MCID: PCM002
MIFTS: 30

Pauci-Immune Glomerulonephritis

Categories: Immune diseases, Nephrological diseases, Rare diseases

Aliases & Classifications for Pauci-Immune Glomerulonephritis

MalaCards integrated aliases for Pauci-Immune Glomerulonephritis:

Name: Pauci-Immune Glomerulonephritis 60

Characteristics:

Orphanet epidemiological data:

60
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: 60  
Rare renal diseases


External Ids:

ICD10 via Orphanet 35 N05.7
Orphanet 60 ORPHA93126

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 SMAD Signaling Network. Affiliated tissues include neutrophil 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:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 embryo MP:0005380 9.46 BMP7 CCN2 GREM1 SMAD2
2 respiratory system MP:0005388 9.26 BMP7 CCN2 GREM1 SMAD2
3 skeleton MP:0005390 8.92 BMP7 CCN2 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:

42
Neutrophil, Bone

Publications for Pauci-Immune Glomerulonephritis

Articles related to Pauci-Immune Glomerulonephritis:

(show top 50) (show all 52)
# Title Authors Year
1
Ciliary metaplasia in a patient with antineutrophil cytoplasmic autoantibody-associated pauci-immune glomerulonephritis. ( 29295613 )
2019
2
Paraneoplastic Seronegative Pauci-Immune Glomerulonephritis Associated with Lung Adenocarcinoma Responds to Rituximab: A Case Report. ( 30022938 )
2018
3
Pulmonary renal syndrome: A case report of diffuse alveolar hemorrhage in association with ANCA negative pauci-immune glomerulonephritis. ( 29879042 )
2018
4
Pauci-immune Glomerulonephritis in Systemic Lupus Erythematosus (SLE). ( 30202678 )
2018
5
Complement your knowledge with a rare cause of pauci-immune glomerulonephritis. ( 30203317 )
2018
6
Methimazole-Induced Pauci-Immune Glomerulonephritis and Anti-Phospholipid Syndrome: An Important Association to Be Aware of. ( 30214651 )
2018
7
ANCA-associated pauci-immune glomerulonephritis in a patient with bacterial endocarditis: a challenging clinical dilemma. ( 29043145 )
2017
8
Paraneoplastic pauci-immune glomerulonephritis in a patient with lung carcinoma. ( 28946967 )
2017
9
Antineutrophil cytoplasmic antibody-positive pauci-immune glomerulonephritis associated with mantle cell lymphoma. ( 29043141 )
2017
10
A case report of paraproteinemia-associated pauci-immune glomerulonephritis - a new form of monoclonal gammopathy of renal significance? ( 29043147 )
2017
11
ANCA-associated pauci-immune glomerulonephritis: always pauci-immune? ( 28229825 )
2017
12
Favorable renal outcome in Japanese children with ANCA-associated pauci-immune glomerulonephritis: impact of urine screening program. ( 27885416 )
2017
13
Favorable renal outcome in Japanese children with ANCA-associated pauci-immune glomerulonephritis: authors' response to comments. ( 27885417 )
2017
14
Bartonella Endocarditis and Pauci-Immune Glomerulonephritis: A Case Report and Review of the Literature. ( 27885316 )
2016
15
Beneficial Effect of Rituximab in the Treatment of Esophageal Cancer-Associated Pauci-Immune Glomerulonephritis. ( 29142922 )
2016
16
Pauci-immune glomerulonephritis in a captive chimpanzee (Pan troglodytes), and a review of spontaneous cases in animals. ( 27506330 )
2016
17
The Prevalence and Management of Pauci-Immune Glomerulonephritis and Vasculitis in Western Countries. ( 27536682 )
2016
18
Pauci-immune glomerulonephritis: the ANCA-negative side of the coin. ( 26820837 )
2016
19
Anti-neutrophil cytoplasmic antibody-associated pauci-immune glomerulonephritis in a patient with chronic lymphocytic leukaemia. ( 27095466 )
2016
20
Cocaine/levamisole-induced systemic vasculitis with retiform purpura and pauci-immune glomerulonephritis. ( 27119429 )
2016
21
Hydralazine-induced pauci-immune glomerulonephritis: intriguing case series with misleading diagnoses. ( 27124161 )
2016
22
Pauci-immune glomerulonephritis: does negativity of anti-neutrophilic cytoplasmic antibodies matters? ( 26269223 )
2016
23
A historical study of American patients with anti-neutrophil cytoplasmic antibody negative pauci-immune glomerulonephritis. ( 26445939 )
2016
24
Antineutrophil cytoplasmic antibody-negative pauci-immune glomerulonephritis with massive intestinal bleeding. ( 26484044 )
2015
25
Pauci-immune glomerulonephritis in children: a clinicopathologic study of 21 patients. ( 25669759 )
2015
26
ANCA-associated vasculitis and pauci-immune glomerulonephritis in HIV disease. ( 24692374 )
2014
27
Pauci-immune glomerulonephritis in individuals with disease associated with levamisole-adulterated cocaine: a series of 4 cases. ( 25398064 )
2014
28
Clinical manifestations and outcome of ANCA-related pauci-immune glomerulonephritis in patients with Sjögren's syndrome. ( 24708918 )
2014
29
Histopathological classification of pauci-immune glomerulonephritis and its impact on outcome. ( 24838685 )
2014
30
Clinical characteristics and outcome of pauci-immune glomerulonephritis in African Americans. ( 24387818 )
2014
31
Hydralazine associated pauci-immune glomerulonephritis. ( 24561415 )
2014
32
Validation of the new classification of pauci-immune glomerulonephritis in a United States cohort and its correlation with renal outcome. ( 24093336 )
2013
33
Recurrence of ANCA-negative renal-limited pauci-immune glomerulonephritis in the renal allograft. ( 26064517 )
2013
34
Limited scleroderma with pauci-immune glomerulonephritis in the presence of renal cell carcinoma. ( 23656799 )
2013
35
Nephroquiz 7: Novel treatments for antineutrophil cytoplasmic antibody-associated Pauci-immune glomerulonephritis. ( 22797104 )
2012
36
Palisaded neutrophilic and granulomatous dermatitis in an adolescent girl with perinuclear antineutrophil cytoplasmic antibody-positive pauci-immune glomerulonephritis and arthritis. ( 22980283 )
2012
37
ANCA-positive pauci-immune glomerulonephritis and febuxostat treatment. ( 26019835 )
2012
38
Diffuse tubulointerstitial nephritis associated with ANCA-negative pauci-immune glomerulonephritis. ( 22270187 )
2012
39
Anti-LAMP-2 autoantibodies in ANCA-associated pauci-immune glomerulonephritis. ( 22323640 )
2012
40
ANCA negative pauci-immune glomerulonephritis with systemic involvement. ( 20535271 )
2010
41
Immune-complex deposits in "pauci-immune" glomerulonephritis: a case report and brief review of recent literature. ( 22371811 )
2010
42
Hydralazine-induced ANCA-positive pauci-immune glomerulonephritis: a case report and literature review. ( 19814644 )
2009
43
Complement deposition in renal histopathology of patients with ANCA-associated pauci-immune glomerulonephritis. ( 18940884 )
2009
44
Pauci-immune glomerulonephritis associated with primary antiphospholipid syndrome. ( 17899091 )
2008
45
The patient with C-ANCA/PR3-ANCA-positive crescentic pauci-immune glomerulonephritis and recurrence of nephritic sediment. ( 18310720 )
2008
46
Expression of gremlin, a bone morphogenetic protein antagonist, in glomerular crescents of pauci-immune glomerulonephritis. ( 17403698 )
2007
47
Cytoplasmic antineutrophil cytoplasmic antibody positive pauci-immune glomerulonephritis associated with infectious endocarditis. ( 17176917 )
2006
48
Poor renal outcome of antineutrophil cytoplasmic antibody negative Pauci-immune glomerulonephritis in Taiwanese. ( 17000453 )
2006
49
Temporal arteritis with pauci-immune glomerulonephritis: a systemic disease. ( 15571185 )
2004
50
Prominence of cell-mediated immunity effectors in "pauci-immune" glomerulonephritis. ( 10073600 )
1999

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 CCN2
5 positive regulation of gene expression GO:0010628 9.58 BMP7 CCN2 SMAD2
6 lung development GO:0030324 9.56 CCN2 SMAD2
7 ossification GO:0001503 9.55 BMP7 CCN2
8 response to glucose GO:0009749 9.52 CCN2 SMAD2
9 negative regulation of cell death GO:0060548 9.51 BMP7 CCN2
10 pattern specification process GO:0007389 9.49 BMP7 SMAD2
11 response to peptide hormone GO:0043434 9.48 BMP7 CCN2
12 SMAD protein signal transduction GO:0060395 9.46 BMP7 SMAD2
13 embryonic limb morphogenesis GO:0030326 9.43 BMP7 GREM1
14 positive regulation of cell death GO:0010942 9.37 BMP7 CCN2
15 positive regulation of cell differentiation GO:0045597 9.32 BMP7 CCN2
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 CCN2
2 heparin binding GO:0008201 8.96 BMP7 CCN2
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 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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