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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

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
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
Epidemiology and management of interstitial lung disease in ANCA-associated vasculitis.
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

Recent clinical studies

Etiology

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
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
ANCA Glomerulonephritis and Vasculitis.
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
ANCA-associated vasculitis.
Yates M, Watts R
Clin Med (Lond) 2017 Feb;17(1):60-64. doi: 10.7861/clinmedicine.17-1-60. PMID: 28148583Free PMC Article

Diagnosis

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
Lung involvement in ANCA-associated vasculitis.
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
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
ANCA Glomerulonephritis and Vasculitis.
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
ANCA-associated vasculitis.
Yates M, Watts R
Clin Med (Lond) 2017 Feb;17(1):60-64. doi: 10.7861/clinmedicine.17-1-60. PMID: 28148583Free PMC Article

Therapy

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
ANCA associated vasculitis.
Salvador F
Eur J Intern Med 2020 Apr;74:18-28. Epub 2020 Jan 29 doi: 10.1016/j.ejim.2020.01.011. PMID: 32005600
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
ANCA Glomerulonephritis and Vasculitis.
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
Rituximab versus cyclophosphamide for ANCA-associated vasculitis.
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

Novel Therapies for ANCA-associated Vasculitis.
Monti S, Brandolino F, Milanesi A, Xoxi B, Delvino P, Montecucco C
Curr Rheumatol Rep 2021 Apr 28;23(6):38. doi: 10.1007/s11926-021-01010-0. PMID: 33909172
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
Epidemiology and management of interstitial lung disease in ANCA-associated vasculitis.
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
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
Interstital lung disease in ANCA vasculitis.
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

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
Epidemiology and management of interstitial lung disease in ANCA-associated vasculitis.
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
Pathogenesis of ANCA-Associated Pulmonary Vasculitis.
Alba MA, Jennette JC, Falk RJ
Semin Respir Crit Care Med 2018 Aug;39(4):413-424. Epub 2018 Nov 7 doi: 10.1055/s-0038-1673386. PMID: 30404109Free PMC Article
ANCA-Associated Vasculitis Pathogenesis: A Commentary.
Gapud EJ, Seo P, Antiochos B
Curr Rheumatol Rep 2017 Apr;19(4):15. doi: 10.1007/s11926-017-0641-0. PMID: 28361331
Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis.
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

Performance of MPO-ANCA and PR3-ANCA immunoassays for the stratification of specific ANCA-associated vasculitis: A systematic review and meta-analysis.
Walker BS, Peterson LK, Koening C, White SK, Schmidt RL, Tebo AE
Autoimmun Rev 2022 Jun;21(6):103100. Epub 2022 Apr 19 doi: 10.1016/j.autrev.2022.103100. PMID: 35452854
Induction and maintenance of remission with mycophenolate mofetil in ANCA-associated vasculitis: a systematic review and meta-analysis.
Berti A, Alsawas M, Jawaid T, Prokop LJ, Lee JM, Jeong GH, Quintana LF, Moiseev S, Vaglio A, Tesar V, Geetha D, Shin JIL, Kronbichler A
Nephrol Dial Transplant 2022 Oct 19;37(11):2190-2200. doi: 10.1093/ndt/gfab357. PMID: 34910216
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
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
Efficacy of plasma exchange for antineutrophil cytoplasmic antibody-associated systemic vasculitis: a systematic review and meta-analysis.
Yamada Y, Harada M, Hara Y, Iwabuchi R, Hashimoto K, Yamamoto S, Kamijo Y
Arthritis Res Ther 2021 Jan 14;23(1):28. doi: 10.1186/s13075-021-02415-z. PMID: 33446268Free PMC Article

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