MCID: RPD001
MIFTS: 44

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 56 14 69
Idiopathic Crescentic Glomerulonephritis 69
Glomerulonephritis Rapidly Progressive 52
Crescentic Glomerulonephritis 56
Rpgn 56

Characteristics:

Orphanet epidemiological data:

56
rapidly progressive glomerulonephritis
Age of onset: All ages;

Classifications:

Orphanet: 56  
Rare renal diseases


External Ids:

Disease Ontology 12 DOID:4776
ICD10 33 N01
NCIt 47 C35264
SNOMED-CT 64 236392004 45406000
Orphanet 56 ORPHA280569
UMLS via Orphanet 70 C0221239 C0403416
ICD10 via Orphanet 34 N01
UMLS 69 C0221239

Summaries for Rapidly Progressive Glomerulonephritis

MalaCards based summary : Rapidly Progressive Glomerulonephritis, also known as idiopathic crescentic glomerulonephritis, is related to cryoglobulinemia and rheumatoid arthritis. An important gene associated with Rapidly Progressive Glomerulonephritis is MPO (Myeloperoxidase), and among its related pathways/superpathways are Innate Immune System and Spinal Cord Injury. The drugs Cyclophosphamide and Methylprednisolone 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 183)
id Related Disease Score Top Affiliating Genes
1 cryoglobulinemia 30.2 CRP ELANE MPO
2 rheumatoid arthritis 28.4 CD79A CRP ICAM1 TGFB1
3 systemic lupus erythematosus 27.9 CD79A CRP ICAM1 MPO PRTN3
4 chronic rapidly progressive glomerulonephritis 12.2
5 goodpasture syndrome 11.6
6 glomerulonephritis 10.9
7 cannabis dependence 10.7 CRP MPO
8 nodular ganglioneuroblastoma 10.7 CRP MPO
9 myotonic cataract 10.6 CRP MPO
10 placenta praevia 10.6 ELANE MPO
11 central nervous system leiomyosarcoma 10.6 CRP MPO
12 inhalation anthrax 10.6 CRP MPO
13 bullous pemphigoid 10.6 ELANE PRTN3
14 acute dacryoadenitis 10.6 CRP PRTN3
15 facial dermatosis 10.6 CRP MPO
16 cardiocranial syndrome 10.5 CRP ELANE
17 arthus reaction 10.5 CRP MPO
18 central nervous system mature teratoma 10.5 ELANE MPO
19 pericholangitis 10.5 CRP MPO
20 glioblastoma multiforme 10.5 MPO PRTN3
21 spastic paraplegia 43, autosomal recessive 10.5 ELANE PRTN3
22 malignant otitis externa 10.5 CD79A PRTN3
23 vulvar disease 10.5 CD79A MPO
24 46 xy gonadal dysgenesis 10.5 CD79A PRTN3
25 eosinophilic fasciitis 10.5 CD79A MPO
26 primary lacrimal atrophy 10.5 CD79A PRTN3
27 patau syndrome 10.5 CRP PRTN3
28 diphtheritic cystitis 10.5 CD79A CRP
29 leiomyosarcoma 10.4 CD79A CRP
30 pancreatic vasoactive intestinal peptide producing tumor 10.4 CD79A CRP
31 clostridium difficile colitis 10.4 CD79A MPO
32 chronic mucocutaneous candidiasis 10.4 CD79A CRP
33 toxic myocarditis 10.4 CD79A MPO
34 inclusion conjunctivitis 10.4 CD79A PRTN3
35 hemangioma of orbit 10.4 CD79A ELANE
36 ariboflavinosis 10.4 CD79A CRP
37 pyrimidine metabolic disorder 10.4 CD79A CRP
38 glycogen storage disease viii 10.4 CD79A CRP
39 diabetes mellitus, insulin-dependent, 3 10.4 MPO PRTN3
40 atrophic vulva 10.4 MPO TGFB1
41 arteriosclerosis 10.4 CD79A ELANE
42 testicular infarct 10.3 CD79A PRTN3
43 hepatocellular adenoma 10.3 CRP MPO PRTN3
44 hypolipoproteinemia 10.3 CRP MPO PRTN3
45 partial central choroid dystrophy 10.3 CRP MPO PRTN3
46 hypertrophic elongation of cervix 10.3 CD79A CRP
47 spastic entropion 10.3 CD79A CRP
48 neutropenia, severe congenital 1, autosomal dominant 10.3 ELANE TGFB1
49 vocal cord scarring 10.3 CRP MPO PRTN3
50 bardet-biedl syndrome 8 10.3 CRP ELANE

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:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.73 TGFB1 C5 CRP ICAM1 MPO SYK
2 hematopoietic system MP:0005397 9.7 CD79A ELANE ICAM1 MPO PRTN3 SYK
3 homeostasis/metabolism MP:0005376 9.56 C5 CD79A CRP ELANE ICAM1 MPO
4 immune system MP:0005387 9.28 C5 CD79A CRP ELANE ICAM1 MPO

Drugs & Therapeutics for Rapidly Progressive Glomerulonephritis

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

(show all 34)
id 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
Methylprednisolone Approved, Vet_approved Phase 2, Phase 3 83-43-2 6741
3
Prednisolone Approved, Vet_approved Phase 2, Phase 3 50-24-8 5755
4
Prednisone Approved, Vet_approved Phase 2, Phase 3 53-03-2 5865
5
Pentoxifylline Approved, Investigational Phase 3 6493-05-6 4740
6 Immunosuppressive Agents Phase 2, Phase 3
7 Protective Agents Phase 2, Phase 3
8 Antiemetics Phase 2, Phase 3
9 Anti-Inflammatory Agents Phase 2, Phase 3
10 Antineoplastic Agents, Hormonal Phase 2, Phase 3
11 Autonomic Agents Phase 2, Phase 3
12 Gastrointestinal Agents Phase 2, Phase 3
13 glucocorticoids Phase 2, Phase 3
14 Hormone Antagonists Phase 2, Phase 3
15 Hormones Phase 2, Phase 3
16 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2, Phase 3
17 Immunoglobulin A Phase 2, Phase 3
18 Methylprednisolone acetate Phase 2, Phase 3
19 Methylprednisolone Hemisuccinate Phase 2, Phase 3
20 Neuroprotective Agents Phase 2, Phase 3
21 Peripheral Nervous System Agents Phase 2, Phase 3
22 Prednisolone acetate Phase 2, Phase 3
23 Prednisolone hemisuccinate Phase 2, Phase 3
24 Prednisolone phosphate Phase 2, Phase 3
25 Antioxidants Phase 3
26 Phosphodiesterase Inhibitors Phase 3
27 Platelet Aggregation Inhibitors Phase 3
28 Radiation-Protective Agents Phase 3
29 Vasodilator Agents Phase 3
30
Lisinopril Approved, Investigational Phase 2 83915-83-7, 76547-98-3 5362119
31 Angiotensin-Converting Enzyme Inhibitors Phase 2
32 Antihypertensive Agents Phase 2
33 HIV Protease Inhibitors Phase 2
34
protease inhibitors Phase 2

Interventional clinical trials:


id 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 ACE-inhibitors in Extracapillary Glomerulonephritis Recruiting NCT02682459 Phase 2 Lisinopril
4 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:

39
Kidney, Neutrophil, Monocytes, T Cells, Endothelial, Testes, Liver

Publications for Rapidly Progressive Glomerulonephritis

Articles related to Rapidly Progressive Glomerulonephritis:

(show top 50) (show all 280)
id Title Authors Year
1
Rapidly progressive glomerulonephritis due to coexistent anti-glomerular basement membrane and anti-myeloperoxidase antibody. ( 28709840 )
2017
2
Rapidly progressive glomerulonephritis due to coexistent anti-glomerular basement membrane disease and fibrillary glomerulonephritis. ( 26798468 )
2016
3
Simultaneous comprehensive multiplex autoantibody analysis for rapidly progressive glomerulonephritis. ( 27858870 )
2016
4
Interleukin-17RA Promotes Humoral Responses and Glomerular Injury in Experimental Rapidly Progressive Glomerulonephritis. ( 27941331 )
2016
5
Renal outcomes and risk factors for ESRD in children with rapidly progressive glomerulonephritis. ( 27542664 )
2016
6
Bilateral renal infarction mimicking rapidly progressive glomerulonephritis. ( 26822292 )
2016
7
Evidence-based clinical practice guidelines for rapidly progressive glomerulonephritis 2014. ( 27099135 )
2016
8
Induced regulatory T cells are phenotypically unstable and do not protect mice from rapidly progressive glomerulonephritis. ( 27606831 )
2016
9
Severe acute cardiotoxicity following two intravenous doses of cyclophosphamide in an adolescent treated for rapidly progressive glomerulonephritis. ( 27654474 )
2016
10
Rapidly Progressive Glomerulonephritis in Autosomal Dominant Polycystic Kidney Disease ( 27512779 )
2015
11
FMS-Like Tyrosine Kinase 3 Ligand Treatment Does Not Ameliorate Experimental Rapidly Progressive Glomerulonephritis. ( 25849330 )
2015
12
Fibrillary glomerulonephritis masquerading as rapidly progressive glomerulonephritis with pseudo-linear glomerular basement membrane staining. ( 26249548 )
2015
13
Myeloid cell-mediated renal injury in rapidly progressive glomerulonephritis depends upon spleen tyrosine kinase. ( 26251216 )
2015
14
What is new in the management of rapidly progressive glomerulonephritis? ( 25815169 )
2015
15
The Clinicopathologic Spectrum of Rapidly Progressive Glomerulonephritis Based on Glomerular Immune Deposition and Antineutrophil Cytoplasmic Antibody. ( 26551339 )
2015
16
Presence of Anti-Glomerular Basement Membrane Antibodies and Myeloperoxidase Anti-Neutrophilic Cytoplasmic Antibodies in a Case of Rapidly Progressive Glomerulonephritis. ( 26301224 )
2015
17
Microscopic polyangiitis causing diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. ( 26424944 )
2015
18
Rapidly progressive glomerulonephritis in tuberculosis. ( 24969205 )
2014
19
Pathological spectrums and renal prognosis of severe lupus patients with rapidly progressive glomerulonephritis. ( 25281226 )
2014
20
Rapidly progressive glomerulonephritis due to anti-glomerular basement membrane disease accompanied by IgA nephropathy: a case report. ( 23149250 )
2014
21
A fatal case of propylthiouracil-induced ANCA-associated vasculitis resulting in rapidly progressive glomerulonephritis, acute hepatic failure, and cerebral angiitis. ( 25208313 )
2014
22
Renal hemosiderosis and rapidly progressive glomerulonephritis associated with primary hemochromatosis. ( 24588645 )
2014
23
A case of IgA nephropathy associated with mycosis fungoides that developed into rapidly progressive glomerulonephritis. ( 23364207 )
2013
24
Pathogenesis of rapidly progressive glomerulonephritis: what do we learn? ( 23689582 )
2013
25
Antineutrophil cytoplasmic antibody-associated vasculitis involving diffuse alveolar hemorrhage, rapidly progressive glomerulonephritis and hypereosinophilia. ( 24088762 )
2013
26
Rapidly progressive glomerulonephritis in a patient with familial Mediterranean fever and renal amyloidosis. ( 23924034 )
2013
27
Complicated acute motor axonal neuropathy with delayed acute respiratory distress syndrome and rapidly progressive glomerulonephritis: a case report. ( 23479243 )
2013
28
Plasma exchange in the treatment of rapidly progressive glomerulonephritis. ( 23689585 )
2013
29
Fibrillary glomerulonephritis presenting as rapidly progressive glomerulonephritis. ( 22398109 )
2012
30
Rapidly progressive glomerulonephritis as a presenting feature of chronic lymphocytic leukemia. ( 23233032 )
2012
31
Rapidly progressive glomerulonephritis in a patient with renal amyloidosis: Case report and review of the literature. ( 23326051 )
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
Plasma exchange for renal vasculitis and idiopathic rapidly progressive glomerulonephritis: a meta-analysis. ( 21194817 )
2011
35
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
36
Challenges in clinical-pathologic correlations: acute tubular necrosis in a patient with collapsing focal and segmental glomerulosclerosis mimicking rapidly progressive glomerulonephritis. ( 20722570 )
2010
37
ANCA-positive pauci-immune rapidly progressive glomerulonephritis and the nephrotic syndrome. ( 20427883 )
2010
38
Rapidly progressive glomerulonephritis complicating primary AL amyloidosis and multiple myeloma. ( 20042399 )
2010
39
Atypical clinical presentation of Churg-Strauss syndrome with rapidly progressive glomerulonephritis: case report. ( 20010471 )
2009
40
A nationwide survey of rapidly progressive glomerulonephritis in Japan: etiology, prognosis and treatment diversity. ( 19533266 )
2009
41
Rapidly progressive glomerulonephritis in a child with Henoch-SchAPnlein Vasculitis and familial Mediterranean fever. ( 19422708 )
2009
42
Churg-Strauss syndrome presenting with diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. ( 19834272 )
2009
43
[Case of rapidly progressive glomerulonephritis with anti-glomerular basement membrane antibody in the course of MPO-ANCA-associated pachymeningitis]. ( 19601559 )
2009
44
IgA deposits along glomerular basement membranes in rapidly progressive glomerulonephritis. ( 18708970 )
2008
45
Anti-glomerular basement membrane antibody disease in Japan: part of the nationwide rapidly progressive glomerulonephritis survey in Japan. ( 18392773 )
2008
46
First report of rapidly progressive glomerulonephritis in tumor necrosis factor receptor-associated periodic syndrome. ( 18821681 )
2008
47
Association of idiopathic retroperitoneal fibrosis, rapidly progressive glomerulonephritis and antiproteinase 3 antineutrophil cytoplasmic antibodies (anti PR3-ANCA). ( 18793568 )
2008
48
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
49
Fibrotic mechanisms in idiopathic rapidly progressive glomerulonephritis: the role of TGF-beta1 and C5b-9. ( 18300128 )
2008
50
A 59-kD renal antigen as a new target for rapidly progressive glomerulonephritis. ( 17472855 )
2007

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:

id Super pathways Score Top Affiliating Genes
1
Show member pathways
12.87 C5 CD79A CRP ELANE ICAM1 MPO
2 11.51 C5 ICAM1 TGFB1
3 11.22 ELANE MPO PRTN3
4 11.06 ICAM1 SYK
5 11.01 ICAM1 TGFB1
6 10.81 ELANE ICAM1 MPO
7 10.53 ICAM1 MPO

GO Terms for Rapidly Progressive Glomerulonephritis

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

id 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)
id 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 9.69 ELANE ICAM1 TGFB1
4 response to lipopolysaccharide GO:0032496 9.63 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 positive regulation of vascular endothelial growth factor production GO:0010575 9.48 C5 TGFB1
9 leukocyte cell-cell adhesion GO:0007159 9.43 ICAM1 SYK
10 negative regulation of growth of symbiont in host GO:0044130 9.4 ELANE MPO
11 negative regulation of phagocytosis GO:0050765 9.37 PRTN3 TGFB1
12 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.33 ICAM1 SYK TGFB1
13 positive regulation of superoxide anion generation GO:0032930 9.32 CRP 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
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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