MCID: GDP001
MIFTS: 47

Goodpasture Syndrome malady

Rare diseases, Cardiovascular diseases, Nephrological diseases, Respiratory diseases, Bone diseases, Immune diseases categories

Aliases & Classifications for Goodpasture Syndrome

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

Aliases & Descriptions for Goodpasture Syndrome:

Name: Goodpasture Syndrome 10 45 47 12 51
Anti-Glomerular Basement Membrane Disease 10 51 65 36
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
Anti-Gbm Disease 47


Classifications:



Characteristics (Orphanet epidemiological data):

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


External Ids:

Disease Ontology10 DOID:9808
MeSH36 D019867
NCIt42 C34649
ICD9CM29 446.21
Orphanet51 375
ICD10 via Orphanet28 M31.0+, N08.5*
UMLS via Orphanet66 C0403529

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 membranous nephropathy and thrombotic thrombocytopenic purpura, and has symptoms including proteinuria, anemia and respiratory insufficiency. An important gene associated with Goodpasture Syndrome is COL4A3 (Collagen, Type IV, Alpha 3 (Goodpasture Antigen)), and among its related pathways are Integrin cell surface interactions and Collagen biosynthesis and modifying enzymes. Affiliated tissues include kidney, lung and lung, and related mouse phenotypes are cardiovascular system and renal/urinary 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 top 50)    (show all 66)
idRelated DiseaseScoreTop Affiliating Genes
1membranous nephropathy10.5
2thrombotic thrombocytopenic purpura10.5
3dermatomyositis10.4
4psoriasis10.4
5relapsing-remitting multiple sclerosis10.4
6crescentic glomerulonephritis10.4
7membranous glomerulonephritis10.4
8pneumonia10.4
9vasculitis10.2
10basement membrane disease10.2
11diffuse cutaneous systemic sclerosis10.1
12alport syndrome, autosomal dominant10.1COL4A3, COL4A4
13wegener granulomatosis10.1
14adult respiratory distress syndrome10.1
15end stage renal failure10.1
16glomerulonephritis10.1
17churg-strauss syndrome10.1
18rapidly progressive glomerulonephritis10.1
19adenocarcinoma10.1
20eosinophilia10.1
21influenza10.1
22purpura10.1
23respiratory failure10.1
24microscopic polyangiitis10.1
25encephalopathy10.1
26hemangioma10.0
27hepatitis10.0
28cryoglobulinemia10.0
29hepatitis c10.0
30atopic dermatitis10.0
31cavernous hemangioma10.0
32dermatitis10.0
33hemorrhagic fever with renal syndrome10.0
34malignant hypertension10.0
35hemorrhagic fever10.0
36clear cell cystadenofibroma10.0CD79A, MPO
37traumatic glaucoma10.0CD79A, MPO
38depersonalization disorder10.0CD79A, MPO
39anal paget's disease10.0CD79A, MPO
40acute pulmonary heart disease10.0CD79A, MPO
41hypersensitivity reaction type iv disease10.0CD79A, MPO
42hyperthyroidism9.9CD79A, MPO
43posterior corneal pigmentation9.9CD79A, MPO
44cutaneous porphyria9.9CD79A, MPO
45acute inflammation of lacrimal passage9.9CD79A, MPO
46byssinosis9.9CD79A, NID1
47bronchus carcinoma9.9CD79A, MPO
48spinal cord lipoma9.9CD79A, MPO
49ataxia with vitamin e deficiency9.9COL4A3, COL4A4, COL4A5
50penis paget's disease9.9CD79A, MPO

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

idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
MetforminapprovedPhase 2, Phase 31573657-24-9, 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

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, T cells, Monocytes, Bone marrow

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:00053858.2COL4A3, COL4A3BP, COL4A5, MPO, NID1
2MP:00053678.2CD79A, COL4A3, COL4A4, COL4A5
3MP:00053767.4CD79A, COL4A3, COL4A3BP, COL4A4, COL4A5, MPO

Publications for Goodpasture Syndrome

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Articles related to Goodpasture Syndrome:

(show top 50)    (show all 59)
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
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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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 14)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.6COL4A3, COL4A4
2
Show member pathways
9.0COL4A3, COL4A4, COL4A5
3
Show member pathways
9.0COL4A3, COL4A4, COL4A5
49.0COL4A3, 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
128.5COL4A3, COL4A4, COL4A5, NID1
13
Show member pathways
8.5COL4A3, COL4A4, COL4A5, NID1
14
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:00056059.4COL4A4, COL4A5, NID1
2collagen trimerGO:00055819.0COL4A3, COL4A4, COL4A5
3endoplasmic reticulum lumenGO:00057889.0COL4A3, COL4A4, COL4A5
4collagen type IV trimerGO:00055878.9COL4A3, COL4A4, COL4A5
5basement membraneGO:00056048.2COL4A3, COL4A4, COL4A5, NID1

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

idNameGO IDScoreTop Affiliating Genes
1glomerular basement membrane developmentGO:00328369.6COL4A3, COL4A4, NID1
2collagen catabolic processGO:00305748.7COL4A3, COL4A4, COL4A5
3extracellular matrix disassemblyGO:00226178.5COL4A3, COL4A4, COL4A5, NID1
4extracellular matrix organizationGO:00301988.5COL4A3, COL4A4, COL4A5, NID1

Molecular functions related to Goodpasture Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1extracellular matrix structural constituentGO:00052019.0COL4A3, COL4A4, COL4A5

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