MCID: RPD001
MIFTS: 43

Rapidly Progressive Glomerulonephritis

Categories: Cardiovascular diseases, Immune diseases, Nephrological diseases

Aliases & Classifications for Rapidly Progressive Glomerulonephritis

MalaCards integrated aliases for Rapidly Progressive Glomerulonephritis:

Name: Rapidly Progressive Glomerulonephritis 12 36 15 70
Idiopathic Crescentic Glomerulonephritis 70
Glomerulonephritis Rapidly Progressive 54

Classifications:



External Ids:

Disease Ontology 12 DOID:4776
KEGG 36 H01688
NCIt 50 C35264
SNOMED-CT 67 45406000
ICD10 32 N01
UMLS 70 C0221239 C0403416

Summaries for Rapidly Progressive Glomerulonephritis

KEGG : 36 Rapidly progressive glomerulonephritis (RPGN) or crescentic glomerulonephritis is a life-threatening disease that destroys kidneys over a period of days to weeks. Proliferation of epithelial cells and infiltration of inflammatory cells lead to glomerular crescent formation and disruption of the specialized microvascular network in the glomerulus. This causes hematuria, albuminuria and loss of renal function. From an immunopathologic standpoint, primary RPGN is divided into pauci-immune GN (PIGN), anti-glomerular basement membrane antibody (anti-GBM) GN, and immune complex GN. PIGN, the most common etiology of primary RPGN, refers to a necrotizing glomerulonephritis with few or no immune deposits by immunofluorescence or electron microscopy. Approximately 90% of patients with PICG have circulating ANCA antibodies, leading to the systemic small vessel vasculitis such as granulomatosis with polyangiitis (GPA). Corticosteroids and cyclophosphamide are the first-line treatment, but infection is the most common cause of death of RPGN patients. Several new treatment strategies, such as leukocytaphereis (LCAP) and intravenous immunoglobulin (IVIg), have become available and these treatments have made it possible to treat high-risk RPGN patients without inducing serious immunosuppressive states.

MalaCards based summary : Rapidly Progressive Glomerulonephritis, also known as idiopathic crescentic glomerulonephritis, is related to crescentic glomerulonephritis and polyarteritis nodosa. An important gene associated with Rapidly Progressive Glomerulonephritis is PRTN3 (Proteinase 3), and among its related pathways/superpathways are Innate Immune System and Degradation of the extracellular matrix. The drugs Prednisolone acetate and Prednisolone phosphate have been mentioned in the context of this disorder. Affiliated tissues include kidney, neutrophil and t cells, and related phenotypes are homeostasis/metabolism and immune system

Wikipedia : 73 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 390)
# Related Disease Score Top Affiliating Genes
1 crescentic glomerulonephritis 31.7 PRTN3 MPO ICAM1
2 polyarteritis nodosa 31.1 PRTN3 MPO ICAM1 CRP
3 anca-associated vasculitis 31.1 PRTN3 MPO C3
4 vasculitis 30.9 PRTN3 MPO ICAM1 ELANE CRP C3
5 brucellosis 30.7 TGFB1 MPO CRP
6 mononeuritis multiplex 30.6 PRTN3 MPO CRP
7 subacute bacterial endocarditis 30.6 PRTN3 MPO CRP
8 goodpasture syndrome 30.6 PRTN3 PLA2R1 NPHS1 MPO COL4A4 COL4A3
9 granulomatosis with polyangiitis 30.6 PRTN3 MPO ELANE CRP
10 syphilis 30.5 PRTN3 CRP ALB
11 pneumonia 30.4 MPO ELANE CRP ALB
12 branch retinal artery occlusion 30.4 PRTN3 CRP
13 exanthem 30.3 SYK PRTN3 CRP ALB
14 proteinuria, chronic benign 30.3 NPHS1 COL4A4 COL4A3 ALB
15 lung abscess 30.3 PRTN3 CRP ALB
16 thrombophlebitis 30.2 TGFB1 CRP ALB
17 acute poststreptococcal glomerulonephritis 30.2 CFP C5 C3 ALB
18 hyperthyroidism 30.1 PRTN3 CRP ALB
19 erythema nodosum 30.1 ICAM1 CRP C3
20 mononeuropathy 30.1 PRTN3 MPO CRP ALB
21 immune-complex glomerulonephritis 30.1 MPO ICAM1 C3 ALB
22 bronchitis 30.1 MPO ELANE CRP ALB
23 neutropenia 30.0 MPO ELANE CRP ALB
24 microvascular complications of diabetes 3 30.0 TGFB1 NPHS1 ALB
25 otitis media 29.9 MPO ICAM1 CRP C3 ALB
26 henoch-schoenlein purpura 29.9 PRTN3 MPO ICAM1 CRP C3 ALB
27 nephrosclerosis 29.9 TGFB1 NPHS1 MPO
28 respiratory failure 29.8 MPO ICAM1 ELANE CRP ALB
29 skin disease 29.8 PRTN3 MPO ICAM1 CRP ALB
30 membranous nephropathy 29.7 PLA2R1 NPHS1 MPO C3 ALB
31 pulmonary fibrosis, idiopathic 29.6 TGFB1 MPO ICAM1 ELANE CRP ALB
32 nephrotic syndrome 29.6 NPHS1 CRP COL4A4 COL4A3 C3 ALB
33 inflammatory bowel disease 29.4 TGFB1 PRTN3 MPO ICAM1 ELANE CRP
34 type 2 diabetes mellitus 29.4 TGFB1 MPO ICAM1 ELANE CRP C3
35 glomerulonephritis 29.4 TGFB1 PRTN3 NPHS1 MPO ICAM1 ELANE
36 iga glomerulonephritis 29.3 TGFB1 PRTN3 NPHS1 ICAM1 CFP C3
37 membranoproliferative glomerulonephritis 29.2 NPHS1 ICAM1 CFP C3 ALB
38 end stage renal disease 29.1 TGFB1 NPHS1 ICAM1 CRP COL4A4 COL4A3
39 systemic lupus erythematosus 29.1 TGFB1 SYK PRTN3 MPO ICAM1 ELANE
40 kidney disease 28.9 TGFB1 PRTN3 NPHS1 CRP COL4A4 COL4A3
41 diabetes mellitus 28.7 TGFB1 MPO ICAM1 ELANE CRP CFP
42 chronic rapidly progressive glomerulonephritis 11.5
43 microscopic polyangiitis 11.2
44 acute kidney failure 10.6
45 purpura 10.6
46 pauci-immune glomerulonephritis 10.4
47 allergic cutaneous vasculitis 10.4 PRTN3 MPO
48 infective endocarditis 10.4
49 proliferative glomerulonephritis 10.4
50 anterior scleritis 10.4 PRTN3 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 homeostasis/metabolism MP:0005376 10.07 ALB C3 C5 CFP COL4A3 COL4A4
2 immune system MP:0005387 10 ALB C3 C5 CFP COL4A3 COL4A4
3 mortality/aging MP:0010768 9.73 ALB C3 C5 CFP COL4A3 COL4A4
4 renal/urinary system MP:0005367 9.23 ALB C3 C5 COL4A3 COL4A4 NPHS1

Drugs & Therapeutics for Rapidly Progressive Glomerulonephritis

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

(show all 21)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisolone acetate Approved, Vet_approved Phase 3 52-21-1
2
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
3
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
4
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
5
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5
6
Pentoxifylline Approved, Investigational Phase 3 6493-05-6 4740
7
Prednisolone hemisuccinate Experimental Phase 3 2920-86-7
8 Protective Agents Phase 3
9 Neuroprotective Agents Phase 3
10 Vasodilator Agents Phase 3
11 Immunosuppressive Agents Phase 3
12 Methylprednisolone Acetate Phase 3
13 Radiation-Protective Agents Phase 3
14 glucocorticoids Phase 3
15 Hormone Antagonists Phase 3
16 Antineoplastic Agents, Hormonal Phase 3
17 Hormones Phase 3
18 Antioxidants Phase 3
19 Phosphodiesterase Inhibitors Phase 3
20 Anti-Inflammatory Agents Phase 3
21 Platelet Aggregation Inhibitors Phase 3

