MCID: PCM002
MIFTS: 30

Pauci-Immune Glomerulonephritis

Categories: Nephrological diseases, Rare diseases, Immune diseases

Aliases & Classifications for Pauci-Immune Glomerulonephritis

MalaCards integrated aliases for Pauci-Immune Glomerulonephritis:

Name: Pauci-Immune Glomerulonephritis 59

Characteristics:

Orphanet epidemiological data:

59
pauci-immune glomerulonephritis
Inheritance: Not applicable; Age of onset: Adolescent,Adult,Childhood,Elderly; Age of death: adolescent,adult,elderly,late childhood,young Adult;

Classifications:

Orphanet: 59  
Rare renal diseases


External Ids:

Orphanet 59 ORPHA93126
ICD10 via Orphanet 34 N05.7

Summaries for Pauci-Immune Glomerulonephritis

MalaCards based summary : Pauci-Immune Glomerulonephritis is related to pauci-immune glomerulonephritis without anca and pauci-immune glomerulonephritis with anca. An important gene associated with Pauci-Immune Glomerulonephritis is BMP7 (Bone Morphogenetic Protein 7), and among its related pathways/superpathways are Apelin signaling pathway and Hippo signaling pathway. Affiliated tissues include neutrophil, lung and bone, and related phenotypes are embryo and respiratory system

Related Diseases for Pauci-Immune Glomerulonephritis

Graphical network of the top 20 diseases related to Pauci-Immune Glomerulonephritis:



Diseases related to Pauci-Immune Glomerulonephritis

Symptoms & Phenotypes for Pauci-Immune Glomerulonephritis

MGI Mouse Phenotypes related to Pauci-Immune Glomerulonephritis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 embryo MP:0005380 9.46 BMP7 CTGF GREM1 SMAD2
2 respiratory system MP:0005388 9.26 BMP7 CTGF GREM1 SMAD2
3 skeleton MP:0005390 8.92 BMP7 CTGF GREM1 SMAD2

Drugs & Therapeutics for Pauci-Immune Glomerulonephritis

Search Clinical Trials , NIH Clinical Center for Pauci-Immune Glomerulonephritis

Genetic Tests for Pauci-Immune Glomerulonephritis

Anatomical Context for Pauci-Immune Glomerulonephritis

MalaCards organs/tissues related to Pauci-Immune Glomerulonephritis:

41
Neutrophil, Lung, Bone

Publications for Pauci-Immune Glomerulonephritis

Articles related to Pauci-Immune Glomerulonephritis:

(show all 46)
# Title Authors Year
1
Pulmonary renal syndrome: A case report of diffuse alveolar hemorrhage in association with ANCA negative pauci-immune glomerulonephritis. ( 29879042 )
2018
2
Ciliary metaplasia in a patient with antineutrophil cytoplasmic autoantibody-associated pauci-immune glomerulonephritis. ( 29295613 )
2018
3
Antineutrophil cytoplasmic antibody-positive pauci-immune glomerulonephritis associated with mantle cell lymphoma. ( 29043141 )
2017
4
ANCA-associated pauci-immune glomerulonephritis in a patient with bacterial endocarditis: a challenging clinical dilemma. ( 29043145 )
2017
5
ANCA-associated pauci-immune glomerulonephritis: always pauci-immune? ( 28229825 )
2017
6
Paraneoplastic pauci-immune glomerulonephritis in a patient with lung carcinoma. ( 28946967 )
2017
7
A case report of paraproteinemia-associated pauci-immune glomerulonephritis - a new form of monoclonal gammopathy of renal significance? ( 29043147 )
2017
8
Bartonella Endocarditis and Pauci-Immune Glomerulonephritis: A Case Report and Review of the Literature. ( 27885316 )
2016
9
Favorable renal outcome in Japanese children with ANCA-associated pauci-immune glomerulonephritis: impact of urine screening program. ( 27885416 )
2016
10
Favorable renal outcome in Japanese children with ANCA-associated pauci-immune glomerulonephritis: authors' response to comments. ( 27885417 )
2016
11
Pauci-immune glomerulonephritis in a captive chimpanzee (Pan troglodytes), and a review of spontaneous cases in animals. ( 27506330 )
2016
12
Beneficial Effect of Rituximab in the Treatment of Esophageal Cancer-Associated Pauci-Immune Glomerulonephritis. ( 29142922 )
2016
13
Pauci-immune glomerulonephritis: the ANCA-negative side of the coin. ( 26820837 )
2016
14
Anti-neutrophil cytoplasmic antibody-associated pauci-immune glomerulonephritis in a patient with chronic lymphocytic leukaemia. ( 27095466 )
2016
15
Hydralazine-induced pauci-immune glomerulonephritis: intriguing case series with misleading diagnoses. ( 27124161 )
2016
16
The Prevalence and Management of Pauci-Immune Glomerulonephritis and Vasculitis in Western Countries. ( 27536682 )
2016
17
Cocaine/levamisole-induced systemic vasculitis with retiform purpura and pauci-immune glomerulonephritis. ( 27119429 )
2016
18
Pauci-immune glomerulonephritis in children: A clinicopathologic study of 21 patients. ( 25669759 )
2015
19
Pauci-immune glomerulonephritis: does negativity of anti-neutrophilic cytoplasmic antibodies matters? ( 26269223 )
2015
20
Antineutrophil cytoplasmic antibody-negative pauci-immune glomerulonephritis with massive intestinal bleeding. ( 26484044 )
2015
21
A historical study of American patients with anti-neutrophil cytoplasmic antibody negative pauci-immune glomerulonephritis. ( 26445939 )
2015
22
ANCA-associated vasculitis and pauci-immune glomerulonephritis in HIV disease. ( 24692374 )
2014
23
Pauci-immune glomerulonephritis in individuals with disease associated with levamisole-adulterated cocaine: a series of 4 cases. ( 25398064 )
2014
24
Histopathological classification of pauci-immune glomerulonephritis and its impact on outcome. ( 24838685 )
2014
25
Clinical manifestations and outcome of ANCA-related pauci-immune glomerulonephritis in patients with SjAPgren's syndrome. ( 24708918 )
2014
26
Hydralazine associated pauci-immune glomerulonephritis. ( 24561415 )
2014
27
Limited scleroderma with pauci-immune glomerulonephritis in the presence of renal cell carcinoma. ( 23656799 )
2013
28
Recurrence of ANCA-negative renal-limited pauci-immune glomerulonephritis in the renal allograft. ( 26064517 )
2013
29
Clinical characteristics and outcome of pauci-immune glomerulonephritis in African Americans. ( 24387818 )
2013
30
Validation of the new classification of pauci-immune glomerulonephritis in a United States cohort and its correlation with renal outcome. ( 24093336 )
2013
31
Nephroquiz 7: Novel treatments for antineutrophil cytoplasmic antibody-associated Pauci-immune glomerulonephritis. ( 22797104 )
2012
32
Anti-LAMP-2 autoantibodies in ANCA-associated pauci-immune glomerulonephritis. ( 22323640 )
2012
33
Diffuse tubulointerstitial nephritis associated with ANCA-negative pauci-immune glomerulonephritis. ( 22270187 )
2012
34
ANCA-positive pauci-immune glomerulonephritis and febuxostat treatment. ( 26019835 )
2012
35
Palisaded neutrophilic and granulomatous dermatitis in an adolescent girl with perinuclear antineutrophil cytoplasmic antibody-positive pauci-immune glomerulonephritis and arthritis. ( 22980283 )
2012
36
ANCA negative pauci-immune glomerulonephritis with systemic involvement. ( 20535271 )
2010
37
Complement deposition in renal histopathology of patients with ANCA-associated pauci-immune glomerulonephritis. ( 18940884 )
2009
38
Hydralazine-induced ANCA-positive pauci-immune glomerulonephritis: a case report and literature review. ( 19814644 )
2009
39
The patient with C-ANCA/PR3-ANCA-positive crescentic pauci-immune glomerulonephritis and recurrence of nephritic sediment. ( 18310720 )
2008
40
Pauci-immune glomerulonephritis associated with primary antiphospholipid syndrome. ( 17899091 )
2008
41
Expression of gremlin, a bone morphogenetic protein antagonist, in glomerular crescents of pauci-immune glomerulonephritis. ( 17403698 )
2007
42
Poor renal outcome of antineutrophil cytoplasmic antibody negative Pauci-immune glomerulonephritis in Taiwanese. ( 17000453 )
2006
43
Cytoplasmic antineutrophil cytoplasmic antibody positive pauci-immune glomerulonephritis associated with infectious endocarditis. ( 17176917 )
2006
44
Temporal arteritis with pauci-immune glomerulonephritis: a systemic disease. ( 15571185 )
2004
45
Sequential development of anti-GBM nephritis and ANCA-associated Pauci-immune glomerulonephritis. ( 10430985 )
1999
46
Pauci-immune glomerulonephritis associated with bacterial infection. ( 8414162 )
1993

Variations for Pauci-Immune Glomerulonephritis

Expression for Pauci-Immune Glomerulonephritis

Search GEO for disease gene expression data for Pauci-Immune Glomerulonephritis.

Pathways for Pauci-Immune Glomerulonephritis

GO Terms for Pauci-Immune Glomerulonephritis

Biological processes related to Pauci-Immune Glomerulonephritis according to GeneCards Suite gene sharing:

(show all 19)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription, DNA-templated GO:0045893 9.74 BMP7 GREM1 SMAD2
2 negative regulation of transcription, DNA-templated GO:0045892 9.71 BMP7 GREM1 SMAD2
3 animal organ morphogenesis GO:0009887 9.58 BMP7 GREM1
4 response to estradiol GO:0032355 9.58 BMP7 CTGF
5 lung development GO:0030324 9.56 CTGF SMAD2
6 ossification GO:0001503 9.55 BMP7 CTGF
7 positive regulation of gene expression GO:0010628 9.54 BMP7 CTGF SMAD2
8 response to glucose GO:0009749 9.52 CTGF SMAD2
9 negative regulation of cell death GO:0060548 9.51 BMP7 CTGF
10 SMAD protein signal transduction GO:0060395 9.49 BMP7 SMAD2
11 response to peptide hormone GO:0043434 9.48 BMP7 CTGF
12 embryonic limb morphogenesis GO:0030326 9.46 BMP7 GREM1
13 pattern specification process GO:0007389 9.43 BMP7 SMAD2
14 positive regulation of cell death GO:0010942 9.4 BMP7 CTGF
15 positive regulation of cell differentiation GO:0045597 9.32 BMP7 CTGF
16 positive regulation of epithelial to mesenchymal transition GO:0010718 9.26 BMP7 SMAD2
17 ureteric bud development GO:0001657 9.16 BMP7 SMAD2
18 mesoderm formation GO:0001707 8.96 BMP7 SMAD2
19 embryonic pattern specification GO:0009880 8.62 BMP7 SMAD2

Molecular functions related to Pauci-Immune Glomerulonephritis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 growth factor activity GO:0008083 9.16 BMP7 CTGF
2 heparin binding GO:0008201 8.96 BMP7 CTGF
3 transforming growth factor beta receptor binding GO:0005160 8.62 BMP7 SMAD2

Sources for Pauci-Immune 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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