MCID: GDP001
MIFTS: 48

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 villonodular synovitis and rapidly progressive glomerulonephritis, 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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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, Colon, Bone marrow, T cells, Endothelial

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 59)
idTitleAuthorsYear
1
Association study of inflammatory genes with rheumatic heart disease in North Indian population: A multi-analytical approach. (27118427)
2016
2
Enzyme replacement therapy with idursulfase for mucopolysaccharidosis type II (Hunter syndrome). (24399699)
2014
3
Serum uric acid and new-onset hypertension: a possible therapeutic avenue? (24430708)
2014
4
Glucose-induced regulation of protein import receptor Tom22 by cytosolic and mitochondria-bound kinases. (24093680)
2013
5
Allelotypes of lung adenocarcinomas featuring ALK fusion demonstrate fewer onco- and suppressor gene changes. (23289484)
2013
6
Impact of treatment with continuous positive airway pressure (CPAP) on weight in obstructive sleep apnea. (24127141)
2013
7
ERG transcriptional networks in primary acute leukemia cells implicate a role for ERG in deregulated kinase signaling. (23300998)
2013
8
Short-term outcomes of extracorporeal shock wave therapy for the treatment of chronic non-calcific tendinopathy of the supraspinatus: a double-blind, randomized, placebo-controlled trial. (22672772)
2012
9
Diagnosis and management of iron deficiency anemia in the 21st century. (21694802)
2011
10
Clinical trial on the efficacy of a new symbiotic formulation, Flortec, in patients with acute diarrhea: a multicenter, randomized study in primary care. (20616746)
2010
11
Serum levels of apelin and ghrelin in patients with acute coronary syndromes and established coronary artery disease--KOZANI STUDY. (20403579)
2010
12
Clopidogrel resistance: pharmacokinetic or pharmacogenetic? (19246723)
2009
13
Light chain deposition disease as a rare cause of restrictive cardiomyopathy. (20128164)
2009
14
Gab1 but not Grb2 mediates tumor progression in Met overexpressing colorectal cancer cells. (18192688)
2008
15
Natural history and clinical management of chronic hepatitis B virus infection in children. (19669296)
2008
16
Histopathology of Salzmann nodular corneal degeneration. (18216567)
2008
17
Follow-up of healthy donors receiving granulocyte colony-stimulating factor for peripheral blood progenitor cell mobilization and collection. Results of the Spanish Donor Registry. (18387976)
2008
18
Andrographolide induces cell cycle arrest at G2/M phase and cell death in HepG2 cells via alteration of reactive oxygen species. (17512926)
2007
19
Ovarian Vein Thrombophlebitis: An unusual cause of postpartum fever and abdominal pain. (21654948)
2007
20
Impact of nitrite exposure on endocrine, osmoregulatory and cytoprotective functions in the marine teleost Sparus sarba. (17367878)
2007
21
The role of oxyntomodulin and peptide tyrosine-tyrosine (PYY) in appetite control. (17082808)
2006
22
Bacteriuria in pregnant women with sickle cell trait. (16647923)
2006
23
Decompression of idiopathic lumbar epidural lipomatosis: diagnostic magnetic resonance imaging evaluation and review of the literature. (16506462)
2006
24
C-peptide stimulates ERK1/2 and JNK MAP kinases via activation of protein kinase C in human renal tubular cells. (15624099)
2005
25
(99m)Tc-Ethylenenediamine-N,N'-diacetic acid/hydrazinonicotinamide[Lys(3)]-bombesin (20641296)
2004
26
Expression and purification of enzymatically active forms of the human lysyl oxidase-like protein 4. (14551188)
2003
27
Alpha(v)beta3 integrin expression up-regulates cdc2, which modulates cell migration. (12771130)
2003
28
Lamellar body count and interleukin-6 levels in women with preterm labor]. (14669437)
2003
29
Human recombinant factor IX: safety and efficacy studies in hemophilia B patients previously treated with plasma-derived factor IX concentrates. (12433699)
2002
30
Gastric tonometry after gastroschisis repair. (12244016)
2002
31
Randomized, double-blind, controlled study of cefoperazone-sulbactam plus cotrimoxazole versus ceftazidime plus cotrimoxazole for the treatment of severe melioidosis. (11389491)
2001
32
Prolonged bone marrow failure with monosomy 7 after engraftment failure following bone marrow transplantation. (11372741)
2001
33
Somatostatin receptor 1 (SSTR1)-mediated inhibition of cell proliferation correlates with the activation of the MAP kinase cascade: role of the phosphotyrosine phosphatase SHP-2. (11088001)
2000
34
Low-affinity LFA-1/ICAM-3 interactions augment LFA-1/ICAM-1-mediated T cell adhesion and signaling by redistribution of LFA-1. (10639327)
2000
35
Mycobacterial DNA not detected in liver sections from patients with primary biliary cirrhosis. (9551681)
1998
36
PCNA binding through a conserved motif. (9631646)
1998
37
Lunatomalacia associated with congenital shortening of the ulna in Langer-Giedion syndrome: a case report. (9195447)
1997
38
Lack of proliferative response by gluten-specific T cells in the blood and gut of patients with dermatitis herpetiformis. (7492350)
1995
39
Sites within the 39-kDa protein important for regulating ligand binding to the low-density lipoprotein receptor-related protein. (7533537)
1995
40
Value of prostate-specific antigen as a tumor marker. (7505232)
1993
41
Diffuse and localized tenosynovial giant cell tumor and pigmented villonodular synovitis: a clinicopathologic and flow cytometric DNA analysis. (1319390)
1992
42
Pulmonary blastoma. A case report. (1580111)
1992
43
Human isolated ileum: motor responses of the circular muscle to electrical field stimulation and exogenous neuropeptides. (1692976)
1990
44
Neuroendocrine carcinoma of the colon and rectum. A clinicopathologic, ultrastructural, and immunohistochemical study of 24 cases. (2173427)
1990
45
Klippel-TrAcnaunay-Weber syndrome--report of a case associated with an incomplete form of Sturge-Weber syndrome. (6091958)
1984
46
Tuberculoid leprosy vs. tuberculosis of skin. A histopathologic study. (7338397)
1981
47
Goniophotocoagulation in the management of rubeosis iridis. (6176826)
1981
48
Growth inhibition of homografts of a plasma-cell neoplasm in cell-impenetrable diffusion chambers placed in hyperimmunized mice. (13792639)
1959
49
Adrenal neuroblastoma. (20278246)
1947
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