 | issue=6| url=https://zenodo.org/record/1431949 }}</ref><ref>{{cite journal |vauthors=Salama AD, Levy JB, Lightstone L, Pusey CD |title=Goodpasture's disease |journal=Lancet |volume=358 |issue=9285 |pages=917–920 |date=September 2001 |pmid=11567730 |doi=10.1016/S0140-6736(01)06077-9}}</ref>
+==Signs and Symptoms==
+Typical clinical features include:
+* Hemoptysis (coughing up blood)
+* Shortness of breath
+* Fatigue
+* Pallor
+* Hematuria (blood in urine)
+* Edema due to renal impairment
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@@ Line 39: / Line 45: @@
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 </youtube>
-==Signs and symptoms==
-The anti[[glomerular basement membrane]] (GBM) [[antibodies]] primarily attack the kidneys and lungs, although, generalized symptoms like malaise, weight loss, fatigue, fever, and chills are also common, as are joint aches and pains.<ref name = PMSR>{{cite web|title=Goodpasture Syndrome Clinical Presentation|work=Medscape Reference|publisher=WebMD|date=21 May 2013|accessdate=14 March 2014|url=http://emedicine.medscape.com/article/240556-clinical#showall|editor=Batuman, C|author1=Kathuria, P |author2=Sanghera, P |author3=Stevenson, FT |author4=Sharma, S |author5=Lederer, E |author6=Lohr, JW |author7=Talavera, F |author8=Verrelli, M }}</ref> 60 to 80% of those with the condition experience both lung and kidney involvement; 20-40% have kidney involvement alone, and less than 10% have lung involvement alone.<ref name = PMSR/> Lung symptoms usually antedate kidney symptoms and usually include: [[hemoptysis|coughing up blood]], chest pain (in less than 50% of cases overall), cough, and [[dyspnoea|shortness of breath]].<ref name = MM>{{cite web|title=Goodpasture Syndrome: Diffuse Alveolar Hemorrhage and Pulmonary-Renal Syndrome|work=Merck Manual Professional|date=November 2013|accessdate=14 March 2014|url=http://www.merckmanuals.com/professional/pulmonary_disorders/diffuse_alveolar_hemorrhage_and_pulmonary-renal_syndrome/goodpasture_syndrome.html|author=Schwarz, MI}}</ref> Kidney symptoms usually include [[hematuria|blood in the urine]], [[proteinuria|protein in the urine]], [[edema|unexplained swelling of limbs or face]], [[uremia|high amounts of urea in the blood]], and [[hypertension|high blood pressure]].<ref name = PMSR/>
-==Cause==
-Its precise cause is unknown, but an insult to the blood vessels taking blood from and to the lungs is believed to be required to allow the anti-GBM antibodies to come into contact with the [[Pulmonary alveolus|alveoli]].<ref name = MSR>{{cite web|title=Goodpasture Syndrome|work=Medscape Reference|publisher=WebMD|date=21 May 2013|accessdate=14 March 2014|url=http://emedicine.medscape.com/article/240556-overview#showall|editor=Batuman, C|author1=Kathuria, P |author2=Sanghera, P |author3=Stevenson, FT |author4=Sharma, S |author5=Lederer, E |author6=Lohr, JW |author7=Talavera, F |author8=Verrelli, M }}</ref> Examples of such an insult include:<ref name = MSR/>
-exposure to organic solvents (e.g. [[chloroform]]) or hydrocarbons,
-exposure to [[tobacco]] smoke,
-certain alleles (''[[HLA-DR15]]''),
-infection (such as [[influenza A]]),
-[[cocaine]] inhalation,
-metal dust inhalation,
-[[bacteraemia]],
-[[sepsis]],
-high-oxygen environments, and
-treatment with antilymphocytic treatment (especially [[monoclonal antibodies]]).
 ==Pathophysiology==
-GPS is caused by abnormal [[plasma cells|plasma cell]] production of anti-GBM antibodies.<ref name = MSR/> The anti-GBM antibodies attack the alveoli and [[glomeruli]] basement membranes.<ref name = MSR/> These antibodies bind their reactive [[epitopes]] to the basement membranes and activate the complement cascade, leading to the death of tagged cells.<ref name = MSR/> [[T cells]] are also implicated, though it is generally considered a type II hypersensitivity reaction.<ref name = MSR/>
+Goodpasture syndrome occurs when the immune system mistakenly produces antibodies directed against collagen type IV, a key structural component of the basement membranes in pulmonary alveoli and renal glomeruli. This antibody attack results in inflammation and damage, leading to bleeding within the lungs and impaired kidney function.
 ==Diagnosis==
-The diagnosis of GPS is often difficult, as numerous other diseases can cause the various manifestations of the condition and the condition itself is rare.<ref name = WMSR>{{cite web|title=Goodpasture Syndrome Workup|work=Medscape Reference|publisher=WebMD|date=21 May 2013|accessdate=14 March 2014|url=http://emedicine.medscape.com/article/240556-workup#showall|editor=Batuman, C|author1=Kathuria, P |author2=Sanghera, P |author3=Stevenson, FT |author4=Sharma, S |author5=Lederer, E |author6=Lohr, JW |author7=Talavera, F |author8=Verrelli, M }}</ref> The most accurate means of achieving the diagnosis is testing the affected tissues by means of a [[biopsy]], especially the kidney, as it is the best-studied organ for obtaining a sample for the presence of anti-GBM antibodies.<ref name = WMSR/> On top of the anti-GBM antibodies implicated in the disease, about one in three of those affected also has [[c-ANCA|cytoplasmic antineutrophilic antibodies]] in their bloodstream, which often predates the anti-GBM antibodies by about a few months or even years.<ref name = WMSR/> The later the disease is diagnosed, the worse the outcome is for the affected person.<ref name = MSR/>
+Diagnosis typically involves:
+* Detection of anti-glomerular basement membrane (anti-GBM) antibodies in blood tests
+* Kidney biopsy showing crescentic glomerulonephritis
+* Chest imaging (X-ray or CT scan) showing signs of pulmonary hemorrhage
 ==Treatment==
-The major mainstay of treatment for GPS is [[plasmapheresis]], a procedure in which the affected person's blood is sent through a [[centrifuge]] and the various components separated based on weight.<ref name = TMSR/> The [[Blood plasma|plasma]] contains the anti-GBM antibodies that attack the affected person's lungs and kidneys, and is filtered out.<ref name = TMSR/> The other parts of the blood (the [[red blood cells]], [[white blood cells]], and [[platelets]]) are recycled and intravenously reinfused.<ref name = TMSR>{{cite web|title=Goodpasture Syndrome Treatment & Management|work=Medscape Reference|publisher=WebMD|date=21 May 2013|accessdate=14 March 2014|url=http://emedicine.medscape.com/article/240556-treatment#showall|editor=Batuman, C|author1=Kathuria, P |author2=Sanghera, P |author3=Stevenson, FT |author4=Sharma, S |author5=Lederer, E |author6=Lohr, JW |author7=Talavera, F |author8=Verrelli, M }}</ref> Most individuals affected by the disease also need to be treated with [[immunosuppressant]] drugs, especially [[cyclophosphamide]], [[prednisone]], and [[rituximab]], to prevent the formation of new [[Anti-glomerular basement membrane antibody|anti-GBM]] antibodies so as to prevent further damage to the kidneys and lungs.<ref name = TMSR/> Other, less toxic immunosuppressants such as [[azathioprine]] may be used to maintain remission.<ref name = TMSR/>
+Treatment strategies include:
+* '''Plasmapheresis''' to remove circulating anti-GBM antibodies
+* Immunosuppressive medications, including corticosteroids and cyclophosphamide, to reduce antibody production and inflammation
+* Supportive therapy for kidney and lung function
 ==Prognosis==
-With treatment, the five-year survival rate is >80% and fewer than 30% of affected individuals require long-term dialysis.<ref name = MSR/> A study performed in [[Australia]] and [[New Zealand]] demonstrated that in patients requiring renal replacement therapy (including dialysis) the median survival time is 5.93 years.<ref name = MSR/> Without treatment, virtually every affected person will die from either advanced kidney failure or lung hemorrhages.<ref name = MSR/>
+The prognosis varies widely depending on how quickly the diagnosis is made and treatment initiated. Early intervention can lead to good outcomes, whereas delayed diagnosis or severe presentation increases the risk of irreversible kidney damage or respiratory failure.
+==Prevention==
+Primary preventive measures include reducing exposure to known risk factors, notably smoking and occupational exposure to hydrocarbons or solvents.
 ==Epidemiology==
-GPS is rare, affecting about 0.5–1.8 per million people per year in Europe and Asia.<ref name = MSR/> It is also unusual among [[autoimmune disease]]s in that it is more common in males than in females and is also less common in blacks than whites, but more common in the [[Māori people]] of New Zealand.<ref name = MSR/> The peak age ranges for the onset of the disease are 20–30 and 60–70 years.<ref name = MSR/>
+Goodpasture syndrome is rare, with an estimated incidence of approximately 1 case per million individuals annually. It predominantly affects young adults and older individuals.
-==See also==
+==See Also==
+* '''[[Autoimmune disease]]'''
+* '''[[Glomerulonephritis]]'''
+* '''[[Pulmonary hemorrhage]]'''
+* '''[[Kidney failure]]'''
+* '''[[Plasmapheresis]]'''
 * {{sectionlink|HLA-DR|DR2}}
 * [[Pulmonary-renal syndrome]]
-==References==
-{{Reflist}}
 == External links ==
@@ Line 97: / Line 98: @@
 {{Collagen disease}}
 {{Urologic disease}}
 [[Category:Autoimmune diseases]]
 [[Category:Kidney diseases]]

Goodpasture syndrome

Synonyms	Goodpasture’s disease, antiglomerular basement antibody disease, anti-GBM disease
Pronounce
Field	Nephrology, Pulmonology, Rheumatology
Symptoms	Hemoptysis, cough, shortness of breath, hematuria
Complications	Lung hemorrhage, kidney failure
Onset	Typically young adults or elderly individuals
Duration	Chronic (manageable with treatment)
Types
Causes	Autoantibodies against basement membrane antigens
Risks	Genetic predisposition, environmental factors (e.g., smoking, exposure to hydrocarbons)
Diagnosis	Kidney biopsy, detection of anti-GBM antibodies, chest X-ray
Differential diagnosis	Granulomatosis with polyangiitis, systemic lupus erythematosus, other forms of glomerulonephritis
Prevention	Avoidance of risk factors (e.g., smoking cessation)
Treatment	Immunosuppressive therapy, plasmapheresis
Medication	Corticosteroids, cyclophosphamide
Prognosis	Variable, depends on early diagnosis and treatment
Frequency	Rare (~1 per million annually)
Deaths	Rare with prompt treatment