MCID: RPD001
MIFTS: 47

Rapidly Progressive Glomerulonephritis

Categories: Nephrological diseases, Rare diseases

Aliases & Classifications for Rapidly Progressive Glomerulonephritis

MalaCards integrated aliases for Rapidly Progressive Glomerulonephritis:

Name: Rapidly Progressive Glomerulonephritis 12 59 37 15 73
Idiopathic Crescentic Glomerulonephritis 73
Glomerulonephritis Rapidly Progressive 55
Crescentic Glomerulonephritis 59
Rpgn 59

Characteristics:

Orphanet epidemiological data:

59
rapidly progressive glomerulonephritis
Age of onset: All ages;

Classifications:

Orphanet: 59  
Rare renal diseases


External Ids:

Disease Ontology 12 DOID:4776
ICD10 33 N01
NCIt 50 C35264
SNOMED-CT 68 236392004 45406000
Orphanet 59 ORPHA280569
UMLS via Orphanet 74 C0221239 C0403416
ICD10 via Orphanet 34 N01
KEGG 37 H01688
UMLS 73 C0221239

Summaries for Rapidly Progressive Glomerulonephritis

MalaCards based summary : Rapidly Progressive Glomerulonephritis, also known as idiopathic crescentic glomerulonephritis, is related to goodpasture syndrome and churg-strauss syndrome. An important gene associated with Rapidly Progressive Glomerulonephritis is MPO (Myeloperoxidase), and among its related pathways/superpathways are Innate Immune System and Folate Metabolism. The drugs Cyclophosphamide and Prednisolone have been mentioned in the context of this disorder. Affiliated tissues include kidney, neutrophil and monocytes, and related phenotypes are cardiovascular system and hematopoietic system

Wikipedia : 76 Rapidly progressive glomerulonephritis (RPGN) is a syndrome of the kidney that is characterized by a... more...

Related Diseases for Rapidly Progressive Glomerulonephritis

Diseases in the Rapidly Progressive Glomerulonephritis family:

Chronic Rapidly Progressive Glomerulonephritis

Diseases related to Rapidly Progressive Glomerulonephritis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 194)
# Related Disease Score Top Affiliating Genes
1 goodpasture syndrome 33.6 CD79A MPO
2 churg-strauss syndrome 30.8 MPO PRTN3
3 anca-associated vasculitis 30.6 MPO PRTN3
4 pneumonia 30.2 CRP ELANE MPO
5 acute poststreptococcal glomerulonephritis 30.1 C5 CD79A
6 polyarteritis nodosa 30.0 CRP MPO PRTN3
7 crescentic glomerulonephritis 29.5 ICAM1 MPO
8 wegener granulomatosis 29.3 CRP ELANE MPO PRTN3
9 peritonitis 29.2 CRP ICAM1 MPO
10 glomerulonephritis 29.1 CD79A ICAM1 MPO PRTN3 TGFB1
11 membranoproliferative glomerulonephritis 28.9 CD79A ICAM1
12 henoch-schoenlein purpura 28.9 CD79A ICAM1 MPO
13 vasculitis 27.8 CD79A CRP ELANE ICAM1 MPO PRTN3
14 kidney disease 27.7 CD79A CRP TGFB1
15 systemic lupus erythematosus 27.6 CD79A CRP ICAM1 MPO PRTN3
16 rheumatoid arthritis 27.6 CD79A CRP ICAM1 PRTN3 TGFB1
17 inflammatory bowel disease 26.7 CRP ELANE ICAM1 MPO PRTN3 TGFB1
18 chronic rapidly progressive glomerulonephritis 12.3
19 mononeuritis multiplex 10.7 CRP MPO
20 aortitis 10.7 CRP MPO
21 mediastinitis 10.7 CRP MPO
22 pulmonary aspergilloma 10.6 CRP MPO
23 ethmoid sinusitis 10.6 CRP PRTN3
24 cerebral aneurysms 10.6 ELANE MPO
25 pasteurellosis 10.6 ELANE MPO
26 pleural disease 10.6 CRP MPO
27 proctitis 10.6 CRP MPO
28 impetigo herpetiformis 10.5 ELANE PRTN3
29 aleukemic leukemia cutis 10.5 ELANE MPO
30 pelvic inflammatory disease 10.5 CRP MPO
31 palindromic rheumatism 10.5 CRP PRTN3
32 cardiac rupture 10.5 CRP ELANE
33 maxillary sinusitis 10.5 CD79A MPO
34 orbital granuloma 10.5 CD79A PRTN3
35 sclerosing keratitis 10.5 CD79A PRTN3
36 suppurative cholangitis 10.5 CRP MPO
37 intestinal tuberculosis 10.5 CD79A CRP
38 acute maxillary sinusitis 10.4 CD79A CRP
39 ulcerative stomatitis 10.4 CD79A MPO
40 collagenous colitis 10.4 CD79A MPO
41 epileptic encephalopathy, childhood-onset 10.4 MPO PRTN3
42 chronic orbital inflammation 10.4 CD79A PRTN3
43 intussusception 10.4 CD79A CRP
44 anterior scleritis 10.4 CD79A PRTN3
45 pyelitis 10.4 CD79A CRP
46 middle ear disease 10.4 CD79A PRTN3
47 farmer's lung 10.3 CD79A ELANE
48 ascending cholangitis 10.3 MPO TGFB1
49 dysentery 10.3 CD79A CRP
50 granulomatous hepatitis 10.3 CD79A CRP

