MCID: GDP001
MIFTS: 53

Goodpasture Syndrome malady

Rare diseases, Nephrological diseases, Respiratory diseases, Immune diseases categories
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Summaries for Goodpasture Syndrome

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NIH Rare Diseases:42 Goodpasture syndrome is an autoimmune disease of unknown cause that affects the lungs and kidneys. the typical symptoms of goodpasture syndrome include pulmonary alveolar hemorrhage (bleeding in the lungs), glomerulonephritis, and the presence of anti-glomerular basement membranes (anti-gbm) antibodies in the blood. last updated: 9/17/2008

MalaCards based summary: Goodpasture Syndrome, also known as anti-glomerular basement membrane disease, is related to nephritis and glomerulonephritis, and has symptoms including lung/pulmonary infiltrates, thoracic/chest pain and respiratory distress/dyspnea/respiratory failure/lung volume reduction. An important gene associated with Goodpasture Syndrome is COL4A3 (collagen, type IV, alpha 3 (Goodpasture antigen)), and among its related pathways are Degradation of the extracellular matrix and Cell adhesion ECM remodeling. The compounds heparin and serine have been mentioned in the context of this disorder. Affiliated tissues include kidney, lung and kidney, and related mouse phenotypes are other and muscle.

Disease Ontology:8 A hypersensitivity reaction type ii disease that is characterized by glomerulonephritis located in kidney and hemorrhaging located in lung.

Wikipedia:65 Goodpasture syndrome (GPS; also known as Goodpasture?s disease, anti-glomerular basement antibody... more...

Description from OMIM:46 233450

Aliases & Classifications for Goodpasture Syndrome

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Sources:
8Disease Ontology, 42NIH Rare Diseases, 10DISEASES, 44Novoseek, 48Orphanet, 46OMIM, 62UMLS, 57SNOMED-CT, 27ICD9CM, 34MeSH, 39NCIt, 26ICD10 via Orphanet, 63UMLS via Orphanet
See all sources

Goodpasture Syndrome, Aliases & Descriptions:

Name: Goodpasture Syndrome 8 42 10 44 48
Anti-Glomerular Basement Membrane Disease 8 48 46 62
Rapidly Progressive Glomerulonephritis with Pulmonary Hemorrhage 42 62
Glomerulonephritis - Pulmonary Hemorrhage 42 62
 
Anti-Glomerular Basement Membrane Antibody Disease 42
Anti Glomerular Basement Membrane Antibody Disease 62
Pulmonary Renal Syndrome 42
Anti-Gbm Syndrome 48


Classifications:



Characteristics (Orphanet epidemiological data):

48
goodpasture syndrome:
Inheritance: Sporadic; Age of onset: Adolescence / Young adulthood


External Ids:

Disease Ontology8 DOID:9808
ICD9CM27 446.21
MeSH34 D019867
OMIM46 233450
NCIt39 C34649
ICD10 via Orphanet26 M31.0+, N08.5*
UMLS via Orphanet63 C0403529

Related Diseases for Goodpasture Syndrome

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Graphical network of the top 20 diseases related to Goodpasture Syndrome:



Diseases related to goodpasture syndrome

Symptoms for Goodpasture Syndrome

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Symptoms by clinical synopsis from OMIM:

233450

Clinical features from OMIM:

233450

Symptoms:

48 (show all 19)
  • lung/pulmonary infiltrates
  • thoracic/chest pain
  • respiratory distress/dyspnea/respiratory failure/lung volume reduction
  • cough
  • hemoptysis
  • lung/pulmonary/alveolar hemorrhage/hematoma
  • vascularitis/vasculitides/arteritis
  • renal glomerular defect/glomerulopathy
  • proteinuria
  • autoimmunity/autoimmune reaction/autoantibodies
  • anaemia
  • hematuria/microhematuria
  • retinal detachment
  • purpura/petichiae
  • renal failure
  • myalgia/muscular pain
  • articular/joint pain/arthralgia
  • arthritis/synovitis/synovial proliferation
  • fever/chilling

HPO human phenotypes related to Goodpasture Syndrome:

(show all 27)
id Description Frequency HPO Source Accession
1 proteinuria hallmark (90%) HP:0000093
2 anemia hallmark (90%) HP:0001903
3 respiratory insufficiency hallmark (90%) HP:0002093
4 hemoptysis hallmark (90%) HP:0002105
5 pulmonary infiltrates hallmark (90%) HP:0002113
6 pulmonary embolism hallmark (90%) HP:0002204
7 vasculitis hallmark (90%) HP:0002633
8 autoimmunity hallmark (90%) HP:0002960
9 chest pain hallmark (90%) HP:0100749
10 glomerulopathy hallmark (90%) HP:0100820
11 hematuria typical (50%) HP:0000790
12 renal insufficiency occasional (7.5%) HP:0000083
13 retinal detachment occasional (7.5%) HP:0000541
14 arthritis occasional (7.5%) HP:0001369
15 subcutaneous hemorrhage occasional (7.5%) HP:0001933
16 arthralgia occasional (7.5%) HP:0002829
17 myalgia occasional (7.5%) HP:0003326
18 abnormality of temperature regulation occasional (7.5%) HP:0004370
19 autosomal recessive inheritance HP:0000007
20 renal insufficiency HP:0000083
21 proteinuria HP:0000093
22 glomerulonephritis HP:0000099
23 hematuria HP:0000790
24 dyspnea HP:0002094
25 hemoptysis HP:0002105
26 pulmonary infiltrates HP:0002113
27 autoimmunity HP:0002960

Drugs & Therapeutics for Goodpasture Syndrome

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Drug clinical trials:

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Search NIH Clinical Center for Goodpasture Syndrome

Genetic Tests for Goodpasture Syndrome

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Anatomical Context for Goodpasture Syndrome

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MalaCards organs/tissues related to Goodpasture Syndrome:

32
Kidney, Lung, Bone marrow, Bone, Monocytes, T cells

FMA organs/tissues related to Goodpasture Syndrome:

14
Kidney, Lung

Animal Models for Goodpasture Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Goodpasture Syndrome:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053959.1NID1, COL4A3
2MP:00053698.2NID1, COL4A3BP, MPO
3MP:00053857.9NID1, COL4A3BP, COL4A3, MPO

Publications for Goodpasture Syndrome

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Articles related to Goodpasture Syndrome:

(show top 50)    (show all 54)
idTitleAuthorsYear
1
Plasma exchange in Goodpasture syndrome associated with Turner's syndrome: A case report. (23515881)
2012
2
Cellular and molecular aspects of Goodpasture syndrome. (22218111)
2012
3
Reversible posterior leukoencephalopathy syndrome in Goodpasture syndrome. (22806294)
2012
4
Extracorporeal membrane oxygenation as a platform for recovery: a case report of a child with pulmonary hemorrhage, refractory hypoxemic respiratory failure, and new onset goodpasture syndrome. (22893987)
2012
5
Reversible posterior leukoencephalopathy in the course of Goodpasture syndrome. (22251235)
2012
6
Epidemiology and etiology of wegener granulomatosis, microscopic polyangiitis, churg-strauss syndrome and goodpasture syndrome: vasculitides with frequent lung involvement. (21674413)
2011
7
Plasma exchange for Goodpasture syndrome. (20117054)
2010
8
Goodpasture's 1919 article on the etiology of influenza-the historical road to what we now call Goodpasture Syndrome. (19680022)
2009
9
Migratory pulmonary infiltrates. Goodpasture syndrome. (19124794)
2009
10
Patient with antibody-negative relapse of Goodpasture syndrome. (17474560)
2007
11
Anti-glomerular basement membrane antibodies in the diagnosis of Goodpasture syndrome: a comparison of different assays. (16234289)
2006
12
Goodpasture syndrome in a pregnant woman. (16260570)
2005
13
Goodpasture syndrome. (16183752)
2005
14
Pathogenesis of Goodpasture syndrome: a molecular perspective. (14631560)
2003
15
Goodpasture syndrome during the course of a SchAPnlein-Henoch purpura. (11979333)
2002
16
Goodpasture syndrome: pathophysiology, diagnosis, and management. (12143452)
2001
17
Renal fibroblast-like cells in Goodpasture syndrome rats. (11473642)
2001
18
Genetically modified bone marrow continuously supplies anti-inflammatory cells and suppresses renal injury in mouse Goodpasture syndrome. (11418463)
2001
19
Reactive oxygen species expose cryptic epitopes associated with autoimmune goodpasture syndrome. (10748075)
2000
20
Goodpasture syndrome. (15912919)
2000
21
Inhibition of monocyte chemoattractant protein-1 expression in tubular epithelium attenuates tubulointerstitial alteration in rat Goodpasture syndrome. (10720946)
2000
22
Goodpasture syndrome. (10027952)
1999
23
Complete recovery of renal function in a dialysis-dependent patient with Goodpasture syndrome. (9509464)
1998
24
Goodpasture syndrome involving overlap with Wegener's granulomatosis and anti-glomerular basement membrane disease. (9355084)
1997
25
Anti-GBM antibodies in Goodpasture syndrome; anatomy of an epitope. (9140985)
1997
26
Susceptibility to anti-glomerular basement membrane disease and Goodpasture syndrome is linked to MHC class II genes and the emergence of T cell-mediated immunity in mice. (9410904)
1997
27
Specificity of circulating and tissue-bound autoantibodies in Goodpasture syndrome. (8705733)
1996
28
Anti-alpha1(IV) collagen autoantibodies associated with lung adenocarcinoma presenting as the Goodpasture syndrome. (8607593)
1996
29
Goodpasture syndrome: selective removal of anti-alpha 3 (IV) collagen autoantibodies. A potential therapeutic alternative to plasmapheresis. (8864724)
1996
30
Pregnancy in a patient with Goodpasture syndrome and renal transplantation. (8672189)
1996
31
Identification of the alpha 3 chain of type IV collagen as the common autoantigen in antibasement membrane disease and Goodpasture syndrome. (8589284)
1995
32
The alpha 3 chain of type IV collagen induces autoimmune Goodpasture syndrome. (8016138)
1994
33
Anti-type IV collagen antibodies in Goodpasture syndrome. (8004967)
1994
34
Goodpasture syndrome: molecular and clinical advances. (8041241)
1994
35
Provocation of pulmonary haemorrhage in Goodpasture syndrome by chlorine gas. (8384347)
1993
36
Pregnancy complicated by Goodpasture syndrome. (1565371)
1992
37
Isolated pulmonary Goodpasture syndrome. (1946080)
1991
38
Goodpasture syndrome. Localization of the epitope for the autoantibodies to the carboxyl-terminal region of the alpha 3(IV) chain of basement membrane collagen. (1721062)
1991
39
Goodpasture syndrome: molecular architecture and function of basement membrane antigen. (2671489)
1989
40
A rapid assay for circulating anti-glomerular basement membrane antibodies in Goodpasture syndrome. (2647855)
1989
41
Isolation of the specific glomerular basement membrane antigen involved in Goodpasture syndrome. (6324201)
1984
42
Immune-complex-mediated glomerulonephritis and pulmonary hemorrhage simulating Goodpasture syndrome. (149843)
1978
43
Goodpasture syndrome treated with immunosuppression and plasma exchange. (879947)
1977
44
Goodpasture syndrome: recovery after severe renal insufficiency. (938176)
1976
45
Treatment of Goodpasture syndrome with cyclophosphamide, prednisone and plasma exchange transfusions. (949874)
1976
46
Goodpasture syndrome with recovery after renal failure. (4406940)
1974
47
Goodpasture syndrome. Dissociation from antibodies to glomerular basement membrane. (4124196)
1973
48
Goodpasture syndrome. (5923420)
1966
49
Idiopathic pulmonary haemosiderosis and the Goodpasture syndrome. (5833618)
1965
50
On the Goodpasture syndrome. Comments on two cases of colagenosis of the lung. (5881737)
1965

Variations for Goodpasture Syndrome

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Expression for genes affiliated with Goodpasture Syndrome

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Expression patterns in normal tissues for genes affiliated with Goodpasture Syndrome

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Pathways for genes affiliated with Goodpasture Syndrome

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Pathways related to Goodpasture Syndrome according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.1NID1, COL4A3
2
Show member pathways
9.1NID1, COL4A3

Compounds for genes affiliated with Goodpasture Syndrome

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Sources:
44Novoseek, 28IUPHAR, 24HMDB, 11DrugBank
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Compounds related to Goodpasture Syndrome according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1heparin44 28 24 1112.0NID1, MPO
2serine448.2NID1, COL4A3BP, MPO

GO Terms for genes affiliated with Goodpasture Syndrome

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Cellular components related to Goodpasture Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1basement membraneGO:0056049.1NID1, COL4A3

Biological processes related to Goodpasture Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cell proliferationGO:0082839.4COL4A3BP, COL4A3
2glomerular basement membrane developmentGO:0328369.1NID1, COL4A3
3extracellular matrix disassemblyGO:0226179.0NID1, COL4A3
4extracellular matrix organizationGO:0301988.8NID1, COL4A3

Products for genes affiliated with Goodpasture Syndrome

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  • Antibodies
  • Proteins
  • Lysates

Sources for Goodpasture Syndrome

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet