MCID: RPD001
MIFTS: 48

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 55 36 14 69
Idiopathic Crescentic Glomerulonephritis 69
Glomerulonephritis Rapidly Progressive 51
Crescentic Glomerulonephritis 55
Rpgn 55

Characteristics:

Orphanet epidemiological data:

55
rapidly progressive glomerulonephritis
Age of onset: All ages;

Classifications:

Orphanet: 55  
Rare renal diseases


External Ids:

Disease Ontology 12 DOID:4776
ICD10 32 N01
NCIt 46 C35264
SNOMED-CT 64 236392004 45406000
Orphanet 55 ORPHA280569
UMLS via Orphanet 70 C0221239 C0403416
ICD10 via Orphanet 33 N01
KEGG 36 H01688
UMLS 69 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 Selenium Micronutrient Network. 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 : 72 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 190)
# Related Disease Score Top Affiliating Genes
1 goodpasture syndrome 33.1 CD79A MPO
2 churg-strauss syndrome 30.5 MPO PRTN3
3 anca-associated vasculitis 30.2 MPO PRTN3
4 pneumonia 30.1 CRP ELANE MPO
5 glomerulonephritis 29.9 CD79A ICAM1 MPO PRTN3 TGFB1
6 acute poststreptococcal glomerulonephritis 29.9 C5 CD79A
7 polyarteritis nodosa 29.9 CRP MPO PRTN3
8 crescentic glomerulonephritis 29.5 ICAM1 MPO
9 membranoproliferative glomerulonephritis 29.2 CD79A ICAM1
10 henoch-schoenlein purpura 29.2 CD79A ICAM1 MPO
11 vasculitis 28.8 CD79A CRP ELANE ICAM1 MPO PRTN3
12 rheumatoid arthritis 28.5 CD79A CRP ICAM1 PRTN3 TGFB1
13 inflammatory bowel disease 27.2 CD79A CRP ELANE ICAM1 MPO PRTN3
14 systemic lupus erythematosus 26.1 C5 CD79A CRP ELANE ICAM1 MPO
15 chronic rapidly progressive glomerulonephritis 12.3
16 mononeuritis multiplex 10.4 CRP MPO
17 aortitis 10.4 CRP MPO
18 mediastinitis 10.4 CRP MPO
19 pasteurellosis 10.4 ELANE MPO
20 pulmonary aspergilloma 10.4 CRP MPO
21 ethmoid sinusitis 10.4 CRP PRTN3
22 impetigo herpetiformis 10.4 ELANE PRTN3
23 aleukemic leukemia cutis 10.4 ELANE MPO
24 proctitis 10.3 CRP MPO
25 cardiac rupture 10.3 CRP ELANE
26 pleural disease 10.3 CRP MPO
27 maxillary sinusitis 10.3 CD79A MPO
28 orbital granuloma 10.3 CD79A PRTN3
29 sclerosing keratitis 10.3 CD79A PRTN3
30 palindromic rheumatism 10.3 CRP PRTN3
31 suppurative cholangitis 10.3 CRP MPO
32 intestinal tuberculosis 10.3 CD79A CRP
33 acute maxillary sinusitis 10.3 CD79A CRP
34 ulcerative stomatitis 10.3 CD79A MPO
35 collagenous colitis 10.3 CD79A MPO
36 anterior scleritis 10.3 CD79A PRTN3
37 chronic orbital inflammation 10.3 CD79A PRTN3
38 intussusception 10.2 CD79A CRP
39 farmer's lung 10.2 CD79A ELANE
40 epileptic encephalopathy, childhood-onset 10.2 MPO PRTN3
41 pyelitis 10.2 CD79A CRP
42 plasma protein metabolism disease 10.2 CD79A ELANE
43 middle ear disease 10.2 CD79A PRTN3
44 ascending cholangitis 10.2 MPO TGFB1
45 granulomatous hepatitis 10.2 CD79A CRP
46 diabetic foot ulcers 10.2 CRP TGFB1
47 dysentery 10.2 CD79A CRP
48 arteritic anterior ischemic optic neuropathy 10.2 CRP MPO PRTN3
49 multiple cranial nerve palsy 10.2 CRP MPO PRTN3
50 ulcerative colitis 10.2 CRP MPO PRTN3

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:

43
# 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 50-24-8 5755
3
Methylprednisolone Approved, Vet_approved Phase 2, 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
7 glucocorticoids Phase 2, Phase 3
8 Methylprednisolone acetate Phase 2, Phase 3
9 Methylprednisolone Hemisuccinate Phase 2, Phase 3
10 Gastrointestinal Agents Phase 2, Phase 3
11 Neuroprotective Agents Phase 2, Phase 3
12 Hormone Antagonists Phase 2, Phase 3
13 Hormones Phase 2, Phase 3
14 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2, Phase 3
15 Peripheral Nervous System Agents Phase 2, Phase 3
16 Antiemetics Phase 2, Phase 3
17 Immunoglobulin A Phase 2, Phase 3
18 Prednisolone hemisuccinate Phase 2, Phase 3
19 Immunosuppressive Agents Phase 2, Phase 3
20 Prednisolone phosphate Phase 2, Phase 3
21 Anti-Inflammatory Agents Phase 2, Phase 3
22 Protective Agents Phase 2, Phase 3
23 Antineoplastic Agents, Hormonal Phase 2, Phase 3
24 Autonomic Agents Phase 2, Phase 3
25 Vasodilator Agents Phase 3
26 Phosphodiesterase Inhibitors Phase 3
27 Platelet Aggregation Inhibitors Phase 3
28 Radiation-Protective Agents Phase 3
29 Antioxidants 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:

38
Kidney, Neutrophil, Monocytes, T Cells, Endothelial, Testes, Liver

Publications for Rapidly Progressive Glomerulonephritis

Articles related to Rapidly Progressive Glomerulonephritis:

(show top 50) (show all 282)
# Title Authors Year
1
Rapidly progressive glomerulonephritis caused by overlap syndrome of IgG4-related tubulointerstitial nephritis and myeloperoxidase-antineutrophil cytoplasmic antibody-associated necrotising glomerulonephritis. ( 29352845 )
2018
2
Rapidly progressive glomerulonephritis due to anti-glomerular basement membrane disease accompanied by IgA nephropathy: An unusual association. ( 29265054 )
2017
3
Rapidly progressive glomerulonephritis due to coexistent anti-glomerular basement membrane and anti-myeloperoxidase antibody. ( 28709840 )
2017
4
Evidence-based clinical practice guidelines for rapidly progressive glomerulonephritis 2014. ( 27099135 )
2016
5
Induced regulatory T cells are phenotypically unstable and do not protect mice from rapidly progressive glomerulonephritis. ( 27606831 )
2016
6
Renal outcomes and risk factors for ESRD in children with rapidly progressive glomerulonephritis. ( 27542664 )
2016
7
Severe acute cardiotoxicity following two intravenous doses of cyclophosphamide in an adolescent treated for rapidly progressive glomerulonephritis. ( 27654474 )
2016
8
Interleukin-17RA Promotes Humoral Responses and Glomerular Injury in Experimental Rapidly Progressive Glomerulonephritis. ( 27941331 )
2016
9
Simultaneous comprehensive multiplex autoantibody analysis for rapidly progressive glomerulonephritis. ( 27858870 )
2016
10
Rapidly progressive glomerulonephritis due to coexistent anti-glomerular basement membrane disease and fibrillary glomerulonephritis. ( 26798468 )
2016
11
Bilateral renal infarction mimicking rapidly progressive glomerulonephritis. ( 26822292 )
2016
12
Rapidly Progressive Glomerulonephritis in Autosomal Dominant Polycystic Kidney Disease ( 27512779 )
2015
13
Presence of Anti-Glomerular Basement Membrane Antibodies and Myeloperoxidase Anti-Neutrophilic Cytoplasmic Antibodies in a Case of Rapidly Progressive Glomerulonephritis. ( 26301224 )
2015
14
The Clinicopathologic Spectrum of Rapidly Progressive Glomerulonephritis Based on Glomerular Immune Deposition and Antineutrophil Cytoplasmic Antibody. ( 26551339 )
2015
15
What is new in the management of rapidly progressive glomerulonephritis? ( 25815169 )
2015
16
Myeloid cell-mediated renal injury in rapidly progressive glomerulonephritis depends upon spleen tyrosine kinase. ( 26251216 )
2015
17
Fibrillary glomerulonephritis masquerading as rapidly progressive glomerulonephritis with pseudo-linear glomerular basement membrane staining. ( 26249548 )
2015
18
FMS-Like Tyrosine Kinase 3 Ligand Treatment Does Not Ameliorate Experimental Rapidly Progressive Glomerulonephritis. ( 25849330 )
2015
19
Microscopic polyangiitis causing diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. ( 26424944 )
2015
20
Renal hemosiderosis and rapidly progressive glomerulonephritis associated with primary hemochromatosis. ( 24588645 )
2014
21
Pathological spectrums and renal prognosis of severe lupus patients with rapidly progressive glomerulonephritis. ( 25281226 )
2014
22
A fatal case of propylthiouracil-induced ANCA-associated vasculitis resulting in rapidly progressive glomerulonephritis, acute hepatic failure, and cerebral angiitis. ( 25208313 )
2014
23
Rapidly progressive glomerulonephritis in tuberculosis. ( 24969205 )
2014
24
Rapidly progressive glomerulonephritis due to anti-glomerular basement membrane disease accompanied by IgA nephropathy: a case report. ( 23149250 )
2014
25
Rapidly progressive glomerulonephritis in a patient with familial Mediterranean fever and renal amyloidosis. ( 23924034 )
2013
26
A case of IgA nephropathy associated with mycosis fungoides that developed into rapidly progressive glomerulonephritis. ( 23364207 )
2013
27
Antineutrophil cytoplasmic antibody-associated vasculitis involving diffuse alveolar hemorrhage, rapidly progressive glomerulonephritis and hypereosinophilia. ( 24088762 )
2013
28
Pathogenesis of rapidly progressive glomerulonephritis: what do we learn? ( 23689582 )
2013
29
Complicated acute motor axonal neuropathy with delayed acute respiratory distress syndrome and rapidly progressive glomerulonephritis: a case report. ( 23479243 )
2013
30
Plasma exchange in the treatment of rapidly progressive glomerulonephritis. ( 23689585 )
2013
31
Fibrillary glomerulonephritis presenting as rapidly progressive glomerulonephritis. ( 22398109 )
2012
32
Renal histology in pauci-immune rapidly progressive glomerulonephritis: 8-year retrospective study. ( 22499296 )
2012
33
Rapidly progressive glomerulonephritis associated with PR3-ANCA positive subacute bacterial endocarditis. ( 22989831 )
2012
34
Rapidly progressive glomerulonephritis in a patient with renal amyloidosis: Case report and review of the literature. ( 23326051 )
2012
35
Rapidly progressive glomerulonephritis as a presenting feature of chronic lymphocytic leukemia. ( 23233032 )
2012
36
Plasma exchange for renal vasculitis and idiopathic rapidly progressive glomerulonephritis: a meta-analysis. ( 21194817 )
2011
37
Rapidly progressive glomerulonephritis complicating primary AL amyloidosis and multiple myeloma. ( 20042399 )
2010
38
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
39
Challenges in clinical-pathologic correlations: acute tubular necrosis in a patient with collapsing focal and segmental glomerulosclerosis mimicking rapidly progressive glomerulonephritis. ( 20722570 )
2010
40
ANCA-positive pauci-immune rapidly progressive glomerulonephritis and the nephrotic syndrome. ( 20427883 )
2010
41
Churg-Strauss syndrome presenting with diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. ( 19834272 )
2009
42
A nationwide survey of rapidly progressive glomerulonephritis in Japan: etiology, prognosis and treatment diversity. ( 19533266 )
2009
43
Rapidly progressive glomerulonephritis in a child with Henoch-SchAPnlein Vasculitis and familial Mediterranean fever. ( 19422708 )
2009
44
Atypical clinical presentation of Churg-Strauss syndrome with rapidly progressive glomerulonephritis: case report. ( 20010471 )
2009
45
[Case of rapidly progressive glomerulonephritis with anti-glomerular basement membrane antibody in the course of MPO-ANCA-associated pachymeningitis]. ( 19601559 )
2009
46
Anti-glomerular basement membrane antibody disease in Japan: part of the nationwide rapidly progressive glomerulonephritis survey in Japan. ( 18392773 )
2008
47
First report of rapidly progressive glomerulonephritis in tumor necrosis factor receptor-associated periodic syndrome. ( 18821681 )
2008
48
Association of idiopathic retroperitoneal fibrosis, rapidly progressive glomerulonephritis and antiproteinase 3 antineutrophil cytoplasmic antibodies (anti PR3-ANCA). ( 18793568 )
2008
49
Fibrotic mechanisms in idiopathic rapidly progressive glomerulonephritis: the role of TGF-beta1 and C5b-9. ( 18300128 )
2008
50
High incidence of Dieulafoy's lesions in upper gastrointestinal bleeding associated with polyarteritis--clinical examination of patients of polyarteritis nodosa with rapidly progressive glomerulonephritis. ( 18705275 )
2008

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 region GO:0005576 9.8 C5 CRP ELANE MPO PRTN3 TGFB1
2 azurophil granule lumen GO:0035578 9.43 ELANE MPO PRTN3
3 secretory granule GO:0030141 9.33 ELANE MPO TGFB1
4 extracellular space GO:0005615 9.17 C5 CRP ELANE ICAM1 MPO PRTN3
5 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 leukocyte migration GO:0050900 9.7 ELANE ICAM1 TGFB1
3 defense response to bacterium GO:0042742 9.69 ELANE MPO SYK
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 9.49 ELANE MPO
8 leukocyte cell-cell adhesion GO:0007159 9.48 ICAM1 SYK
9 positive regulation of vascular endothelial growth factor production GO:0010575 9.46 C5 TGFB1
10 negative regulation of growth of symbiont in host GO:0044130 9.4 ELANE MPO
11 positive regulation of superoxide anion generation GO:0032930 9.37 CRP TGFB1
12 negative regulation of phagocytosis GO:0050765 9.32 PRTN3 TGFB1
13 acute inflammatory response to antigenic stimulus GO:0002438 9.16 ELANE ICAM1
14 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.13 ICAM1 SYK TGFB1
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
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
Content
Loading form....