Graphical network of the top 20 diseases related to Rapidly Progressive Glomerulonephritis:



Diseases related to Rapidly Progressive Glomerulonephritis

Symptoms & Phenotypes for Rapidly Progressive Glomerulonephritis

MGI Mouse Phenotypes related to Rapidly Progressive Glomerulonephritis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.73 MPO SYK TGFB1 C5 CRP ICAM1
2 hematopoietic system MP:0005397 9.7 MPO PRTN3 SYK TGFB1 CD79A ELANE
3 homeostasis/metabolism MP:0005376 9.56 MPO SYK TGFB1 C5 CD79A CRP
4 immune system MP:0005387 9.28 MPO PRTN3 SYK TGFB1 C5 CD79A

Drugs & Therapeutics for Rapidly Progressive Glomerulonephritis

Drugs for Rapidly Progressive Glomerulonephritis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 29)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
2
Prednisolone Approved, Vet_approved Phase 2, Phase 3,Phase 3 50-24-8 5755
3
Methylprednisolone Approved, Vet_approved Phase 2, Phase 3,Phase 3 83-43-2 6741
4
Prednisone Approved, Vet_approved Phase 2, Phase 3 53-03-2 5865
5
Pentoxifylline Approved, Investigational Phase 3 6493-05-6 4740
6 Prednisolone acetate Phase 2, Phase 3,Phase 3
7 glucocorticoids Phase 2, Phase 3,Phase 3
8 Methylprednisolone acetate Phase 2, Phase 3,Phase 3
9 Methylprednisolone Hemisuccinate Phase 2, Phase 3,Phase 3
10 Gastrointestinal Agents Phase 2, Phase 3,Phase 3
11 Neuroprotective Agents Phase 2, Phase 3,Phase 3
12 Hormone Antagonists Phase 2, Phase 3,Phase 3
13 Hormones Phase 2, Phase 3,Phase 3
14 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2, Phase 3,Phase 3
15 Peripheral Nervous System Agents Phase 2, Phase 3,Phase 3
16 Immunoglobulin A Phase 2, Phase 3
17 Immunosuppressive Agents Phase 2, Phase 3,Phase 3
18 Prednisolone hemisuccinate Phase 2, Phase 3,Phase 3
19 Prednisolone phosphate Phase 2, Phase 3,Phase 3
20 Antiemetics Phase 2, Phase 3,Phase 3
21 Protective Agents Phase 2, Phase 3,Phase 3
22 Anti-Inflammatory Agents Phase 2, Phase 3,Phase 3
23 Autonomic Agents Phase 2, Phase 3,Phase 3
24 Antineoplastic Agents, Hormonal Phase 2, Phase 3,Phase 3
25 Vasodilator Agents Phase 3
26 Phosphodiesterase Inhibitors Phase 3
27 Platelet Aggregation Inhibitors Phase 3
28 Antioxidants Phase 3
29 Radiation-Protective Agents Phase 3

