MCID: GDP001
MIFTS: 52

Goodpasture Syndrome malady

Categories: Rare diseases, Cardiovascular diseases, Nephrological diseases, Respiratory diseases, Bone diseases, Immune diseases

Aliases & Classifications for Goodpasture Syndrome

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Sources:
10Disease Ontology, 45NIH Rare Diseases, 47Novoseek, 12DISEASES, 51Orphanet, 36MeSH, 65UMLS, 27ICD10, 29ICD9CM, 42NCIt, 59SNOMED-CT, 28ICD10 via Orphanet, 66UMLS via Orphanet, 61The Human Phenotype Ontology
See all MalaCards sources

Aliases & Descriptions for Goodpasture Syndrome:

Name: Goodpasture Syndrome 10 45 47 12 51
Anti-Glomerular Basement Membrane Disease 10 51 36 65
Pulmonary Renal Syndrome 45 65
Rapidly Progressive Glomerulonephritis with Pulmonary Hemorrhage 45
 
Anti-Glomerular Basement Membrane Antibody Disease 45
Glomerulonephritis - Pulmonary Hemorrhage 45
Anti-Gbm Syndrome 51

Characteristics:

Orphanet epidemiological data:

51
goodpasture syndrome:
Inheritance: Not applicable; Prevalence: <1/1000000 (Europe); Age of onset: Adolescent,Adult


Classifications:



External Ids:

Disease Ontology10 DOID:9808
ICD1027 M31.0
ICD9CM29 446.21
MeSH36 D019867
Orphanet51 375
ICD10 via Orphanet28 M31.0+, N08.5*
UMLS via Orphanet66 C0403529
UMLS65 C0403529, C0549143

Summaries for Goodpasture Syndrome

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NIH Rare Diseases:45 Goodpasture syndrome is an autoimmune disease of unknown cause that affects the lungs and kidneys and is characterized by pulmonary alveolar hemorrhage (bleeding in the lungs), and a kidney disease known as glomerulonephritis. some use the term "goodpasture syndrome" for the findings of glomerulonephritis and pulmonary hemorrhage and the term "goodpasture disease" for those patients with glomerulonephritis, pulmonary hemorrhage, and anti-gbm antibodies. currently, the preferred term for both conditions is “anti-gbm antibody disease”. circulating antibodies are directed against the collagen of the part of the kidney known as the glomerular basement membrane (gbm), thereby resulting in acute or rapidly progressive glomerulonephritis. antibodies also attack the collagen of the air sacs of the lung (alveolus) resulting in bleeding of the lung (pulmonary hemorrhage). anti-gbm antibody disease is most often idiopathic, although it can occasionally follow pulmonary infections or be associated with pulmonary injury. diagnosis is confirmed with the presence of anti-gbm antibody in blood or in the kidney. the treatment of choice is plasmapheresis in conjunction with prednisone and cyclophosphamide. last updated: 9/22/2015

MalaCards based summary: Goodpasture Syndrome, also known as anti-glomerular basement membrane disease, is related to alport syndrome, autosomal dominant and dermatomyositis, and has symptoms including glomerulopathy, chest pain and autoimmunity. An important gene associated with Goodpasture Syndrome is COL4A3 (Collagen Type IV Alpha 3), and among its related pathways are PI3K-Akt signaling pathway and ECM-receptor interaction. Affiliated tissues include kidney, lung and lung, and related mouse phenotypes are renal/urinary system and cardiovascular system.

Disease Ontology:10 A hypersensitivity reaction type II disease that is characterized by glomerulonephritis located in kidney and hemorrhaging located in lung.

Wikipedia:68 Goodpasture syndrome (GPS; also known as Goodpasture’s disease, antiglomerular basement antibody... more...

Related Diseases for Goodpasture Syndrome

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Diseases related to Goodpasture Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 46)
idRelated DiseaseScoreTop Affiliating Genes
1alport syndrome, autosomal dominant10.3COL4A3, COL4A4
2dermatomyositis10.1
3psoriasis10.1
4craniofacial anomalies and anterior segment dysgenesis syndrome10.0COL4A3, COL4A4
5rapidly progressive glomerulonephritis10.0
6choroid plexus meningioma9.9CD79A, MPO
7generalized atherosclerosis9.9CD79A, MPO
8post-traumatic stress disorder9.9CD79A, MPO
9exudative glomerulonephritis9.9CD79A, MPO
10basement membrane disease9.9
11angioedema9.9CD79A, MPO
12diffuse secondary choroid atrophy9.9CD79A, MPO
13crescentic glomerulonephritis9.9CD79A, MPO
14hemosiderosis9.9CD79A, MPO
15lymphocytic colitis9.8CD79A, MPO
16bronchial neoplasm9.8CD79A, MPO
17qualitative platelet defect9.8CD79A, MPO
18vasculitis9.8
19focal embolic glomerulonephritis9.8CD79A, MPO
20gallbladder leiomyoma9.8COL4A4, COL4A5
21palindromic rheumatism9.8CD79A, NID1
22cervical incompetence9.8CD79A, MPO
23ataxia with vitamin e deficiency9.8COL4A3, COL4A4, COL4A5
24hematuria, benign familial9.8COL4A3, COL4A4, COL4A5
25alport syndrome, autosomal recessive9.8COL4A3, COL4A4, COL4A5
26basilar migraine9.7COL4A3, COL4A4, COL4A5
27wegener granulomatosis9.7
28adult respiratory distress syndrome9.7
29end stage renal failure9.7
30glomerulonephritis9.7
31respiratory failure9.7
32adenocarcinoma9.7
33churg-strauss syndrome9.7
34purpura9.7
35influenza9.7
36eosinophilia9.7
37microscopic polyangiitis9.7
38encephalopathy9.7
39deafness, x-linked 69.7COL4A3, COL4A4, COL4A5
40carbohydrate metabolic disorder9.7COL4A3, COL4A4, COL4A5
41villonodular synovitis9.7CD79A, MPO
42hodgkin's granuloma9.6CD79A, MPO
43liver leiomyosarcoma9.5CD79A, MPO
44epidermolysis bullosa dystrophica9.2CD79A, COL4A5
45glycogen storage disease8.5CD79A, COL4A3, COL4A4, COL4A5, MPO
46hypersensitivity vasculitis7.3CD79A, COL4A3, COL4A3BP, COL4A4, COL4A5, MPO

Comorbidity relations with Goodpasture Syndrome via Phenotypic Disease Network (PDN):


Deficiency AnemiaAcute Kidney Failure
Chronic Kidney FailureRespiratory Failure
Heart Disease

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:

 51 (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 26)
id Description Frequency HPO Source Accession
1 glomerulopathy hallmark (90%) HP:0100820
2 chest pain hallmark (90%) HP:0100749
3 autoimmunity hallmark (90%) HP:0002960
4 vasculitis hallmark (90%) HP:0002633
5 pulmonary embolism hallmark (90%) HP:0002204
6 pulmonary infiltrates hallmark (90%) HP:0002113
7 hemoptysis hallmark (90%) HP:0002105
8 respiratory insufficiency hallmark (90%) HP:0002093
9 anemia hallmark (90%) HP:0001903
10 proteinuria hallmark (90%) HP:0000093
11 hematuria typical (50%) HP:0000790
12 abnormality of temperature regulation occasional (7.5%) HP:0004370
13 myalgia occasional (7.5%) HP:0003326
14 arthralgia occasional (7.5%) HP:0002829
15 subcutaneous hemorrhage occasional (7.5%) HP:0001933
16 arthritis occasional (7.5%) HP:0001369
17 retinal detachment occasional (7.5%) HP:0000541
18 renal insufficiency occasional (7.5%) HP:0000083
19 autoimmunity HP:0002960
20 pulmonary infiltrates HP:0002113
21 hemoptysis HP:0002105
22 dyspnea HP:0002094
23 hematuria HP:0000790
24 glomerulonephritis HP:0000099
25 proteinuria HP:0000093
26 renal insufficiency HP:0000083

