MCID: PCM002
MIFTS: 29

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 58

Characteristics:

Orphanet epidemiological data:

58
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: 58  
Rare renal diseases


External Ids:

ICD10 via Orphanet 33 N05.7
Orphanet 58 ORPHA93126

Summaries for Pauci-Immune Glomerulonephritis

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

Related Diseases for Pauci-Immune Glomerulonephritis

Diseases related to Pauci-Immune Glomerulonephritis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 80)
# Related Disease Score Top Affiliating Genes
1 crescentic glomerulonephritis 30.5 SYNPO CCN2
2 pauci-immune glomerulonephritis without anca 12.9
3 pauci-immune glomerulonephritis with anca 12.7
4 glomerulonephritis 11.0
5 vasculitis 10.8
6 anca-associated vasculitis 10.6
7 end stage renal failure 10.4
8 microscopic polyangiitis 10.4
9 systemic lupus erythematosus 10.4
10 granulomatosis with polyangiitis 10.4
11 endocarditis 10.4
12 acute kidney failure 10.4
13 churg-strauss syndrome 10.4
14 purpura 10.4
15 rapidly progressive glomerulonephritis 10.4
16 obsolete: rapidly progressive glomerulonephritis 10.4
17 exanthem 10.3
18 neutropenia 10.3
19 drug-induced vasculitis 10.3
20 diffuse alveolar hemorrhage 10.3
21 autoimmune disease 10.1
22 gastroesophageal reflux 10.1
23 esophageal cancer 10.1
24 renal cell carcinoma, nonpapillary 10.1
25 temporal arteritis 10.1
26 lymphoma, hodgkin, classic 10.1
27 myeloma, multiple 10.1
28 lymphoma, non-hodgkin, familial 10.1
29 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.1
30 lung cancer susceptibility 3 10.1
31 microvascular complications of diabetes 3 10.1
32 microvascular complications of diabetes 4 10.1
33 microvascular complications of diabetes 6 10.1
34 microvascular complications of diabetes 7 10.1
35 mantle cell lymphoma 10.1
36 infective endocarditis 10.1
37 lymphoma 10.1
38 xerophthalmia 10.1
39 thalassemia 10.1
40 respiratory failure 10.1
41 mitral valve insufficiency 10.1
42 nephrotic syndrome 10.1
43 keratoconjunctivitis sicca 10.1
44 limited scleroderma 10.1
45 arteriosclerosis 10.1
46 splenic infarction 10.1
47 dermatitis 10.1
48 membranoproliferative glomerulonephritis 10.1
49 antiphospholipid syndrome 10.1
50 adenocarcinoma 10.1

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:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 embryo MP:0005380 9.26 BMP7 CCN2 GREM1 SMAD2
2 respiratory system MP:0005388 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:

40
Kidney, Lung, Neutrophil, T Cells, Testes, Endothelial, Bone

Publications for Pauci-Immune Glomerulonephritis

Articles related to Pauci-Immune Glomerulonephritis:

(show top 50) (show all 165)
# Title Authors PMID Year
1
Successful Treatment With Multitarget Therapy of Mycophenolate Mofetil and Tacrolimus for Cyclophosphamide-Resistant Antineutrophil Cytoplasmic Antibody-Associated Pauci-Immune Glomerulonephritis Developed Independently of Systemic Lupus Erythematosus. 61
31895115 2019
2
Renal Vasculitis and Pauci-immune Glomerulonephritis Associated With Immune Checkpoint Inhibitors. 61
31204194 2019
3
A Case of Granulomatosis with Polyangiitis: Consequences of Delayed Diagnosis in a Life-threatening Malady. 61
31890388 2019
4
Sensitivity and Specificity of Pathologic Findings to Diagnose Lupus Nephritis. 61
31653670 2019
5
The role of mammalian target of rapamycin pathway in the pathogenesis of pauci-immune glomerulonephritis. 61
31658846 2019
6
Association between neutrophil-lymphocyte & platelet lymphocyte ratios with prognosis & mortality in rapidly progressive glomerulonephritis. 61
31823922 2019
7
De novo pauci-immune glomerulonephritis in renal allografts. 61
31477812 2019
8
Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement. 61
30199560 2019
9
A novel glucocorticoid-free maintenance regimen for anti-neutrophil cytoplasm antibody-associated vasculitis. 61
30239910 2019
10
Prognostic Value of Microscopic Hematuria after Induction of Remission in Antineutrophil Cytoplasmic Antibodies-Associated Vasculitis. 61
31117066 2019
11
Ciliary metaplasia in a patient with antineutrophil cytoplasmic autoantibody-associated pauci-immune glomerulonephritis. 61
29295613 2019
12
ANCA vasculitis presenting with acute interstitial nephritis without glomerular involvement. 61
31346511 2019
13
Nephrotoxicity of immune checkpoint inhibitors beyond tubulointerstitial nephritis: single-center experience. 61
30612580 2019
14
Bacterial endocarditis manifesting as autoimmune pulmonary renal syndrome: ANCA-associated lung hemorrhage and pauci-immune crescentic glomerulonephritis
. 61
30369400 2018
15
Complement your knowledge with a rare cause of pauci-immune glomerulonephritis. 61
30203317 2018
16
Case report: de novo ANCA-associated vasculitis after kidney transplantation treated with rituximab and plasma exchange. 61
30340563 2018
17
Hydralazine-associated vasculitis: Overlapping features of drug-induced lupus and vasculitis. 61
29519741 2018
18
Methimazole-Induced Pauci-Immune Glomerulonephritis and Anti-Phospholipid Syndrome: An Important Association to Be Aware of. 61
30214651 2018
19
Pauci-immune Glomerulonephritis in Systemic Lupus Erythematosus (SLE). 61
30202678 2018
20
Pulmonary renal syndrome: A case report of diffuse alveolar hemorrhage in association with ANCA negative pauci-immune glomerulonephritis. 61
29879042 2018
21
Paraneoplastic Seronegative Pauci-Immune Glomerulonephritis Associated with Lung Adenocarcinoma Responds to Rituximab: A Case Report. 61
30022938 2018
22
Patient Outcomes in Renal-Limited Antineutrophil Cytoplasmic Antibody Vasculitis With Inactive Histology. 61
29854975 2018
23
Hydralazine-associated adverse events: a report of two cases of hydralazine-induced ANCA vasculitis. 61
29738027 2018
24
Histopathological Classification-A Prognostic Tool for Rapidly Progressive Glomerulonephritis. 61
30344248 2018
25
Impact of Proteinase 3 versus Myeloperoxidase Positivity on Risk of End-Stage Renal Disease in ANCA-Associated Glomerulonephritis Stratified by Histological Classification: A Population-Based Cohort Study. 61
29854025 2018
26
Prognostic Factors in Anti-Neutrophil Cytoplasmic Antibody-Associated Glomerulonephritis with Severe Glomerular Sclerosis: A National Registry-Based Cohort Study. 61
29973979 2018
27
Management of Elderly Patients with Rapidly Progressive Glomerulonephritis. 61
29478046 2018
28
[Renal manifestation in ANCA-associated vasculitis]. 61
29359288 2018
29
Paraneoplastic pauci-immune glomerulonephritis in a patient with lung carcinoma. 61
28946967 2017
30
Antineutrophil cytoplasmic antibody-associated glomerulonephritis with immunoglobulin deposition. 61
27830351 2017
31
Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. 61
28371513 2017
32
[Extranodal NK-T-cell lymphoma, nasal type in granulomatosis with polyangiitis. A case report]. 61
28440998 2017
33
MMP2 and MMP9 associate with crescentic glomerulonephritis. 61
28584626 2017
34
ANCA-associated pauci-immune glomerulonephritis: always pauci-immune? 61
28229825 2017
35
Favorable renal outcome in Japanese children with ANCA-associated pauci-immune glomerulonephritis: authors' response to comments. 61
27885417 2017
36
Favorable renal outcome in Japanese children with ANCA-associated pauci-immune glomerulonephritis: impact of urine screening program. 61
27885416 2017
37
Cancer in ANCA-Associated Glomerulonephritis: A Registry-Based Cohort Study. 61
29403663 2017
38
ANCA-associated pauci-immune glomerulonephritis in a patient with bacterial endocarditis: a challenging clinical dilemma. 61
29043145 2017
39
Two clinical cases of granulomatosis with polyangiitis with isolated otitis media and mastoiditis. 61
29332965 2017
40
Antineutrophil cytoplasmic antibody-positive pauci-immune glomerulonephritis associated with mantle cell lymphoma. 61
29043141 2017
41
A case report of paraproteinemia-associated pauci-immune glomerulonephritis - a new form of monoclonal gammopathy of renal significance? 61
29043147 2017
42
Pauci-immune glomerulonephritis in a captive chimpanzee (Pan troglodytes), and a review of spontaneous cases in animals. 61
27506330 2016
43
"I'm coughing up blood". 61
30281235 2016
44
Kidney disease in the elderly: biopsy based data from 14 renal centers in Poland. 61
27884116 2016
45
Systemic vasculitis associated with vemurafenib treatment: Case report and literature review. 61
27861332 2016
46
Central nervous system vasculitis in pediatric microscopic polyangiitis. 61
27156236 2016
47
Beneficial Effect of Rituximab in the Treatment of Esophageal Cancer-Associated Pauci-Immune Glomerulonephritis. 61
29142922 2016
48
Bartonella Endocarditis and Pauci-Immune Glomerulonephritis: A Case Report and Review of the Literature. 61
27885316 2016
49
Clinical spectrum and outcomes of crescentic glomerulonephritis: A single center experience. 61
27512296 2016
50
Anti-neutrophil cytoplasmic antibody negative crescentic paucimmune glomerulonephritis in a case of scleroderma with systemic lupus erythematosus overlap. 61
27215259 2016