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Clinical Efficacy of Combined Pentoxifylline and Conventional Immunosuppressive Regimens on Patients With Rapidly Progressive Glomerulonephritis Terminated NCT00354198 Phase 3 pentoxifylline;corticosteroids

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:

40
Kidney, Neutrophil, T Cells, Thyroid, Endothelial, Bone Marrow, Spleen

Publications for Rapidly Progressive Glomerulonephritis

Articles related to Rapidly Progressive Glomerulonephritis:

(show top 50) (show all 1311)
# Title Authors PMID Year
1
[Case of rapidly progressive glomerulonephritis with anti-glomerular basement membrane antibody in the course of MPO-ANCA-associated pachymeningitis]. 61 54
19601559 2009
2
Treatment with cytapheresis for antineutrophil cytoplasmic antibody-associated renal vasculitis and its effect on anti-inflammatory factors. 54 61
16076370 2005
3
Intravenous immunoglobulin (IVIg) therapy in MPO-ANCA related polyangiitis with rapidly progressive glomerulonephritis in Japan. 61 54
15507757 2004
4
Improved survival and amelioration of nephrotoxic nephritis in intercellular adhesion molecule-1 knockout mice. 61 54
9773781 1998
5
Rapid screening assay for anti-GBM antibody and ANCAs; an important tool for the differential diagnosis of pulmonary renal syndromes. 61 54
9306336 1997
6
Binding and inhibition of myeloperoxidase (MPO): a major function of ceruloplasmin? 61 54
9097926 1997
7
Significance of myeloperoxidase in rapidly progressive glomerulonephritis. 54 61
7611243 1995
8
Distribution of the granulocyte serine proteinases proteinase 3 and elastase in human glomerulonephritis. 61 54
7847352 1995
9
Beta 1 and beta 3 integrin upregulation in rapidly progressive glomerulonephritis. 61 54
7478117 1995
10
Some patients with anti-myeloperoxidase autoantibodies have a C-ANCA pattern. 61 54
7516271 1994
11
Classic and perinuclear anti-neutrophil cytoplasm antibodies and antimyeloperoxidase antibodies in rapidly progressive glomerulonephritis. 54 61
1665954 1991
12
The clinical and immunological features of the post-extracorporeal shock wave lithotripsy anti-glomerular basement membrane disease. 61
33435789 2021
13
Rapidly progressive IgA vasculitis-associated nephritis successfully treated with immunosuppressive therapy in an adolescent with chronic granulomatous disease. 61
33683583 2021
14
Histopathologic and clinicopathologic classifications of antineutrophil cytoplasmic antibody-associated glomerulonephritis: a validation study in a Korean cohort. 61
33789384 2021
15
Pediatric double positive anti-glomerular basement membrane antibody and anti-neutrophil cytoplasmic antibody glomerulonephritis-A case report with review of literature. 61
33629780 2021
16
Galactose-deficient IgA1 and nephritis-associated plasmin receptors as markers for IgA-dominant infection-related glomerulonephritis: A case report. 61
33592898 2021
17
Demographic, clinical and laboratory characteristics of rapidly progressive glomerulonephritis in Turkey: Turkish Society of Nephrology-Glomerular Diseases (TSN-GOLD) Working Group. 61
33040246 2021
18
Long-term outcomes in kidney transplant recipients with end-stage kidney disease due to anti-glomerular basement membrane disease. 61
33259076 2021
19
SARS-CoV-2 infection and recurrence of anti-glomerular basement disease: a case report. 61
33639869 2021
20
Evaluation of the diagnostic performance of an immunoblot for ANCA and anti-GBM antibody detection. 61
33215537 2021
21
Rapidly progressive glomerulonephritis after introduction of certolizumab pegol: a case report. 61
32687015 2021
22
Atypical anti-glomerular basement membrane disease complicated by methicillin-susceptible Staphylococcus aureus infection-related rapidly progressive glomerulonephritis: a case report and literature review. 61
33460013 2021
23
Slowly progressive anti-neutrophil cytoplasmic antibody-associated renal vasculitis: clinico-pathological characterization and outcome. 61
33564436 2021
24
Anca-positive vasculitis with full-house nephropathy, an unusual association: a case report and review of literature. 61
33501927 2021
25
Concurrent Anti-Glomerular Basement Antibody Disease and Membranous Nephropathy: A Case Series. 61
33421452 2021
26
Staphylococcus-induced glomerulonephritis: potential role for corticosteroids. 61
33504520 2021
27
A Case of Glomerulonephritis Caused by Bartonella spp. Infective Endocarditis: The Difficulty and Importance of Differentiation from Anti-neutrophil Cytoplasmic Antibody-related Rapidly Progressive Glomerulonephritis. 61
33456034 2021
28
More difficult still: Treating severe rapidly progressive glomerulonephritis in the context of COVID-19 pneumonia. 61
33781578 2021
29
Hypocomplementemic urticarial vasculitis syndrome associated with rapidly progressive glomerulonephritis: lessons for the clinical nephrologist. 61
33433859 2021
30
Slo-Mo anti-neutrophil cytoplasmic antibody-associated renal vasculitis. 61
33564403 2021
31
Plasma exchange in ANCA-associated vasculitis: the pro position. 61
33374018 2021
32
The development of rapidly progressive glomerulonephritis associated with both antineutrophil cytoplasmic antibody-associated vasculitis and anti-glomerular basement membrane nephritis in the course of nontuberculous mycobacterium infection: a case report. 61
33308324 2020
33
Impaired HVJ-stimulated Interferon producing capacity in MPO-ANCA-associated vasculitis with rapidly progressive glomerulonephritis lead to susceptibility to infection. 61
32828064 2020
34
Antineutrophil Cytoplasmic Antibody in Lupus Nephritis: Correlation with Clinicopathological Characteristics and Disease Activity. 61
33292154 2020
35
Rapidly progressive glomerulonephritis caused by tegafur/gimeracil/oteracil resulted in diabetes nephropathy, in a patient with minor risk of diabetes nephropathy: a case report. 61
32378178 2020
36
Granulomatous interstitial nephritis in granulomatosis with polyangiitis mimicking leprosy: A case report. 61
33532434 2020
37
Fibrillary glomerulonephritis presenting as crescentic glomerulonephritis in a young female: a case study. 61
32657244 2020
38
A case report of progressive multifocal leukoencephalopathy during steroid treatment for ANCA-associated renal vasculitis. 61
32388828 2020
39
IgA Nephropathy with Dominant IgA2 Deposition Accompanied by Mantle Cell Lymphoma. 61
33116018 2020
40
Characteristics and prognosis of ANCA-positive retroperitoneal fibrosis: A systematic literature review. 61
32801045 2020
41
Correction to: Temporal change in life and renal prognosis of rapidly progressive glomerulonephritis in Japan via nationwide questionnaire survey. 61
32720202 2020
42
Urinary cytology: a potential tool for differential diagnosis of acute kidney injury in patients with nephrotic syndrome. 61
32854763 2020
43
Resolution of IgA and C3 immune deposits in Staphylococcus infection-associated glomerulonephritis in a kidney transplant recipient
. 61
32567542 2020
44
An unusual presentation of propylthiouracil-induced anti-MPO and PR3 positive ANCA vasculitis with associated anti-GBM antibodies, IgA nephropathy and an IgG4 interstitial infiltrate: a case report. 61
32703233 2020
45
Rapidly progressive glomerulonephritis due to systemic lupus erythematosus and ANCA-associated vasculitis overlap. 61
32316845 2020
46
Urinary bladder rupture years after excision of urachal remnant. 61
33100570 2020
47
Non-lupus full house nephropathy in pediatrics: Case reports 61
32673451 2020
48
Renal disorders in rheumatologic diseases: the spectrum is changing (Part 1: connective tissue diseases). 61
32529559 2020
49
Rapidly progressive glomerulonephritis and acute kidney injury associated with cocaine use - Case report. 61
32573647 2020
50
Glomerular Diseases Associated with Malignancies: Histopathological Pattern and Association with Circulating Autoantibodies. 61
32466285 2020

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:

(show all 15)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.8 TGFB1 SYK PRTN3 MPO ICAM1 ELANE
2
Show member pathways
12.46 TGFB1 ICAM1 ELANE COL4A4 COL4A3
3
Show member pathways
12.07 MPO ICAM1 CRP ALB
4 11.92 TGFB1 SYK PLA2R1 C3
5 11.8 TGFB1 ICAM1 C5
6 11.71 TGFB1 COL4A4 COL4A3
7
Show member pathways
11.66 CRP CFP C5 C3
8 11.65 ICAM1 C5 C3
9
Show member pathways
11.56 CFP C5 C3
10 11.52 PRTN3 MPO ELANE
11 11.26 TGFB1 ICAM1 COL4A4 COL4A3
12 11.19 TGFB1 COL4A4 COL4A3
13 11.11 MPO ICAM1 ELANE
14 10.79 TGFB1 NPHS1 COL4A4 COL4A3
15 10.74 MPO ICAM1

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 10.03 PRTN3 NPHS1 MPO ICAM1 ELANE C5
2 extracellular region GO:0005576 9.93 TGFB1 PRTN3 PLA2R1 MPO ELANE CRP
3 cell surface GO:0009986 9.85 TGFB1 PLA2R1 ICAM1 ELANE C3
4 endoplasmic reticulum lumen GO:0005788 9.77 COL4A4 COL4A3 CFP C3 ALB
5 secretory granule GO:0030141 9.62 TGFB1 MPO ELANE CFP
6 azurophil granule lumen GO:0035578 9.56 PRTN3 MPO ELANE C3
7 collagen-containing extracellular matrix GO:0062023 9.5 TGFB1 PRTN3 ICAM1 ELANE COL4A4 COL4A3
8 extracellular space GO:0005615 9.44 TGFB1 PRTN3 MPO MIR92A1 ICAM1 ELANE
9 collagen type IV trimer GO:0005587 9.4 COL4A4 COL4A3

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

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 innate immune response GO:0045087 9.91 SYK CRP CFP C5 C3
2 neutrophil degranulation GO:0043312 9.8 PRTN3 MPO ELANE CFP C3
3 defense response to bacterium GO:0042742 9.73 SYK MPO ELANE CFP
4 regulation of complement activation GO:0030449 9.72 CFP C5 C3
5 complement activation GO:0006956 9.67 CFP C5 C3
6 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.65 TGFB1 SYK ICAM1
7 positive regulation of leukocyte adhesion to vascular endothelial cell GO:1904996 9.54 MIR92A1 ICAM1
8 positive regulation of monocyte chemotactic protein-1 production GO:0071639 9.52 SYK MIR92A1
9 collagen-activated tyrosine kinase receptor signaling pathway GO:0038063 9.46 SYK COL4A3
10 positive regulation of vascular endothelial growth factor production GO:0010575 9.43 TGFB1 C5 C3
11 acute inflammatory response to antigenic stimulus GO:0002438 9.37 ICAM1 ELANE
12 positive regulation of superoxide anion generation GO:0032930 9.33 TGFB1 SYK CRP
13 response to yeast GO:0001878 9.32 MPO ELANE
14 complement activation, alternative pathway GO:0006957 9.13 CFP C5 C3
15 glomerular basement membrane development GO:0032836 8.8 NPHS1 COL4A4 COL4A3

Sources for Rapidly Progressive Glomerulonephritis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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