Drugs & Therapeutics for Goodpasture Syndrome

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Drugs for Goodpasture Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
MetforminapprovedPhase 2, Phase 31645657-24-9, 1115-70-414219, 4091
Synonyms:
1,1-Dimethyl biguanide
1,1-Dimethylbiguanide
3-(diaminomethylidene)-1,1-dimethylguanidine
657-24-9
AC1L1HE4
AKOS000121065
Apo-Metformin
BIDD:GT0697
BPBio1_000009
BRD-K79602928-003-04-1
BSPBio_000007
BSPBio_002314
C07151
C4H11N5
CAS-1115-70-4
CCRIS 9321
CHEBI:6801
CHEMBL1431
CID4091
D04966
DB00331
DMBG
DMGG
Diabefagos
Diabetosan
Diabex
Diaformin
Dianben
Dimethylbiguanid
Dimethylbiguanide
Dimethylbiguanidine
Dimethyldiguanide
Dimethylguanylguanidine
EINECS 211-517-8
Fluamine
Flumamine
Fortamet
Gen-Metformin
Glifage
Gliguanid
Glucophage
Glucophage XR
Glumetza
Glycon
HMS2089D19
HSCI1_000295
Haurymelin
Haurymellin
Islotin
KBio2_002310
KBio2_004878
KBio2_007446
KBio3_002790
KBioGR_002310
 
KBioSS_002312
LA-6023
LS-43899
Meguan
Melbin
Metformin
Metformin (USAN/INN)
Metformin HCL
Metformin Hydrochloride
Metformin [USAN:INN:BAN]
Metformina
Metformina [DCIT]
Metformina [Spanish]
Metformine
Metformine [INN-French]
Metformine pamoate
Metforminum
Metforminum [INN-Latin]
Metiguanide
MolPort-002-929-560
MolPort-004-288-389
MolPort-005-767-418
Mylan-Metformin
N,N-Dimethylbiguanide
N,N-Dimethyldiguanide
N,N-Dimethylimidodicarbonimidic diamide
N,N-dimethylimidodicarbonimidic diamide
N1,N1-Dimethylbiguanide
NCGC00016564-01
NCGC00016564-02
NCGC00016564-03
NNDG
Novo-Metformin
Nu-Metformin
Obimet
PMS-Metformin
Prestwick0_000004
Prestwick1_000004
Prestwick2_000004
Prestwick3_000004
Ran-Metformin
Ratio-Metformin
Riomet
S2483_Selleck
SPBio_001928
STK011633
Sandoz Metformin
Siofor
T5895664
Teva-Metformin
UNII-9100L32L2N
ZINC12859773
cMAP_000016
metformin
metformin hydrochloride
2Hypoglycemic AgentsPhase 2, Phase 34503

Interventional clinical trials:

idNameStatusNCT IDPhase
1Comparison Study of Cefazolin Versus Cefazolin Plus Azithromycin Prophylaxis in Post-Cesarean EndometritisWithdrawnNCT01721616Phase 4
2Efficacy Study of Metformin Glycinate on Postprandial LipemiaNot yet recruitingNCT02064881Phase 2, Phase 3

Search NIH Clinical Center for Goodpasture Syndrome


Cochrane evidence based reviews: anti-glomerular basement membrane disease

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:

33
Lung, Kidney, Bone, Monocytes, Endothelial, Bone marrow, T cells

FMA organs/tissues related to Goodpasture Syndrome:

16
Kidney, Lung

Animal Models for Goodpasture Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Goodpasture Syndrome:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053678.5CD79A, COL4A3, COL4A4, COL4A5
2MP:00053858.5COL4A3, COL4A3BP, COL4A5, MPO, NID1
3MP:00053877.5CD79A, COL4A3, COL4A3BP, COL4A4, COL4A5, MPO

Publications for Goodpasture Syndrome

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Articles related to Goodpasture Syndrome:

(show top 50)    (show all 60)
idTitleAuthorsYear
1
Severe adult respiratory distress syndrome from Goodpasture syndrome. Survival using extracorporeal membrane oxygenation. (25590156)
2015
2
Clinical management of pregnancy in women with Goodpasture syndrome. (25634441)
2015
3
Goodpasture syndrome and posterior reversible encephalopathy syndrome. (25982503)
2015
4
Plasma exchange in Goodpasture syndrome associated with Turner's syndrome: A case report. (23515881)
2012
5
Cellular and molecular aspects of Goodpasture syndrome. (22218111)
2012
6
Reversible posterior leukoencephalopathy syndrome in Goodpasture syndrome. (22806294)
2012
7
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
8
Reversible posterior leukoencephalopathy in the course of Goodpasture syndrome. (22251235)
2012
9
A case of Goodpasture syndrome positive for anti-GBM antibody and MPO-ANCA complicated by a variety of serious infections. (21426895)
2011
10
Epidemiology and etiology of wegener granulomatosis, microscopic polyangiitis, churg-strauss syndrome and goodpasture syndrome: vasculitides with frequent lung involvement. (21674413)
2011
11
Plasma exchange for Goodpasture syndrome. (20117054)
2010
12
Patient with antibody-negative relapse of Goodpasture syndrome. (17474560)
2007
13
Anti-glomerular basement membrane antibodies in the diagnosis of Goodpasture syndrome: a comparison of different assays. (16234289)
2006
14
Goodpasture syndrome in a pregnant woman. (16260570)
2005
15
Goodpasture syndrome. (16183752)
2005
16
Pathogenesis of Goodpasture syndrome: a molecular perspective. (14631560)
2003
17
Goodpasture syndrome during the course of a SchAPnlein-Henoch purpura. (11979333)
2002
18
Goodpasture syndrome: pathophysiology, diagnosis, and management. (12143452)
2001
19
Renal fibroblast-like cells in Goodpasture syndrome rats. (11473642)
2001
20
Genetically modified bone marrow continuously supplies anti-inflammatory cells and suppresses renal injury in mouse Goodpasture syndrome. (11418463)
2001
21
Reactive oxygen species expose cryptic epitopes associated with autoimmune goodpasture syndrome. (10748075)
2000
22
Goodpasture syndrome. (15912919)
2000
23
Inhibition of monocyte chemoattractant protein-1 expression in tubular epithelium attenuates tubulointerstitial alteration in rat Goodpasture syndrome. (10720946)
2000
24
Goodpasture syndrome. (10027952)
1999
25
Complete recovery of renal function in a dialysis-dependent patient with Goodpasture syndrome. (9509464)
1998
26
Goodpasture syndrome involving overlap with Wegener's granulomatosis and anti-glomerular basement membrane disease. (9355084)
1997
27
Anti-GBM antibodies in Goodpasture syndrome; anatomy of an epitope. (9140985)
1997
28
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
29
Specificity of circulating and tissue-bound autoantibodies in Goodpasture syndrome. (8705733)
1996
30
Anti-alpha1(IV) collagen autoantibodies associated with lung adenocarcinoma presenting as the Goodpasture syndrome. (8607593)
1996
31
Goodpasture syndrome: selective removal of anti-alpha 3 (IV) collagen autoantibodies. A potential therapeutic alternative to plasmapheresis. (8864724)
1996
32
Identification of the alpha 3 chain of type IV collagen as the common autoantigen in antibasement membrane disease and Goodpasture syndrome. (8589284)
1995
33
The alpha 3 chain of type IV collagen induces autoimmune Goodpasture syndrome. (8016138)
1994
34
Anti-type IV collagen antibodies in Goodpasture syndrome. (8004967)
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

Variations for Goodpasture Syndrome

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Expression for genes affiliated with Goodpasture Syndrome

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Search GEO for disease gene expression data for Goodpasture Syndrome.

Pathways for genes affiliated with Goodpasture Syndrome

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Pathways related to Goodpasture Syndrome according to GeneCards Suite gene sharing:

(show all 15)
idSuper pathwaysScoreTop Affiliating Genes
19.0COL4A3, COL4A4, COL4A5
2
Show member pathways
9.0COL4A3, COL4A4, COL4A5
3
Show member pathways
9.0COL4A3, COL4A4, COL4A5
4
Show member pathways
9.0COL4A3, COL4A4, COL4A5
59.0COL4A3, COL4A4, COL4A5
69.0COL4A3, COL4A4, COL4A5
79.0COL4A3, COL4A4, COL4A5
89.0COL4A3, COL4A4, COL4A5
99.0COL4A3, COL4A4, COL4A5
109.0COL4A3, COL4A4, COL4A5
119.0COL4A3, COL4A4, COL4A5
129.0COL4A3, COL4A4, COL4A5
138.5COL4A3, COL4A4, COL4A5, NID1
14
Show member pathways
8.5COL4A3, COL4A4, COL4A5, NID1
15
Show member pathways
8.2CD79A, COL4A3, COL4A4, COL4A5

GO Terms for genes affiliated with Goodpasture Syndrome

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Cellular components related to Goodpasture Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1basal laminaGO:00056059.4COL4A5, NID1
2collagen type IV trimerGO:00055878.9COL4A3, COL4A4, COL4A5

Biological processes related to Goodpasture Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1collagen catabolic processGO:00305749.5COL4A3, COL4A4
2extracellular matrix disassemblyGO:00226178.5COL4A3, COL4A4, COL4A5, NID1

Sources for Goodpasture Syndrome

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2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet