Details of Disease

General Information of Disease (ID: DIS74KSO)

• Disease Name: Microscopic polyangiitis
• Synonyms: MPA; microscopic polyarteritis; Micropolyangiitis
• Definition: Microscopic polyangiitis (MPA) is an inflammatory, necrotizing, systemic vasculitis that affects predominantly small vessels (i.e. small arteries, arterioles, capillaries, venules) in multiple organs.
• Disease Hierarchy:
  DIS0R34X: Arteritis
  DISBEQIT: Anti-neutrophil cytoplasmic antibody-associated vasculitis
  DIS74KSO: Microscopic polyangiitis
• Disease Identifiers:
  MONDO ID: MONDO_0019124
  MESH ID: D055953
  UMLS CUI: C2347126
  MedGen ID: 389393
  Orphanet ID: 727
  SNOMED CT ID: 1144805008

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 2 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Methylprednisolone	DM4BDON	Approved	Small molecular drug	[1]
Prednisolone	DMQ8FR2	Approved	Small molecular drug	[2]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 4 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
PMS1	TTX1ISF	Limited	Genetic Variation	[3]
SLCO1B3	TTU86P0	Limited	Genetic Variation	[4]
MPO	TTVCZPI	Strong	Biomarker	[5]
PRTN3	TT5MLC4	Strong	Biomarker	[6]

This Disease Is Related to 13 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
BANF1	OTP7Z38L	Limited	Biomarker	[7]
DEFA4	OTOQWE4C	Limited	Biomarker	[8]
FBL	OTRODIE5	Limited	Biomarker	[9]
FCN1	OTK6ZHXH	Limited	Altered Expression	[10]
FEV	OTYEC4IR	Limited	Biomarker	[11]
GCA	OTAJ7ZHG	Limited	Genetic Variation	[12]
ITPA	OTQ47WVR	Limited	Genetic Variation	[13]
KIR2DS3	OT3PSLDL	Limited	Biomarker	[14]
KIR3DS1	OTJWIO4T	Limited	Biomarker	[14]
LILRA1	OTZ4NA7Q	Limited	Biomarker	[15]
LILRA2	OT1O2K47	Limited	Genetic Variation	[15]
COL9A2	OT1ZBDBV	Strong	Biomarker	[8]
GYPA	OTABU4YV	Strong	Biomarker	[16]

References

• [1] Methylprednisolone FDA Label
• [2] Prednisolone FDA Label
• [3] Novel roles for MLH3 deficiency and TLE6-like amplification in DNA mismatch repair-deficient gastrointestinal tumorigenesis and progression.PLoS Genet. 2008 Jun 13;4(6):e1000092. doi: 10.1371/journal.pgen.1000092.
• [4] Impact of SLCO1B3 polymorphisms on clinical outcomes in lung allograft recipients receiving mycophenolic acid.Pharmacogenomics J. 2020 Feb;20(1):69-79. doi: 10.1038/s41397-019-0086-0. Epub 2019 Apr 17.
• [5] Association between preexisting lung involvements and the risk of diffuse alveolar hemorrhage in patients with microscopic polyangiitis: A multi-center retrospective cohort study.Mod Rheumatol. 2020 Mar;30(2):338-344. doi: 10.1080/14397595.2019.1601855. Epub 2019 Apr 24.
• [6] Clinical implication of chronic paranasal sinusitis for the classification of microscopic polyangiitis.Int J Clin Pract. 2020 Jan;74(1):e13431. doi: 10.1111/ijcp.13431. Epub 2019 Oct 14.
• [7] Molecular targeted therapies for microscopic polyangiitis and granulomatosis with polyangiitis.Korean J Intern Med. 2019 May;34(3):492-503. doi: 10.3904/kjim.2018.366. Epub 2019 Jan 9.
• [8] Prediction of response to remission induction therapy by gene expression profiling of peripheral blood in Japanese patients with microscopic polyangiitis.Arthritis Res Ther. 2017 May 31;19(1):117. doi: 10.1186/s13075-017-1328-7.
• [9] Fibrosis-4 index at diagnosis is associated with all-cause mortality in patients with microscopic polyangiitis and granulomatosis with polyangiitis.BMC Gastroenterol. 2019 Jun 13;19(1):90. doi: 10.1186/s12876-019-1007-z.
• [10] Ficolin-1 is up-regulated in leukocytes and glomeruli from microscopic polyangiitis patients.Autoimmunity. 2013 Dec;46(8):513-24. doi: 10.3109/08916934.2013.822073. Epub 2013 Aug 15.
• [11] Pulmonary outcome in cystic fibrosis is influenced primarily by mucoid Pseudomonas aeruginosa infection and immune status and only modestly by genotype.Infect Immun. 1999 Sep;67(9):4744-50. doi: 10.1128/IAI.67.9.4744-4750.1999.
• [12] Are the 1990 American College of Rheumatology vasculitis classification criteria still valid?.Rheumatology (Oxford). 2017 Jul 1;56(7):1154-1161. doi: 10.1093/rheumatology/kex075.
• [13] Neutropenia related to an azathioprine metabolic disorder induced by an inosine triphosphate pyrophosphohydrolase (ITPA) gene mutation in a patient with PR3-ANCA-positive microscopic polyangiitis?"Honda K. Niikura T
• [14] Association of killer cell immunoglobulin-like receptor genotypes with microscopic polyangiitis.Arthritis Rheum. 2006 Mar;54(3):992-7. doi: 10.1002/art.21653.
• [15] Association of LILRA2 (ILT1, LIR7) splice site polymorphism with systemic lupus erythematosus and microscopic polyangiitis.Genes Immun. 2008 Apr;9(3):214-23. doi: 10.1038/gene.2008.5. Epub 2008 Feb 14.
• [16] Brief Report: Circulating Cytokine Profiles and Antineutrophil Cytoplasmic Antibody Specificity in Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.Arthritis Rheumatol. 2018 Jul;70(7):1114-1121. doi: 10.1002/art.40471. Epub 2018 May 7.