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

Cellular components related to Pauci-Immune Glomerulonephritis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 collagen-containing extracellular matrix GO:0062023 8.8 GREM1 CCN2 BMP7

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 SMAD2 GREM1 BMP7
2 negative regulation of transcription, DNA-templated GO:0045892 9.71 SMAD2 GREM1 BMP7
3 positive regulation of gene expression GO:0010628 9.67 SMAD2 CCN2 BMP7
4 animal organ morphogenesis GO:0009887 9.58 GREM1 BMP7
5 response to estradiol GO:0032355 9.58 CCN2 BMP7
6 lung development GO:0030324 9.56 SMAD2 CCN2
7 ossification GO:0001503 9.55 CCN2 BMP7
8 response to glucose GO:0009749 9.52 SMAD2 CCN2
9 negative regulation of cell death GO:0060548 9.51 CCN2 BMP7
10 SMAD protein signal transduction GO:0060395 9.48 SMAD2 BMP7
11 response to peptide hormone GO:0043434 9.46 CCN2 BMP7
12 pattern specification process GO:0007389 9.43 SMAD2 BMP7
13 embryonic limb morphogenesis GO:0030326 9.4 GREM1 BMP7
14 positive regulation of cell death GO:0010942 9.37 CCN2 BMP7
15 positive regulation of cell differentiation GO:0045597 9.32 CCN2 BMP7
16 positive regulation of epithelial to mesenchymal transition GO:0010718 9.26 SMAD2 BMP7
17 ureteric bud development GO:0001657 9.16 SMAD2 BMP7
18 mesoderm formation GO:0001707 8.96 SMAD2 BMP7
19 embryonic pattern specification GO:0009880 8.62 SMAD2 BMP7

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

# Name GO ID Score Top Affiliating Genes
1 transforming growth factor beta receptor binding GO:0005160 8.62 SMAD2 BMP7

Sources for Pauci-Immune Glomerulonephritis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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