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Trial of Plasma Exchange for Severe Crescentic IgA Nephropathy Enrolling by invitation NCT02647255 Phase 2, Phase 3 Methylprednisolone pulse
2 Efficacy of Pentoxifylline on Rapidly Progressive Glomerulonephritis Terminated NCT00354198 Phase 3 pentoxifylline;corticosteroids
3 Retrospective Analysis of Renal Prognosis in Patients With Chronic Kidney Disease Recruiting NCT03201406

Search NIH Clinical Center for Rapidly Progressive Glomerulonephritis

Genetic Tests for Rapidly Progressive Glomerulonephritis

Anatomical Context for Rapidly Progressive Glomerulonephritis

MalaCards organs/tissues related to Rapidly Progressive Glomerulonephritis:

41
Kidney, Neutrophil, Monocytes, T Cells, Endothelial, Testes, Liver

Publications for Rapidly Progressive Glomerulonephritis

Articles related to Rapidly Progressive Glomerulonephritis:

(show top 50) (show all 289)
# Title Authors Year
1
The treatment of ANCA-associated rapidly-progressive glomerulonephritis and Goodpasture syndrome with therapeutic apheresis. ( 29503131 )
2018
2
Assessment of the etiologies and renal outcomes of rapidly progressive glomerulonephritis in pediatric patients at King Abdulaziz University Hospital, Jeddah, Saudi Arabia. ( 29619486 )
2018
3
Rapidly progressive glomerulonephritis: A wild card manifestation of lupus nephritis. ( 29657218 )
2018
4
Rapidly progressive glomerulonephritis caused by overlap syndrome of IgG4-related tubulointerstitial nephritis and myeloperoxidase-antineutrophil cytoplasmic antibody-associated necrotising glomerulonephritis. ( 29352845 )
2018
5
Management of Elderly Patients with Rapidly Progressive Glomerulonephritis. ( 29478046 )
2018
6
Treatment of Rapidly Progressive Glomerulonephritis in the Elderly. ( 29478072 )
2018
7
Rapidly progressive glomerulonephritis due to coexistent anti-glomerular basement membrane and anti-myeloperoxidase antibody. ( 28709840 )
2017
8
Rapidly progressive glomerulonephritis due to anti-glomerular basement membrane disease accompanied by IgA nephropathy: An unusual association. ( 29265054 )
2017
9
Interleukin-17RA Promotes Humoral Responses and Glomerular Injury in Experimental Rapidly Progressive Glomerulonephritis. ( 27941331 )
2016
10
Induced regulatory T cells are phenotypically unstable and do not protect mice from rapidly progressive glomerulonephritis. ( 27606831 )
2016
11
Severe acute cardiotoxicity following two intravenous doses of cyclophosphamide in an adolescent treated for rapidly progressive glomerulonephritis. ( 27654474 )
2016
12
Simultaneous comprehensive multiplex autoantibody analysis for rapidly progressive glomerulonephritis. ( 27858870 )
2016
13
Evidence-based clinical practice guidelines for rapidly progressive glomerulonephritis 2014. ( 27099135 )
2016
14
Renal outcomes and risk factors for ESRD in children with rapidly progressive glomerulonephritis. ( 27542664 )
2016
15
Bilateral renal infarction mimicking rapidly progressive glomerulonephritis. ( 26822292 )
2016
16
[A case of rapidly progressive glomerulonephritis exhibiting granulomatosis with polyangiitis (GPA) and extremely high level of PR3-ANCA with improvement of renal dysfunction after plasma exchange therapy]. ( 27416704 )
2016
17
Rapidly progressive glomerulonephritis due to coexistent anti-glomerular basement membrane disease and fibrillary glomerulonephritis. ( 26798468 )
2016
18
The Clinicopathologic Spectrum of Rapidly Progressive Glomerulonephritis Based on Glomerular Immune Deposition and Antineutrophil Cytoplasmic Antibody. ( 26551339 )
2015
19
What is new in the management of rapidly progressive glomerulonephritis? ( 25815169 )
2015
20
Myeloid cell-mediated renal injury in rapidly progressive glomerulonephritis depends upon spleen tyrosine kinase. ( 26251216 )
2015
21
Fibrillary glomerulonephritis masquerading as rapidly progressive glomerulonephritis with pseudo-linear glomerular basement membrane staining. ( 26249548 )
2015
22
