MCID: GDP001
MIFTS: 47

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:
50OMIM, 11Disease Ontology, 46NIH Rare Diseases, 13DISEASES, 52Orphanet, 48Novoseek, 37MeSH, 66UMLS, 28ICD10, 30ICD9CM, 43NCIt, 29ICD10 via Orphanet, 67UMLS via Orphanet, 60SNOMED-CT, 62The Human Phenotype Ontology
See all MalaCards sources

Aliases & Descriptions for Goodpasture Syndrome:

Name: Goodpasture Syndrome 50 11 46 13 52 48
Anti-Glomerular Basement Membrane Disease 11 52 37 66
Pulmonary Renal Syndrome 46 66
Rapidly Progressive Glomerulonephritis with Pulmonary Hemorrhage 46
 
Anti-Glomerular Basement Membrane Antibody Disease 46
Glomerulonephritis - Pulmonary Hemorrhage 46
Anti-Gbm Syndrome 52
Anti-Gbm Disease 48

Characteristics:

Orphanet epidemiological data:

52
goodpasture syndrome:
Inheritance: Not applicable; Prevalence: <1/1000000 (Europe); Age of onset: Adolescent,Adult

HPO:

62
goodpasture syndrome:
Inheritance: autosomal recessive inheritance


Classifications:



External Ids:

OMIM50 233450
Disease Ontology11 DOID:9808
ICD1028 M31.0
ICD9CM30 446.21
MeSH37 D019867
Orphanet52 ORPHA375
ICD10 via Orphanet29 M31.0+, N08.5*
UMLS via Orphanet67 C0403529

Summaries for Goodpasture Syndrome

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NIH Rare Diseases:46 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 autosomal dominant nocturnal frontal lobe epilepsy and hematuria, benign familial, and has symptoms including proteinuria, anemia and respiratory insufficiency. An important gene associated with Goodpasture Syndrome is COL4A3 (Collagen Type IV Alpha 3 Chain), and among its related pathways are Cell adhesion_Plasmin signaling and Cell adhesion_Endothelial cell contacts by non-junctional mechanisms. Affiliated tissues include kidney, lung and lung.

Disease Ontology:11 A hypersensitivity reaction type ii disease that is characterized by glomerulonephritis located in kidney and hemorrhaging located in lung.

Wikipedia:69 Goodpasture syndrome (GPS; also known as Goodpasture’s disease, antiglomerular basement antibody... more...

Description from OMIM:50 233450

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 63)
idRelated DiseaseScoreTop Affiliating Genes
1autosomal dominant nocturnal frontal lobe epilepsy10.4COL4A3, COL4A5
2hematuria, benign familial10.4COL4A3, COL4A5
3alport syndrome, autosomal recessive10.4COL4A3, COL4A5
4vulvar leiomyoma10.3COL4A3, COL4A5
5heart disease10.1CD79A, COL4A3
6fg syndrome 510.1COL4A3, COL4A5
7dermatomyositis10.1
8psoriasis10.1
9crest syndrome10.0CD79A, COL4A3
10benign meningioma10.0CD79A, MPO
11arteriosclerosis10.0CD79A, MPO
12rapidly progressive glomerulonephritis10.0
13chronic mucocutaneous candidiasis10.0CD79A, MPO
14blue color blindness10.0CD79A, NID1
15acquired thrombocytopenia10.0CD79A, MPO
16polyarteritis nodosa10.0CD79A, MPO
17suppurative uveitis10.0CD79A, MPO
18chromoblastomycosis10.0CD79A, MPO
19complex partial epilepsy9.9CD79A, MPO
20meibomian cyst9.9CD79A, MPO
21clostridium difficile colitis9.9CD79A, MPO
22lung squamous cell carcinoma9.9CD79A, MPO
23yellow fever9.9CD79A, MPO
24pulmonary alveolar proteinosis9.9CD79A, MPO
25vestibule of mouth cancer9.9CD79A, IL17A
26malignant eyelid melanoma9.9CD79A, IL17A
27sphenoid sinusitis9.9CD79A, MPO
28lymphoplasmacytic lymphoma9.9COL4A3, COL4A5
29kidney angiomyolipoma9.9CD79A, MPO
30lymphopenia9.8CD79A, MPO
31tick-borne encephalitis9.8CD79A, IL17A
32vasculitis9.8
33chronic conjunctivitis9.8CD79A, IL17A
34reproductive system disease9.8CD79A, IL17A
35biliary tract disease9.8CD79A, IL17A
36wegener granulomatosis9.7
37adult respiratory distress syndrome9.7
38end stage renal failure9.7
39glomerulonephritis9.7
40respiratory failure9.7
41adenocarcinoma9.7
42churg-strauss syndrome9.7
43purpura9.7
44influenza9.7
45eosinophilia9.7
46microscopic polyangiitis9.7
47encephalopathy9.7
48psoriasis susceptibility 19.7CD79A, IL17A
49uterine corpus dissecting leiomyoma9.7CD79A, IL17A
50blood group incompatibility9.7CD79A, IL17A

