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Microscopic polyangiitis

MedGen UID:
389393
•Concept ID:
C2347126
•
Disease or Syndrome
Synonyms: Microscopic Polyangiitides; Microscopic Polyangiitis; Polyangiitides, Microscopic; Polyangiitis, Microscopic
SNOMED CT: Microscopic polyarteritis (1144805008); MPA - microscopic polyangiitis (1144805008); Micropolyangiitis (1144805008); Microscopic polyangiitis (1144805008)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0019124
Orphanet: ORPHA727

Definition

A rare inflammatory necrotising systemic vasculitis that affects predominantly small vessels (i.e. small arteries, arterioles, capillaries, venules) in multiple organs, including the kidney, the lungs, the skin and the peripheral nerves. This disease is an antineutrophil cytoplasmic autoantibodies (ANCA)-associated autoimmune disease with little or no immune complex deposition. Evidence indicates that ANCA can activate neutrophils and monocytes and cause them to attack vessel walls. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMicroscopic polyangiitis

Professional guidelines

PubMed

Diagnosis and management of ANCA-associated vasculitis.
Kronbichler A, Bajema IM, Bruchfeld A, Mastroianni Kirsztajn G, Stone JH
Lancet 2024 Feb 17;403(10427):683-698. doi: 10.1016/S0140-6736(23)01736-1. PMID: 38368016
EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update.
Hellmich B, Sanchez-Alamo B, Schirmer JH, Berti A, Blockmans D, Cid MC, Holle JU, Hollinger N, Karadag O, Kronbichler A, Little MA, Luqmani RA, Mahr A, Merkel PA, Mohammad AJ, Monti S, Mukhtyar CB, Musial J, Price-Kuehne F, Segelmark M, Teng YKO, Terrier B, Tomasson G, Vaglio A, Vassilopoulos D, Verhoeven P, Jayne D
Ann Rheum Dis 2024 Jan 2;83(1):30-47. doi: 10.1136/ard-2022-223764. PMID: 36927642
Treatment Guidelines in Vasculitis.
Kermani TA, Warrington KJ, Dua AB
Rheum Dis Clin North Am 2022 Aug;48(3):705-724. Epub 2022 Jul 5 doi: 10.1016/j.rdc.2022.03.006. PMID: 35953232

Recent clinical studies

Etiology

Single-cell multi-omics analysis identifies two distinct phenotypes of newly-onset microscopic polyangiitis.
Nishide M, Nishimura K, Matsushita H, Edahiro R, Inukai S, Shimagami H, Kawada S, Kato Y, Kawasaki T, Tsujimoto K, Kamon H, Omiya R, Okada Y, Hattori K, Narazaki M, Kumanogoh A
Nat Commun 2023 Oct 11;14(1):5789. doi: 10.1038/s41467-023-41328-0. PMID: 37821442Free PMC Article
Treatment Guidelines in Vasculitis.
Kermani TA, Warrington KJ, Dua AB
Rheum Dis Clin North Am 2022 Aug;48(3):705-724. Epub 2022 Jul 5 doi: 10.1016/j.rdc.2022.03.006. PMID: 35953232
Obinutuzumab as treatment for ANCA-associated vasculitis.
Amudala NA, Boukhlal S, Sheridan B, Langford CA, Geara A, Merkel PA, Cornec D
Rheumatology (Oxford) 2022 Aug 30;61(9):3814-3817. doi: 10.1093/rheumatology/keab916. PMID: 34958343
ANCA-Associated Vasculitis: Core Curriculum 2020.
Geetha D, Jefferson JA
Am J Kidney Dis 2020 Jan;75(1):124-137. Epub 2019 Jul 26 doi: 10.1053/j.ajkd.2019.04.031. PMID: 31358311
Granulomatous Vasculitis.
Sharma A, Dogra S, Sharma K
Dermatol Clin 2015 Jul;33(3):475-87. doi: 10.1016/j.det.2015.03.012. PMID: 26143427

Diagnosis

Diagnosis and management of ANCA-associated vasculitis.
Kronbichler A, Bajema IM, Bruchfeld A, Mastroianni Kirsztajn G, Stone JH
Lancet 2024 Feb 17;403(10427):683-698. doi: 10.1016/S0140-6736(23)01736-1. PMID: 38368016
ANCA associated vasculitis (AAV): a review for internists.
Yaseen K, Mandell BF
Postgrad Med 2023 Jan;135(sup1):3-13. Epub 2022 Jul 21 doi: 10.1080/00325481.2022.2102368. PMID: 35831990
ANCA-Associated Vasculitis: Core Curriculum 2020.
Geetha D, Jefferson JA
Am J Kidney Dis 2020 Jan;75(1):124-137. Epub 2019 Jul 26 doi: 10.1053/j.ajkd.2019.04.031. PMID: 31358311
Granulomatous Vasculitis.
Sharma A, Dogra S, Sharma K
Dermatol Clin 2015 Jul;33(3):475-87. doi: 10.1016/j.det.2015.03.012. PMID: 26143427
Pulmonary vasculitis.
Lally L, Spiera RF
Rheum Dis Clin North Am 2015 May;41(2):315-31. Epub 2015 Feb 27 doi: 10.1016/j.rdc.2015.01.004. PMID: 25836645

Therapy

Diagnosis and management of ANCA-associated vasculitis.
Kronbichler A, Bajema IM, Bruchfeld A, Mastroianni Kirsztajn G, Stone JH
Lancet 2024 Feb 17;403(10427):683-698. doi: 10.1016/S0140-6736(23)01736-1. PMID: 38368016
EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update.
Hellmich B, Sanchez-Alamo B, Schirmer JH, Berti A, Blockmans D, Cid MC, Holle JU, Hollinger N, Karadag O, Kronbichler A, Little MA, Luqmani RA, Mahr A, Merkel PA, Mohammad AJ, Monti S, Mukhtyar CB, Musial J, Price-Kuehne F, Segelmark M, Teng YKO, Terrier B, Tomasson G, Vaglio A, Vassilopoulos D, Verhoeven P, Jayne D
Ann Rheum Dis 2024 Jan 2;83(1):30-47. doi: 10.1136/ard-2022-223764. PMID: 36927642
Obinutuzumab as treatment for ANCA-associated vasculitis.
Amudala NA, Boukhlal S, Sheridan B, Langford CA, Geara A, Merkel PA, Cornec D
Rheumatology (Oxford) 2022 Aug 30;61(9):3814-3817. doi: 10.1093/rheumatology/keab916. PMID: 34958343
ANCA-Associated Vasculitis: Core Curriculum 2020.
Geetha D, Jefferson JA
Am J Kidney Dis 2020 Jan;75(1):124-137. Epub 2019 Jul 26 doi: 10.1053/j.ajkd.2019.04.031. PMID: 31358311
Granulomatous Vasculitis.
Sharma A, Dogra S, Sharma K
Dermatol Clin 2015 Jul;33(3):475-87. doi: 10.1016/j.det.2015.03.012. PMID: 26143427

Prognosis

Single-cell multi-omics analysis identifies two distinct phenotypes of newly-onset microscopic polyangiitis.
Nishide M, Nishimura K, Matsushita H, Edahiro R, Inukai S, Shimagami H, Kawada S, Kato Y, Kawasaki T, Tsujimoto K, Kamon H, Omiya R, Okada Y, Hattori K, Narazaki M, Kumanogoh A
Nat Commun 2023 Oct 11;14(1):5789. doi: 10.1038/s41467-023-41328-0. PMID: 37821442Free PMC Article
Polyarteritis nodosa: an evolving primary systemic vasculitis.
Springer JM, Byram K
Postgrad Med 2023 Jan;135(sup1):61-68. Epub 2022 Jun 22 doi: 10.1080/00325481.2022.2088940. PMID: 35709399
Immunopathogenesis of ANCA-Associated Vasculitis.
Kronbichler A, Lee KH, Denicolò S, Choi D, Lee H, Ahn D, Kim KH, Lee JH, Kim H, Hwang M, Jung SW, Lee C, Lee H, Sung H, Lee D, Hwang J, Kim S, Hwang I, Kim DY, Kim HJ, Cho G, Cho Y, Kim D, Choi M, Park J, Park J, Tizaoui K, Li H, Smith L, Koyanagi A, Jacob L, Gauckler P, Shin JI
Int J Mol Sci 2020 Oct 3;21(19) doi: 10.3390/ijms21197319. PMID: 33023023Free PMC Article
ANCA-Associated Vasculitis: Core Curriculum 2020.
Geetha D, Jefferson JA
Am J Kidney Dis 2020 Jan;75(1):124-137. Epub 2019 Jul 26 doi: 10.1053/j.ajkd.2019.04.031. PMID: 31358311
Renal Involvement in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.
Zonozi R, Niles JL, Cortazar FB
Rheum Dis Clin North Am 2018 Nov;44(4):525-543. Epub 2018 Sep 7 doi: 10.1016/j.rdc.2018.06.001. PMID: 30274621

Clinical prediction guides

Immunopathogenesis of ANCA-Associated Vasculitis.
Kronbichler A, Lee KH, Denicolò S, Choi D, Lee H, Ahn D, Kim KH, Lee JH, Kim H, Hwang M, Jung SW, Lee C, Lee H, Sung H, Lee D, Hwang J, Kim S, Hwang I, Kim DY, Kim HJ, Cho G, Cho Y, Kim D, Choi M, Park J, Park J, Tizaoui K, Li H, Smith L, Koyanagi A, Jacob L, Gauckler P, Shin JI
Int J Mol Sci 2020 Oct 3;21(19) doi: 10.3390/ijms21197319. PMID: 33023023Free PMC Article
Antineutrophil Cytoplasmic Antibody-Associated Lung Fibrosis.
Borie R, Crestani B
Semin Respir Crit Care Med 2018 Aug;39(4):465-470. Epub 2018 Nov 7 doi: 10.1055/s-0038-1669914. PMID: 30404113
Predictors of Poor Outcome in ANCA-Associated Vasculitis (AAV).
Vega LE, Espinoza LR
Curr Rheumatol Rep 2016 Dec;18(12):70. doi: 10.1007/s11926-016-0619-3. PMID: 27812952
B-cell-targeted therapy in systemic vasculitis.
Lally L, Spiera R
Curr Opin Rheumatol 2016 Jan;28(1):15-20. doi: 10.1097/BOR.0000000000000235. PMID: 26599379
Pulmonary vasculitis.
Lally L, Spiera RF
Rheum Dis Clin North Am 2015 May;41(2):315-31. Epub 2015 Feb 27 doi: 10.1016/j.rdc.2015.01.004. PMID: 25836645

Recent systematic reviews

EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update.
Hellmich B, Sanchez-Alamo B, Schirmer JH, Berti A, Blockmans D, Cid MC, Holle JU, Hollinger N, Karadag O, Kronbichler A, Little MA, Luqmani RA, Mahr A, Merkel PA, Mohammad AJ, Monti S, Mukhtyar CB, Musial J, Price-Kuehne F, Segelmark M, Teng YKO, Terrier B, Tomasson G, Vaglio A, Vassilopoulos D, Verhoeven P, Jayne D
Ann Rheum Dis 2024 Jan 2;83(1):30-47. doi: 10.1136/ard-2022-223764. PMID: 36927642
CanVasc consensus recommendations for the use of avacopan in antineutrophil cytoplasm antibody-associated vasculitis: 2022 addendum.
Turgeon D, Bakowsky V, Baldwin C, Cabral DA, Clements-Baker M, Clifford A, Cohen Tervaert JW, Dehghan N, Ennis D, Famorca L, Fifi-Mah A, Girard LP, Lefebvre F, Liang P, Makhzoum JP, Massicotte-Azarniouch D, Mendel A, Milman N, Reich HN, Robinson DB, Ross C, Rumsey DG, Soowamber M, Towheed TE, Trudeau J, Twilt M, Yacyshyn E, Yardimci GK, Khalidi N, Barra L, Pagnoux C
Rheumatology (Oxford) 2023 Aug 1;62(8):2646-2651. doi: 10.1093/rheumatology/kead087. PMID: 36805625
2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.
Chung SA, Langford CA, Maz M, Abril A, Gorelik M, Guyatt G, Archer AM, Conn DL, Full KA, Grayson PC, Ibarra MF, Imundo LF, Kim S, Merkel PA, Rhee RL, Seo P, Stone JH, Sule S, Sundel RP, Vitobaldi OI, Warner A, Byram K, Dua AB, Husainat N, James KE, Kalot MA, Lin YC, Springer JM, Turgunbaev M, Villa-Forte A, Turner AS, Mustafa RA
Arthritis Rheumatol 2021 Aug;73(8):1366-1383. Epub 2021 Jul 8 doi: 10.1002/art.41773. PMID: 34235894
2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.
Chung SA, Langford CA, Maz M, Abril A, Gorelik M, Guyatt G, Archer AM, Conn DL, Full KA, Grayson PC, Ibarra MF, Imundo LF, Kim S, Merkel PA, Rhee RL, Seo P, Stone JH, Sule S, Sundel RP, Vitobaldi OI, Warner A, Byram K, Dua AB, Husainat N, James KE, Kalot MA, Lin YC, Springer JM, Turgunbaev M, Villa-Forte A, Turner AS, Mustafa RA
Arthritis Care Res (Hoboken) 2021 Aug;73(8):1088-1105. Epub 2021 Jul 8 doi: 10.1002/acr.24634. PMID: 34235880
Vasculitis therapy refines vasculitis mechanistic classification.
Torp CK, Brüner M, Keller KK, Brouwer E, Hauge EM, McGonagle D, Kragstrup TW
Autoimmun Rev 2021 Jun;20(6):102829. Epub 2021 Apr 16 doi: 10.1016/j.autrev.2021.102829. PMID: 33872767

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