Rapidly Progressive Glomerulonephritis in Autosomal Dominant Polycystic Kidney Disease ( 27512779 )
2015
23
Microscopic polyangiitis causing diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. ( 26424944 )
2015
24
FMS-Like Tyrosine Kinase 3 Ligand Treatment Does Not Ameliorate Experimental Rapidly Progressive Glomerulonephritis. ( 25849330 )
2015
25
Presence of Anti-Glomerular Basement Membrane Antibodies and Myeloperoxidase Anti-Neutrophilic Cytoplasmic Antibodies in a Case of Rapidly Progressive Glomerulonephritis. ( 26301224 )
2015
26
A fatal case of propylthiouracil-induced ANCA-associated vasculitis resulting in rapidly progressive glomerulonephritis, acute hepatic failure, and cerebral angiitis. ( 25208313 )
2014
27
Renal hemosiderosis and rapidly progressive glomerulonephritis associated with primary hemochromatosis. ( 24588645 )
2014
28
Rapidly progressive glomerulonephritis due to anti-glomerular basement membrane disease accompanied by IgA nephropathy: a case report. ( 23149250 )
2014
29
Pathological spectrums and renal prognosis of severe lupus patients with rapidly progressive glomerulonephritis. ( 25281226 )
2014
30
Rapidly progressive glomerulonephritis in tuberculosis. ( 24969205 )
2014
31
Plasma exchange in the treatment of rapidly progressive glomerulonephritis. ( 23689585 )
2013
32
A case of IgA nephropathy associated with mycosis fungoides that developed into rapidly progressive glomerulonephritis. ( 23364207 )
2013
33
Antineutrophil cytoplasmic antibody-associated vasculitis involving diffuse alveolar hemorrhage, rapidly progressive glomerulonephritis and hypereosinophilia. ( 24088762 )
2013
34
Pathogenesis of rapidly progressive glomerulonephritis: what do we learn? ( 23689582 )
2013
35
Complicated acute motor axonal neuropathy with delayed acute respiratory distress syndrome and rapidly progressive glomerulonephritis: a case report. ( 23479243 )
2013
36
Rapidly progressive glomerulonephritis in a patient with familial Mediterranean fever and renal amyloidosis. ( 23924034 )
2013
37
Rapidly progressive glomerulonephritis associated with PR3-ANCA positive subacute bacterial endocarditis. ( 22989831 )
2012
38
Rapidly progressive glomerulonephritis in a patient with renal amyloidosis: Case report and review of the literature. ( 23326051 )
2012
39
Rapidly progressive glomerulonephritis as a presenting feature of chronic lymphocytic leukemia. ( 23233032 )
2012
40
Fibrillary glomerulonephritis presenting as rapidly progressive glomerulonephritis. ( 22398109 )
2012
41
Renal histology in pauci-immune rapidly progressive glomerulonephritis: 8-year retrospective study. ( 22499296 )
2012
42
Plasma exchange for renal vasculitis and idiopathic rapidly progressive glomerulonephritis: a meta-analysis. ( 21194817 )
2011
43
Rapidly progressive glomerulonephritis complicating primary AL amyloidosis and multiple myeloma. ( 20042399 )
2010
44
ANCA-positive pauci-immune rapidly progressive glomerulonephritis and the nephrotic syndrome. ( 20427883 )
2010
45
Thrombotic thrombocytopenic purpura in a patient with rapidly progressive glomerulonephritis with both anti-glomerular basement membrane antibodies and myeloperoxidase anti-neutrophil cytoplasmic antibodies. ( 20628891 )
2010
46
Challenges in clinical-pathologic correlations: acute tubular necrosis in a patient with collapsing focal and segmental glomerulosclerosis mimicking rapidly progressive glomerulonephritis. ( 20722570 )
2010
47
Atypical clinical presentation of Churg-Strauss syndrome with rapidly progressive glomerulonephritis: case report. ( 20010471 )
2009
48
[Case of rapidly progressive glomerulonephritis with anti-glomerular basement membrane antibody in the course of MPO-ANCA-associated pachymeningitis]. ( 19601559 )
2009
49
Churg-Strauss syndrome presenting with diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. ( 19834272 )
2009
50
A nationwide survey of rapidly progressive glomerulonephritis in Japan: etiology, prognosis and treatment diversity. ( 19533266 )
2009

Variations for Rapidly Progressive Glomerulonephritis

Expression for Rapidly Progressive Glomerulonephritis

Search GEO for disease gene expression data for Rapidly Progressive Glomerulonephritis.

Pathways for Rapidly Progressive Glomerulonephritis

Pathways related to Rapidly Progressive Glomerulonephritis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.87 C5 CD79A CRP ELANE ICAM1 MPO
2
Show member pathways
11.88 CRP ICAM1 MPO
3 11.51 C5 ICAM1 TGFB1
4 11.22 ELANE MPO PRTN3
5 11.15 ICAM1 TGFB1
6 11.09 ICAM1 SYK
7 11.05 ICAM1 TGFB1
8 10.81 ELANE ICAM1 MPO
9 10.53 ICAM1 MPO

GO Terms for Rapidly Progressive Glomerulonephritis

Cellular components related to Rapidly Progressive Glomerulonephritis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular exosome GO:0070062 9.91 C5 CRP ELANE ICAM1 MPO PRTN3
2 extracellular region GO:0005576 9.8 C5 CRP ELANE MPO PRTN3 TGFB1
3 extracellular matrix GO:0031012 9.58 ICAM1 PRTN3 TGFB1
4 membrane raft GO:0045121 9.54 CD79A ICAM1 PRTN3
5 secretory granule GO:0030141 9.43 ELANE MPO TGFB1
6 azurophil granule lumen GO:0035578 9.33 ELANE MPO PRTN3
7 extracellular space GO:0005615 9.17 C5 CRP ELANE ICAM1 MPO PRTN3
8 B cell receptor complex GO:0019815 8.96 CD79A SYK

Biological processes related to Rapidly Progressive Glomerulonephritis according to GeneCards Suite gene sharing:

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 cell surface receptor signaling pathway GO:0007166 9.72 C5 CD79A SYK
2 defense response to bacterium GO:0042742 9.7 ELANE MPO SYK
3 leukocyte migration GO:0050900 9.67 ELANE ICAM1 TGFB1
4 response to lipopolysaccharide GO:0032496 9.61 ELANE ICAM1 MPO
5 inflammatory response GO:0006954 9.56 C5 CRP SYK TGFB1
6 cellular response to dexamethasone stimulus GO:0071549 9.54 ICAM1 TGFB1
7 defense response to fungus GO:0050832 9.51 ELANE MPO
8 positive regulation of vascular endothelial growth factor production GO:0010575 9.48 C5 TGFB1
9 leukocyte cell-cell adhesion GO:0007159 9.46 ICAM1 SYK
10 positive regulation of superoxide anion generation GO:0032930 9.4 CRP TGFB1
11 negative regulation of growth of symbiont in host GO:0044130 9.37 ELANE MPO
12 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.33 ICAM1 SYK TGFB1
13 negative regulation of phagocytosis GO:0050765 9.32 PRTN3 TGFB1
14 acute inflammatory response to antigenic stimulus GO:0002438 8.96 ELANE ICAM1
15 response to yeast GO:0001878 8.62 ELANE MPO

Sources for Rapidly Progressive Glomerulonephritis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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