MPA
MCID: MCR088
MIFTS: 56

Microscopic Polyangiitis (MPA) malady

Rare diseases, Neuronal diseases, Cardiovascular diseases, Nephrological diseases, Respiratory diseases, Bone diseases categories
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Summaries for Microscopic Polyangiitis

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NIH Rare Diseases:42 Microscopic polyangiitis (mpa) is a disorder that causes blood vessel inflammation (vasculitis), which can lead to organ damage. the kidneys, lungs, nerves, skin, and joints are the most commonly affected areas of the body. mpa is diagnosed in people of all ages, all ethnicities, and both genders. the cause of this disorder is unknown. last updated: 8/13/2014

MalaCards based summary: Microscopic Polyangiitis, also known as microscopic polyarteritis, is related to vasculitis and nephritis, and has symptoms including cutaneous rash, erythema/erythematous lesions/erythroderma/polymorphous erythema and hemoptysis. An important gene associated with Microscopic Polyangiitis is KIR2DS3 (killer cell immunoglobulin-like receptor, two domains, short cytoplasmic tail, 3), and among its related pathways are Fc-GammaR Pathway and Tuberculosis. The compounds tositumomab and ibritumomab have been mentioned in the context of this disorder. Affiliated tissues include skin, lung and kidney.

Wikipedia:65 Microscopic polyangiitis (also known as \"Microscopic polyarteritis,\" \"Microscopic polyarteritis... more...

Aliases & Classifications for Microscopic Polyangiitis

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Sources:
42NIH Rare Diseases, 48Orphanet, 62UMLS, 35MESH via Orphanet, 26ICD10 via Orphanet, 63UMLS via Orphanet, 25ICD10
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Microscopic Polyangiitis, Aliases & Descriptions:

Name: Microscopic Polyangiitis 42 48 62
Microscopic Polyarteritis 48 62
Vasculitis, Leukocytoclastic, Cutaneous 62
 
Hypersensitivity Angiitis 48
Micropolyangiitis 48
Mpa 48


Classifications:



Characteristics (Orphanet epidemiological data):

48
microscopic polyangiitis:
Inheritance: Sporadic; Prevalence: 1-9/100000; Age of onset: Adulthood; Age of death: Adult


External Ids:

MESH via Orphanet35 D055953
ICD10 via Orphanet26 M31.7
UMLS via Orphanet63 C0343192
ICD1025 M31.0, M31.7

Related Diseases for Microscopic Polyangiitis

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Graphical network of the top 20 diseases related to Microscopic Polyangiitis:



Diseases related to microscopic polyangiitis

Symptoms for Microscopic Polyangiitis

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Symptoms:

48 (show all 39)
  • cutaneous rash
  • erythema/erythematous lesions/erythroderma/polymorphous erythema
  • hemoptysis
  • lung/pulmonary/alveolar hemorrhage/hematoma
  • vascularitis/vasculitides/arteritis
  • renal glomerular defect/glomerulopathy
  • renal failure
  • hematuria/microhematuria
  • pollakiuria/polyuria/dysuria/anuria/acute urine retention/oliguria
  • neuritis/polyneuritis/multineuritis
  • autoimmunity/autoimmune reaction/autoantibodies
  • fever/chilling
  • biological inflammatory syndrome/increased erythrocyte sedimentation rate/crp
  • chronic skin infection/ulcerations/ulcers/cancrum
  • purpura/petichiae
  • nausea/vomiting/regurgitation/merycism/hyperemesis
  • acute abdominal pain/colic
  • acute diarrhea
  • gastrointestinal bleeding/hemorrhage/hematemesis/melena/rectorrhagia
  • intestinal perforation
  • peritonitis/peritoneal abscess
  • venous thrombosis/phlebitis/thrombophlebitis
  • mesenteric/intestinal infarction
  • myalgia/muscular pain
  • articular/joint pain/arthralgia
  • chronic uveitis/blepharitis/episcleritis/scleritis/conjonctivitis/keratitis
  • retinal vascular anomalies/retinal telangiectasia
  • nasal congestion/sinusitis/rhinitis/rhinorrhea
  • cutis marmorata/marbled skin/livedo
  • subcutaneous nodules/lipomas/tumefaction/swelling
  • pancreatitis
  • pericardium anomalies/pericarditis/absence/cysts/pericardial effusion
  • cardiac rhythm disorder/arrhythmia
  • heart/cardiac failure
  • gangrena/necrosis
  • peripheral neuropathy
  • paresthesia/dysesthesia/hypoesthesia/anesthesia/numbness
  • arthritis/synovitis/synovial proliferation
  • epistaxis/nose bleeding

HPO human phenotypes related to Microscopic Polyangiitis:

(show all 33)
id Description Frequency HPO Source Accession
1 renal insufficiency hallmark (90%) HP:0000083
2 hematuria hallmark (90%) HP:0000790
3 skin rash hallmark (90%) HP:0000988
4 polyneuropathy hallmark (90%) HP:0001271
5 hemoptysis hallmark (90%) HP:0002105
6 pulmonary embolism hallmark (90%) HP:0002204
7 vasculitis hallmark (90%) HP:0002633
8 autoimmunity hallmark (90%) HP:0002960
9 abnormality of temperature regulation hallmark (90%) HP:0004370
10 glomerulopathy hallmark (90%) HP:0100820
11 subcutaneous hemorrhage typical (50%) HP:0001933
12 diarrhea typical (50%) HP:0002014
13 nausea and vomiting typical (50%) HP:0002017
14 abdominal pain typical (50%) HP:0002027
15 gastrointestinal hemorrhage typical (50%) HP:0002239
16 peritonitis typical (50%) HP:0002586
17 arthralgia typical (50%) HP:0002829
18 myalgia typical (50%) HP:0003326
19 thrombophlebitis typical (50%) HP:0004418
20 gastrointestinal infarctions typical (50%) HP:0005244
21 skin ulcer typical (50%) HP:0200042
22 sinusitis occasional (7.5%) HP:0000246
23 epistaxis occasional (7.5%) HP:0000421
24 cutis marmorata occasional (7.5%) HP:0000965
25 arthritis occasional (7.5%) HP:0001369
26 congestive heart failure occasional (7.5%) HP:0001635
27 abnormality of the pericardium occasional (7.5%) HP:0001697
28 pancreatitis occasional (7.5%) HP:0001733
29 paresthesia occasional (7.5%) HP:0003401
30 abnormality of the retinal vasculature occasional (7.5%) HP:0008046
31 arrhythmia occasional (7.5%) HP:0011675
32 inflammatory abnormality of the eye occasional (7.5%) HP:0100533
33 gangrene occasional (7.5%) HP:0100758

Drugs & Therapeutics for Microscopic Polyangiitis

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Drug clinical trials:

Search ClinicalTrials for Microscopic Polyangiitis

Search NIH Clinical Center for Microscopic Polyangiitis

Genetic Tests for Microscopic Polyangiitis

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Anatomical Context for Microscopic Polyangiitis

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MalaCards organs/tissues related to Microscopic Polyangiitis:

32
Skin, Lung, Kidney, Neutrophil, Bone, Heart, Liver, Breast, Bone marrow, T cells, B cells, Endothelial, Spinal cord, Colon, Thyroid, Prostate, Testes

Animal Models for Microscopic Polyangiitis or affiliated genes

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Publications for Microscopic Polyangiitis

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Articles related to Microscopic Polyangiitis:

(show top 50)    (show all 322)
idTitleAuthorsYear
1
A retrospective study of microscopic polyangiitis patients presenting with pulmonary fibrosis in China. (24468083)
2014
2
Long-term damage assessment in patients with microscopic polyangiitis and renal-limited vasculitis using the Vasculitis Damage Index. (24261767)
2014
3
Cutaneous miliary tuberculosis in a patient with long-term steroid treatment for microscopic polyangiitis. (23608070)
2013
4
Hypertrophic pachymeningitis in an individual with microscopic polyangiitis. (23772738)
2013
5
Current status of the treatment of microscopic polyangiitis and granulomatosis with polyangiitis in Japan. (22760432)
2013
6
Microscopic Polyangiitis Presenting With Hemorrhagic Stroke. (23690295)
2013
7
Prediction of response to treatment by gene expression profiling of peripheral blood in patients with microscopic polyangiitis. (23690996)
2013
8
Microscopic polyangiitis secondary to silica exposure. (23886979)
2013
9
Recombinant activated factor VII for diffuse alveolar hemorrhage in microscopic polyangiitis. (22787316)
2012
10
The contrasting epidemiology of granulomatosis with polyangiitis (Wegener's) and microscopic polyangiitis. (22258386)
2012
11
Clinical features and outcome of microscopic polyangiitis under a new consensus algorithm of ANCA-associated vasculitides in Korea. (21898069)
2012
12
Effects of mycophenolate mofetil combined with corticosteroids for induction therapy of microscopic polyangiitis. (21311184)
2011
13
Chronic pulmonary bleeding as the first sign of microscopic polyangiitis associated with autoimmune thyroiditis. (21738252)
2011
14
Diffuse Alveolar Hemorrhage in a 39-year-old Woman: Unusual Initial Presentation of Microscopic Polyangiitis. (22324034)
2011
15
A case of SjAPgren syndrome with pulmonary amyloidosis complicating microscopic polyangiitis. (21437616)
2011
16
GI involvement of sigmoid mucosal erosion in a 13-year-old girl with microscopic polyangiitis. (21195409)
2011
17
A case of femoral hemorrhage in a patient with microscopic polyangiitis with low levels of myeloperoxidase-antineutrophil cytoplasmic autoantibody. (21318302)
2011
18
AC13, a C-terminal fragment of apolipoprotein A-I, is a candidate biomarker for microscopic polyangiitis. (21792834)
2011
19
Peripheral neuropathy as a first sign of microscopic polyangiitis. (21183256)
2011
20
Prevalence and outcome of pulmonary fibrosis in microscopic polyangiitis. (19926741)
2010
21
B cell abnormalities in Wegener's granulomatosis and microscopic polyangiitis: role of CD25+-expressing B cells. (20716663)
2010
22
Treatment of polyarteritis nodosa and microscopic polyangiitis without poor-prognosis factors: A prospective randomized study of one hundred twenty-four patients. (20131268)
2010
23
Treatment strategies and outcome of induction-refractory Wegener's granulomatosis or microscopic polyangiitis: analysis of 32 patients with first-line induction-refractory disease in the WEGENT trial. (20643762)
2010
24
Cutaneous manifestations and histological features of microscopic polyangiitis. (19059827)
2009
25
Hereditary hemorrhagic telangiectasia in a patient with microscopic polyangiitis. (19640374)
2009
26
Expression of CD57 on CD8+ T lymphocytes of patients with Wegener's granulomatosis and microscopic polyangiitis: evidence for continuous activation of CD8+ cells. (19646341)
2009
27
Microscopic polyangiitis: a rare ANCA-associated small-vessel vasculitis. (19032892)
2008
28
Intravenous immunoglobulin in Wegener's granulomatosis and microscopic polyangiitis. (18576349)
2008
29
The occurrence of sensorineural hearing loss in a patient with myeloperoxidase-anti-neutrophil cytoplasmic antibody-related microscopic polyangiitis. (16969635)
2007
30
Peripheral neuropathy in Wegener's granulomatosis, Churg-Strauss syndrome and microscopic polyangiitis. (17299018)
2007
31
Microscopic polyangiitis or Wegener's granulomatosis? That is the question. (16548912)
2006
32
Microscopic polyangiitis histologically confirmed by biopsy from nasal cavity and paranasal sinuses: a case report. (16625340)
2006
33
Takotsubo (ampulla-shaped) cardiomyopathy associated with microscopic polyangiitis. (15805717)
2005
34
Microscopic polyangiitis complicated by the development of prostate cancer and flutamide-induced hepatitis. (16307333)
2005
35
Microscopic polyangiitis presenting with liver dysfunction preceding rapidly progressive necrotizing glomerulonephritis. (15455987)
2004
36
Asbestosis and probable microscopic polyangiitis. (15332139)
2004
37
Microscopic polyangiitis associated with marked systemic bleeding tendency caused by disseminated intravascular coagulation. (14518675)
2003
38
Successful renal transplantation in a child with ANCA-associated microscopic polyangiitis. (12743792)
2003
39
Anti-myeloperoxidase antibodies in patients with microscopic polyangiitis. (14646155)
2003
40
Vasculitis: Wegener granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, polyarteritis nodosa, and Takayasu arteritis. (12418444)
2002
41
Historical perspective of vasculitis: polyarteritis nodosa and microscopic polyangiitis. (11798985)
2002
42
Peripheral nonulcerative keratitis as a presenting sign of microscopic polyangiitis. (11413410)
2001
43
Treatment of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: where do we stand? (11263763)
2001
44
Microscopic polyangiitis in a patient with relapsing polychondritis. (11257169)
2001
45
Microscopic polyangiitis (microscopic polyarteritis). (11296991)
2001
46
Limited cutaneous systemic sclerosis associated with MPO-ANCA positive renal small vessel vasculitis of the microscopic polyangiitis type. (10977809)
2000
47
Pachymeningitis in microscopic polyangiitis (MPA): a case report and a review of central nervous system involvement in MPA. (10895382)
2000
48
In vitro up-regulation of E-selectin and induction of interleukin-6 in endothelial cells by autoantibodies in Wegener's granulomatosis and microscopic polyangiitis. (10464553)
1999
49
Fibrosing alveolitis predating microscopic polyangiitis. (10503564)
1999
50
Microscopic polyangiitis presenting as schizophrenia. (10399798)
1999

Variations for Microscopic Polyangiitis

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Expression for genes affiliated with Microscopic Polyangiitis

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Expression patterns in normal tissues for genes affiliated with Microscopic Polyangiitis

Search GEO for disease gene expression data for Microscopic Polyangiitis.

Pathways for genes affiliated with Microscopic Polyangiitis

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Pathways related to Microscopic Polyangiitis according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.3FCGR2B, FCGR3B
29.3FCGR2B, FCGR3B
39.3FCGR3B, FCGR2B
49.3FCGR3B, FCGR2B
5
Show member pathways
9.2KIR2DL1, KIR2DS3
68.8FCGR2B, FCGR3B, MPO
7
Show member pathways
8.8KIR3DS1, KIR2DL1, KIR2DS3
8
Show member pathways
8.7KIR2DL1, KIR2DS3, FCGR2B
98.7KIR2DL1, KIR2DS3, FCGR2B
10
Show member pathways
Immune response CD16 signaling in NK cells60
8.6FCGR3B, KIR2DS3, KIR2DL1

Compounds for genes affiliated with Microscopic Polyangiitis

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Compounds related to Microscopic Polyangiitis according to GeneCards/GeneDecks:

(show all 23)
idCompoundScoreTop Affiliating Genes
1tositumomab50 1110.7FCGR2B, FCGR3B
2ibritumomab50 1110.7FCGR2B, FCGR3B
3alefacept44 1110.7FCGR2B, FCGR3B
4bevacizumab50 1110.7FCGR2B, FCGR3B
5efalizumab44 1110.7FCGR3B, FCGR2B
6daclizumab44 1110.6FCGR2B, FCGR3B
7basiliximab44 1110.6FCGR2B, FCGR3B
8natalizumab44 1110.6FCGR3B, FCGR2B
9alemtuzumab44 1110.6FCGR3B, FCGR2B
10gemtuzumab ozogamicin50 44 1111.6FCGR2B, FCGR3B
11adalimumab44 50 1111.6FCGR3B, FCGR2B
12abciximab44 1110.6FCGR2B, FCGR3B
13cetuximab44 50 1111.6FCGR2B, FCGR3B
14etanercept44 50 1111.5FCGR3B, FCGR2B
15cytochalasin b449.5FCGR3B, MPO
16n-formylmethionyl-leucylphenylalanine449.4MPO, FCGR3B
17trastuzumab44 50 1111.4FCGR2B, FCGR3B
18rituximab44 50 1111.3FCGR2B, FCGR3B
19leukotriene b444 61 2411.2MPO, FCGR3B
20fmlp449.2MPO, FCGR3B, FCGR2B
21prednisolone44 28 1111.0FCGR3B, MPO
22superoxide44 2410.0FCGR2B, FCGR3B, MPO
23lipid448.5KIR2DL1, FCGR2B, FCGR3B

GO Terms for genes affiliated with Microscopic Polyangiitis

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Cellular components related to Microscopic Polyangiitis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1integral component of plasma membraneGO:0058878.8KIR3DS1, KIR2DL1, KIR2DS3

Biological processes related to Microscopic Polyangiitis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1regulation of immune responseGO:0507769.1KIR2DL1, FCGR2B
2immune responseGO:0069557.8KIR3DS1, KIR2DL1, FCGR2B, FCGR3B

Molecular functions related to Microscopic Polyangiitis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1IgG bindingGO:0198649.3FCGR2B, FCGR3B

Products for genes affiliated with Microscopic Polyangiitis

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  • Antibodies
  • Proteins
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Sources for Microscopic Polyangiitis

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet