RPGN
MCID: RPD001
MIFTS: 48

Rapidly Progressive Glomerulonephritis (RPGN)

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 45406000
Orphanet 59 ORPHA280569
UMLS via Orphanet 74 C0221239 C0403416
ICD10 via Orphanet 34 N01
KEGG 37 H01688

Summaries for Rapidly Progressive Glomerulonephritis

MalaCards based summary : Rapidly Progressive Glomerulonephritis, also known as idiopathic crescentic glomerulonephritis, is related to glomerulonephritis 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 Prednisolone phosphate and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include kidney, neutrophil and lung, and related phenotypes are cardiovascular system and homeostasis/metabolism

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 242)
# Related Disease Score Top Affiliating Genes
1 glomerulonephritis 31.3 ICAM1 MPO PRTN3 TGFB1
2 churg-strauss syndrome 30.3 MPO PRTN3
3 anca-associated vasculitis 30.1 MPO PRTN3
4 granulomatous angiitis 30.1 CRP PRTN3
5 vasculitis 30.0 CRP ELANE ICAM1 MPO PRTN3
6 pneumonia 29.9 CRP ELANE MPO
7 polyarteritis nodosa 29.9 CRP MPO PRTN3
8 rheumatoid arthritis 29.9 CRP ICAM1 PRTN3 TGFB1
9 henoch-schoenlein purpura 29.8 CRP ICAM1 MPO
10 acute pancreatitis 29.8 CRP ELANE MPO
11 granulomatosis with polyangiitis 29.7 ELANE MPO PRTN3
12 crescentic glomerulonephritis 29.7 ICAM1 MPO
13 inflammatory bowel disease 28.8 CRP ELANE ICAM1 MPO PRTN3 TGFB1
14 systemic lupus erythematosus 28.5 C5 CRP ELANE ICAM1 MPO PRTN3
15 chronic rapidly progressive glomerulonephritis 12.5
16 goodpasture syndrome 12.1
17 pauci-immune glomerulonephritis 10.4
18 pauci-immune glomerulonephritis without anca 10.4
19 pauci-immune glomerulonephritis with anca 10.4
20 purpura 10.2
21 amyloidosis 10.2
22 monoclonal paraproteinemia 10.2 MPO PRTN3
23 mononeuritis multiplex 10.2 CRP MPO
24 aortitis 10.2 CRP MPO
25 mediastinitis 10.2 CRP MPO
26 pulmonary aspergilloma 10.2 CRP MPO
27 chronic maxillary sinusitis 10.2 MPO PRTN3
28 arthritis 10.2
29 endocarditis 10.2
30 iga glomerulonephritis 10.2
31 hemolytic-uremic syndrome 10.2
32 hypocomplementemic urticarial vasculitis 10.2
33 anterior scleritis 10.2 MPO PRTN3
34 ethmoid sinusitis 10.2 CRP PRTN3
35 ascending cholangitis 10.2 MPO TGFB1
36 bone fracture 10.2 CRP TGFB1
37 endocardium disease 10.2 CRP PRTN3
38 proctitis 10.2 CRP MPO
39 transient arthritis 10.2 PRTN3 TGFB1
40 pelvic inflammatory disease 10.1 CRP MPO
41 suppurative cholangitis 10.1 CRP MPO
42 epileptic encephalopathy, childhood-onset 10.1 MPO PRTN3
43 palindromic rheumatism 10.1 CRP PRTN3
44 pancreatitis 10.1
45 calcific tendinitis 10.1 CRP TGFB1
46 cerebral aneurysms 10.1 ELANE MPO
47 pasteurellosis 10.1 ELANE MPO
48 appendicitis 10.1 CRP MPO
49 bronchopneumonia 10.1 ELANE MPO
50 vulvitis 10.1 ELANE MPO

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 9.63 C5 CRP ICAM1 MPO SYK TGFB1
2 homeostasis/metabolism MP:0005376 9.5 C5 CRP ELANE ICAM1 MPO SYK
3 immune system MP:0005387 9.23 C5 CRP ELANE ICAM1 MPO PRTN3

Drugs & Therapeutics for Rapidly Progressive Glomerulonephritis

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

(show all 37)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisolone phosphate Approved, Vet_approved Phase 2, Phase 3,Phase 3 302-25-0
2
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 6055-19-2, 50-18-0 2907
3
Prednisolone Approved, Vet_approved Phase 2, Phase 3,Phase 3 50-24-8 5755
4
Methylprednisolone hemisuccinate Approved Phase 2, Phase 3,Phase 3 2921-57-5
5
Prednisone Approved, Vet_approved Phase 2, Phase 3 53-03-2 5865
6
Methylprednisolone Approved, Vet_approved Phase 2, Phase 3,Phase 3 83-43-2 6741
7
Pentoxifylline Approved, Investigational Phase 3 6493-05-6 4740
8
Prednisolone hemisuccinate Experimental Phase 2, Phase 3,Phase 3 2920-86-7
9 Immunologic Factors Phase 2, Phase 3,Not Applicable
10 Methylprednisolone acetate Phase 2, Phase 3,Phase 3
11 Hormones Phase 2, Phase 3,Phase 3
12 Hormone Antagonists Phase 2, Phase 3,Phase 3
13 Peripheral Nervous System Agents Phase 2, Phase 3,Phase 3
14 Autonomic Agents Phase 2, Phase 3,Phase 3
15 glucocorticoids Phase 2, Phase 3,Phase 3
16 Immunoglobulin A Phase 2, Phase 3
17 Antineoplastic Agents, Hormonal Phase 2, Phase 3,Phase 3
18 Prednisolone acetate Phase 2, Phase 3,Phase 3
19 Neuroprotective Agents Phase 2, Phase 3,Phase 3
20 Antiemetics Phase 2, Phase 3,Phase 3
21 Anti-Inflammatory Agents Phase 2, Phase 3,Phase 3
22 Gastrointestinal Agents Phase 2, Phase 3,Phase 3
23 Immunosuppressive Agents Phase 2, Phase 3,Phase 3
24 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2, Phase 3,Phase 3
25 Protective Agents Phase 2, Phase 3,Phase 3
26 Vasodilator Agents Phase 3
27 Free Radical Scavengers Phase 3
28 Antioxidants Phase 3
29 Platelet Aggregation Inhibitors Phase 3
30 Radiation-Protective Agents Phase 3
31 Phosphodiesterase Inhibitors Phase 3
32
Methenamine Approved, Vet_approved 100-97-0 4101
33 Immunoglobulins Not Applicable
34 Autoantibodies Not Applicable
35 Antibodies Not Applicable
36 Complement System Proteins
37 Methenamine hippurate

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 Searching for Diagnostic/Prognostic Biomarkers in SLE With Renal Involvement by Proteomic Techniques Recruiting NCT03687138
4 Detection of Anti-glomerular Basement Membrane Antibodies (Anti-GBM): a Promising Biomarker for Lupus Nephritis (LN)? Recruiting NCT03664908 Not Applicable
5 Retrospective Analysis of Renal Prognosis in Patients With Chronic Kidney Disease Recruiting NCT03201406
6 Renal Biopsy Unexplained Elevated Serum Creatinine Not yet recruiting NCT03586531

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, Lung, Liver, Bone, Heart, Cortex

Publications for Rapidly Progressive Glomerulonephritis

Articles related to Rapidly Progressive Glomerulonephritis:

(show top 50) (show all 304)
# 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
Acute renal failure by rapidly progressive glomerulonephritis with IgA deposition in a patient concomitantly diagnosed with multibacillary Hansen's disease: a case report. ( 30160772 )
2018
6
Have We Made "Rapid Progress" Understanding the Pathogenesis in Rapidly Progressive Glomerulonephritis? ( 30184521 )
2018
7
PYCARD Gene Plays a Key Role in Rapidly Progressive Glomerulonephritis: Results of a Weighted Gene Co-Expression Network Analysis. ( 30189426 )
2018
8
Histopathological Classification-A Prognostic Tool for Rapidly Progressive Glomerulonephritis. ( 30344248 )
2018
9
A unique case of rapidly progressive glomerulonephritis following dexamethasone/bortezomib/thalidomide treatment for myeloma. ( 30362231 )
2018
10
Immune complex-mediated rapidly progressive glomerulonephritis in a patient with family history of systemic lupus erythematosus. ( 30381526 )
2018
11
Temporal change in life and renal prognosis of rapidly progressive glomerulonephritis in Japan via nationwide questionnaire survey. ( 30382510 )
2018
12
Management of Elderly Patients with Rapidly Progressive Glomerulonephritis. ( 29478046 )
2018
13
Treatment of Rapidly Progressive Glomerulonephritis in the Elderly. ( 29478072 )
2018
14
Rapidly progressive glomerulonephritis due to coexistent anti-glomerular basement membrane and anti-myeloperoxidase antibody. ( 28709840 )
2017
15
Rapidly progressive glomerulonephritis due to anti-glomerular basement membrane disease accompanied by IgA nephropathy: An unusual association. ( 29265054 )
2017
16
Interleukin-17RA Promotes Humoral Responses and Glomerular Injury in Experimental Rapidly Progressive Glomerulonephritis. ( 27941331 )
2016
17
Induced regulatory T cells are phenotypically unstable and do not protect mice from rapidly progressive glomerulonephritis. ( 27606831 )
2016
18
Severe acute cardiotoxicity following two intravenous doses of cyclophosphamide in an adolescent treated for rapidly progressive glomerulonephritis. ( 27654474 )
2016
19
Simultaneous comprehensive multiplex autoantibody analysis for rapidly progressive glomerulonephritis. ( 27858870 )
2016
20
Evidence-based clinical practice guidelines for rapidly progressive glomerulonephritis 2014. ( 27099135 )
2016
21
Renal outcomes and risk factors for ESRD in children with rapidly progressive glomerulonephritis. ( 27542664 )
2016
22
Bilateral renal infarction mimicking rapidly progressive glomerulonephritis. ( 26822292 )
2016
23
[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
24
Rapidly progressive glomerulonephritis due to coexistent anti-glomerular basement membrane disease and fibrillary glomerulonephritis. ( 26798468 )
2016
25
Rapidly progressive glomerulonephritis: recent advances. ( 30375819 )
2016
26
The Clinicopathologic Spectrum of Rapidly Progressive Glomerulonephritis Based on Glomerular Immune Deposition and Antineutrophil Cytoplasmic Antibody. ( 26551339 )
2015
27
What is new in the management of rapidly progressive glomerulonephritis? ( 25815169 )
2015
28
Myeloid cell-mediated renal injury in rapidly progressive glomerulonephritis depends upon spleen tyrosine kinase. ( 26251216 )
2015
29
Fibrillary glomerulonephritis masquerading as rapidly progressive glomerulonephritis with pseudo-linear glomerular basement membrane staining. ( 26249548 )
2015
30
Rapidly Progressive Glomerulonephritis in Autosomal Dominant Polycystic Kidney Disease ( 27512779 )
2015
31
Microscopic polyangiitis causing diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. ( 26424944 )
2015
32
FMS-Like Tyrosine Kinase 3 Ligand Treatment Does Not Ameliorate Experimental Rapidly Progressive Glomerulonephritis. ( 25849330 )
2015
33
Presence of Anti-Glomerular Basement Membrane Antibodies and Myeloperoxidase Anti-Neutrophilic Cytoplasmic Antibodies in a Case of Rapidly Progressive Glomerulonephritis. ( 26301224 )
2015
34
A fatal case of propylthiouracil-induced ANCA-associated vasculitis resulting in rapidly progressive glomerulonephritis, acute hepatic failure, and cerebral angiitis. ( 25208313 )
2014
35
Renal hemosiderosis and rapidly progressive glomerulonephritis associated with primary hemochromatosis. ( 24588645 )
2014
36
Rapidly progressive glomerulonephritis due to anti-glomerular basement membrane disease accompanied by IgA nephropathy: a case report. ( 23149250 )
2014
37
Pathological spectrums and renal prognosis of severe lupus patients with rapidly progressive glomerulonephritis. ( 25281226 )
2014
38
Rapidly progressive glomerulonephritis in tuberculosis. ( 24969205 )
2014
39
Plasma exchange in the treatment of rapidly progressive glomerulonephritis. ( 23689585 )
2013
40
A case of IgA nephropathy associated with mycosis fungoides that developed into rapidly progressive glomerulonephritis. ( 23364207 )
2013
41
Antineutrophil cytoplasmic antibody-associated vasculitis involving diffuse alveolar hemorrhage, rapidly progressive glomerulonephritis and hypereosinophilia. ( 24088762 )
2013
42
Pathogenesis of rapidly progressive glomerulonephritis: what do we learn? ( 23689582 )
2013
43
Complicated acute motor axonal neuropathy with delayed acute respiratory distress syndrome and rapidly progressive glomerulonephritis: a case report. ( 23479243 )
2013
44
Rapidly progressive glomerulonephritis in a patient with familial Mediterranean fever and renal amyloidosis. ( 23924034 )
2013
45
AP-VAS 2012 case report: a case of myeloperoxidase antineutrophil cytoplasmic antibody-positive microscopic polyangiitis with rapidly progressive glomerulonephritis and hearing loss. ( 28509284 )
2013
46
Rapidly progressive glomerulonephritis associated with PR3-ANCA positive subacute bacterial endocarditis. ( 22989831 )
2012
47
Rapidly progressive glomerulonephritis in a patient with renal amyloidosis: Case report and review of the literature. ( 23326051 )
2012
48
Rapidly progressive glomerulonephritis as a presenting feature of chronic lymphocytic leukemia. ( 23233032 )
2012
49
Fibrillary glomerulonephritis presenting as rapidly progressive glomerulonephritis. ( 22398109 )
2012
50
Renal histology in pauci-immune rapidly progressive glomerulonephritis: 8-year retrospective study. ( 22499296 )
2012

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

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 secretory granule GO:0030141 9.43 ELANE MPO TGFB1
3 azurophil granule lumen GO:0035578 9.33 ELANE MPO PRTN3
4 collagen-containing extracellular matrix GO:0062023 9.26 ELANE ICAM1 PRTN3 TGFB1
5 extracellular space GO:0005615 9.17 C5 CRP ELANE ICAM1 MPO PRTN3

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

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 cytokine-mediated signaling pathway GO:0019221 9.71 ICAM1 PRTN3 TGFB1
2 defense response to bacterium GO:0042742 9.7 ELANE MPO SYK
3 leukocyte migration GO:0050900 9.61 ELANE ICAM1 TGFB1
4 response to lipopolysaccharide GO:0032496 9.58 ELANE ICAM1 MPO
5 antimicrobial humoral response GO:0019730 9.55 ELANE PRTN3
6 cellular response to dexamethasone stimulus GO:0071549 9.51 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 positive regulation of superoxide anion generation GO:0032930 9.37 CRP TGFB1
11 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.33 ICAM1 SYK TGFB1
12 negative regulation of growth of symbiont in host GO:0044130 9.32 ELANE MPO
13 negative regulation of phagocytosis GO:0050765 9.26 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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