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 by clinical synopsis from OMIM:

233450

Clinical features from OMIM:

233450

Symptoms:

 52 (show all 19)
  • renal insufficiency
  • proteinuria
  • retinal detachment
  • hematuria
  • purpura
  • arthritis
  • anemia
  • fever
  • respiratory insufficiency
  • hemoptysis
  • pulmonary infiltrates
  • vasculitis
  • arthralgia
  • autoimmunity
  • myalgia
  • persistence of primary teeth
  • cough
  • chest pain
  • glomerulopathy

HPO human phenotypes related to Goodpasture Syndrome:

(show all 26)
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 renal insufficiency HP:0000083
20 proteinuria HP:0000093
21 glomerulonephritis HP:0000099
22 hematuria HP:0000790
23 dyspnea HP:0002094
24 hemoptysis HP:0002105
25 pulmonary infiltrates HP:0002113
26 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
MetforminPhase 2, Phase 31701657-24-914219, 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
DMGG
Diabetosan
Diabex
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
Melbin
Metformin
Metformin (USAN/INN)
Metformin HCL
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
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:

34
Lung, Kidney, Bone, T cells, Monocytes, Bone marrow

FMA organs/tissues related to Goodpasture Syndrome:

17
Kidney, Lung

Animal Models for Goodpasture Syndrome or affiliated genes

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Publications for Goodpasture Syndrome

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Articles related to Goodpasture Syndrome:

(show top 50)    (show all 60)
idTitleAuthorsYear
1
Outcomes of patients with Goodpasture syndrome: A nationwide cohort-based study from the French Society of Hemapheresis. (27267459)
2016
2
Clinical management of pregnancy in women with Goodpasture syndrome. (25634441)
2015
3
Severe adult respiratory distress syndrome from Goodpasture syndrome. Survival using extracorporeal membrane oxygenation. (25590156)
2015
4
Goodpasture syndrome and posterior reversible encephalopathy syndrome. (25982503)
2015
5
Prolonged venovenous extracorporeal membrane oxygenation without anticoagulation: a case of Goodpasture syndrome-related pulmonary haemorrhage. (24588439)
2014
6
Plasma exchange in Goodpasture syndrome associated with Turner's syndrome: A case report. (23515881)
2012
7
Reversible posterior leukoencephalopathy syndrome in Goodpasture syndrome. (22806294)
2012
8
Reversible posterior leukoencephalopathy in the course of Goodpasture syndrome. (22251235)
2012
9
Cellular and molecular aspects of Goodpasture syndrome. (22218111)
2012
10
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
11
A case of Goodpasture syndrome positive for anti-GBM antibody and MPO-ANCA complicated by a variety of serious infections. (21426895)
2011
12
Epidemiology and etiology of wegener granulomatosis, microscopic polyangiitis, churg-strauss syndrome and goodpasture syndrome: vasculitides with frequent lung involvement. (21674413)
2011
13
Plasma exchange for Goodpasture syndrome. (20117054)
2010
14
Goodpasture's 1919 article on the etiology of influenza-the historical road to what we now call Goodpasture Syndrome. (19680022)
2009
15
Patient with antibody-negative relapse of Goodpasture syndrome. (17474560)
2007
16
Anti-glomerular basement membrane antibodies in the diagnosis of Goodpasture syndrome: a comparison of different assays. (16234289)
2006
17
Zebrafish to humans: evolution of the alpha3-chain of type IV collagen and emergence of the autoimmune epitopes associated with Goodpasture syndrome. (16254142)
2006
18
Goodpasture syndrome in a pregnant woman. (16260570)
2005
19
Goodpasture syndrome. (16183752)
2005
20
Pathogenesis of Goodpasture syndrome: a molecular perspective. (14631560)
2003
21
Goodpasture syndrome during the course of a SchAPnlein-Henoch purpura. (11979333)
2002
22
Renal fibroblast-like cells in Goodpasture syndrome rats. (11473642)
2001
23
Genetically modified bone marrow continuously supplies anti-inflammatory cells and suppresses renal injury in mouse Goodpasture syndrome. (11418463)
2001
24
Goodpasture syndrome: pathophysiology, diagnosis, and management. (12143452)
2001
25
Goodpasture syndrome. (15912919)
2000
26
Reactive oxygen species expose cryptic epitopes associated with autoimmune goodpasture syndrome. (10748075)
2000
27
Goodpasture syndrome. (10027952)
1999
28
Complete recovery of renal function in a dialysis-dependent patient with Goodpasture syndrome. (9509464)
1998
29
Goodpasture syndrome involving overlap with Wegener's granulomatosis and anti-glomerular basement membrane disease. (9355084)
1997
30
Anti-GBM antibodies in Goodpasture syndrome; anatomy of an epitope. (9140985)
1997
31
Anti-alpha1(IV) collagen autoantibodies associated with lung adenocarcinoma presenting as the Goodpasture syndrome. (8607593)
1996
32
Pregnancy in a patient with Goodpasture syndrome and renal transplantation. (8672189)
1996
33
Specificity of circulating and tissue-bound autoantibodies in Goodpasture syndrome. (8705733)
1996
34
Goodpasture syndrome: selective removal of anti-alpha 3 (IV) collagen autoantibodies. A potential therapeutic alternative to plasmapheresis. (8864724)
1996
35
The alpha 3 chain of type IV collagen induces autoimmune Goodpasture syndrome. (8016138)
1994
36
Anti-type IV collagen antibodies in Goodpasture syndrome. (8004967)
1994
37
Goodpasture syndrome: molecular and clinical advances. (8041241)
1994
38
Provocation of pulmonary haemorrhage in Goodpasture syndrome by chlorine gas. (8384347)
1993
39
Pregnancy complicated by Goodpasture syndrome. (1565371)
1992
40
Isolated pulmonary Goodpasture syndrome. (1946080)
1991
41
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
42
Goodpasture syndrome: molecular architecture and function of basement membrane antigen. (2671489)
1989
43
A rapid assay for circulating anti-glomerular basement membrane antibodies in Goodpasture syndrome. (2647855)
1989
44
Isolation of the specific glomerular basement membrane antigen involved in Goodpasture syndrome. (6324201)
1984
45
Immune-complex-mediated glomerulonephritis and pulmonary hemorrhage simulating Goodpasture syndrome. (149843)
1978
46
Goodpasture syndrome treated with immunosuppression and plasma exchange. (879947)
1977
47
Goodpasture syndrome: recovery after severe renal insufficiency. (938176)
1976
48
Goodpasture syndrome. Dissociation from antibodies to glomerular basement membrane. (4124196)
1973
49
Goodpasture syndrome. (5923420)
1966
50
Idiopathic pulmonary haemosiderosis and the Goodpasture syndrome. (5833618)
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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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
1collagen type IV trimerGO:00055879.7COL4A3, COL4A5
2basal laminaGO:00056059.5COL4A5, NID1
3basement membraneGO:00056048.9COL4A3, COL4A5, NID1

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

idNameGO IDScoreTop Affiliating Genes
1glomerular basement membrane developmentGO:003283610.1COL4A3, NID1
2collagen catabolic processGO:00305749.9COL4A3, COL4A5
3defense response to fungusGO:00508329.7IL17A, MPO
4extracellular matrix organizationGO:00301989.2COL4A3, COL4A5, NID1

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

idNameGO IDScoreTop Affiliating Genes
1extracellular matrix structural constituentGO:00052019.6COL4A3, COL4A5

Sources for Goodpasture Syndrome

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
25GTR
26HGMD
27HMDB
28ICD10
29ICD10 via Orphanet
30ICD9CM
31IUPHAR
32KEGG
35MedGen
37MeSH
38MESH via Orphanet
39MGI
42NCI
43NCIt
44NDF-RT
47NINDS
48Novoseek
50OMIM
51OMIM via Orphanet
55PubMed
56QIAGEN
61SNOMED-CT via Orphanet
65Tumor Gene Family of Databases
66UMLS
67UMLS via Orphanet