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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.
 
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

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
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
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

Recent clinical studies

Etiology

Yaseen K, Mandell BF
Postgrad Med 2023 Jan;135(sup1):3-13. Epub 2022 Jul 21 doi: 10.1080/00325481.2022.2102368. PMID: 35831990
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
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
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
Jennette JC, Nachman PH
Clin J Am Soc Nephrol 2017 Oct 6;12(10):1680-1691. Epub 2017 Aug 25 doi: 10.2215/CJN.02500317. PMID: 28842398Free PMC Article

Diagnosis

Yaseen K, Mandell BF
Postgrad Med 2023 Jan;135(sup1):3-13. Epub 2022 Jul 21 doi: 10.1080/00325481.2022.2102368. PMID: 35831990
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
Sacoto G, Boukhlal S, Specks U, Flores-Suárez LF, Cornec D
Presse Med 2020 Oct;49(3):104039. Epub 2020 Jul 7 doi: 10.1016/j.lpm.2020.104039. PMID: 32650042
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
Jennette JC, Nachman PH
Clin J Am Soc Nephrol 2017 Oct 6;12(10):1680-1691. Epub 2017 Aug 25 doi: 10.2215/CJN.02500317. PMID: 28842398Free PMC Article

Therapy

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
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
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
Jennette JC, Nachman PH
Clin J Am Soc Nephrol 2017 Oct 6;12(10):1680-1691. Epub 2017 Aug 25 doi: 10.2215/CJN.02500317. PMID: 28842398Free PMC Article
Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, Kallenberg CG, St Clair EW, Turkiewicz A, Tchao NK, Webber L, Ding L, Sejismundo LP, Mieras K, Weitzenkamp D, Ikle D, Seyfert-Margolis V, Mueller M, Brunetta P, Allen NB, Fervenza FC, Geetha D, Keogh KA, Kissin EY, Monach PA, Peikert T, Stegeman C, Ytterberg SR, Specks U; RAVE-ITN Research Group
N Engl J Med 2010 Jul 15;363(3):221-32. doi: 10.1056/NEJMoa0909905. PMID: 20647199Free PMC Article

Prognosis

Sánchez Álamo B, Moi L, Bajema I, Faurschou M, Flossmann O, Hauser T, Hruskova Z, Jayne D, Luqmani R, Mahr A, Åkesson A, Westman K; EUVAS
Nephrol Dial Transplant 2023 Jun 30;38(7):1655-1665. doi: 10.1093/ndt/gfac320. PMID: 36617233
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
Sebastiani M, Manfredi A, Vacchi C, Cassone G, Faverio P, Cavazza A, Sverzellati N, Salvarani C, Luppi F
Clin Exp Rheumatol 2020 Mar-Apr;38 Suppl 124(2):221-231. Epub 2020 Apr 22 PMID: 32324122
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
Alba MA, Flores-Suárez LF, Henderson AG, Xiao H, Hu P, Nachman PH, Falk RJ, Charles Jennette J
Autoimmun Rev 2017 Jul;16(7):722-729. Epub 2017 May 4 doi: 10.1016/j.autrev.2017.05.008. PMID: 28479484Free PMC Article

Clinical prediction guides

Sánchez Álamo B, Moi L, Bajema I, Faurschou M, Flossmann O, Hauser T, Hruskova Z, Jayne D, Luqmani R, Mahr A, Åkesson A, Westman K; EUVAS
Nephrol Dial Transplant 2023 Jun 30;38(7):1655-1665. doi: 10.1093/ndt/gfac320. PMID: 36617233
Suppiah R, Robson JC, Grayson PC, Ponte C, Craven A, Khalid S, Judge A, Hutchings A, Merkel PA, Luqmani RA, Watts RA; DCVAS INVESTIGATORS
Ann Rheum Dis 2022 Mar;81(3):321-326. Epub 2022 Feb 2 doi: 10.1136/annrheumdis-2021-221796. PMID: 35110332
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
Sebastiani M, Manfredi A, Vacchi C, Cassone G, Faverio P, Cavazza A, Sverzellati N, Salvarani C, Luppi F
Clin Exp Rheumatol 2020 Mar-Apr;38 Suppl 124(2):221-231. Epub 2020 Apr 22 PMID: 32324122
Guillevin L, Pagnoux C, Karras A, Khouatra C, Aumaître O, Cohen P, Maurier F, Decaux O, Ninet J, Gobert P, Quémeneur T, Blanchard-Delaunay C, Godmer P, Puéchal X, Carron PL, Hatron PY, Limal N, Hamidou M, Ducret M, Daugas E, Papo T, Bonnotte B, Mahr A, Ravaud P, Mouthon L; French Vasculitis Study Group
N Engl J Med 2014 Nov 6;371(19):1771-80. doi: 10.1056/NEJMoa1404231. PMID: 25372085

Recent systematic reviews

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
Schirmer JH, Sanchez-Alamo B, Hellmich B, Jayne D, Monti S, Luqmani RA, Tomasson G
RMD Open 2023 Jul;9(3) doi: 10.1136/rmdopen-2023-003082. PMID: 37479496Free PMC Article
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
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
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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