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Polyarteritis nodosa

MedGen UID:
14681
•Concept ID:
C0031036
•
Disease or Syndrome
Synonyms: Küssmaul-Maier disease; Periarteritis nodosa
SNOMED CT: Periarteritis nodosa (155441006); Polyarteritis nodosa (155441006); PAN - Polyarteritis nodosa (155441006)
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:0019170
Orphanet: ORPHA767

Definition

A rare, clinically heterogeneous, systemic disease characterized by necrotizing inflammatory lesions affecting medium-sized blood vessels. It most commonly affects skin, joints, peripheral nerves and the gastrointestinal tract. [from ORDO]

Professional guidelines

PubMed

VEXAS syndrome: Clinical manifestations, diagnosis, and treatment.
Loeza-Uribe MP, Hinojosa-Azaola A, Sánchez-Hernández BE, Crispín JC, Apodaca-Chávez E, Ferrada MA, Martín-Nares E
Reumatol Clin (Engl Ed) 2024 Jan;20(1):47-56. Epub 2023 Dec 29 doi: 10.1016/j.reumae.2023.12.004. PMID: 38160120
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 Polyarteritis Nodosa.
Chung SA, Gorelik M, Langford CA, Maz M, Abril A, 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 M, Lin YC, Springer JM, Turgunbaev M, Villa-Forte A, Turner AS, Mustafa RA
Arthritis Care Res (Hoboken) 2021 Aug;73(8):1061-1070. Epub 2021 Jul 8 doi: 10.1002/acr.24633. PMID: 34235889

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
2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Polyarteritis Nodosa.
Chung SA, Gorelik M, Langford CA, Maz M, Abril A, 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 M, Lin YC, Springer JM, Turgunbaev M, Villa-Forte A, Turner AS, Mustafa RA
Arthritis Care Res (Hoboken) 2021 Aug;73(8):1061-1070. Epub 2021 Jul 8 doi: 10.1002/acr.24633. PMID: 34235889
Medium- and Large-Vessel Vasculitis.
Saadoun D, Vautier M, Cacoub P
Circulation 2021 Jan 19;143(3):267-282. doi: 10.1161/CIRCULATIONAHA.120.046657. PMID: 33464968
Vasculitides in HIV Infection.
Vega LE, Espinoza LR
Curr Rheumatol Rep 2020 Aug 26;22(10):60. doi: 10.1007/s11926-020-00945-0. PMID: 32845496Free PMC Article
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

Polyarteritis Nodosa: Old Disease, New Etiologies.
Wolff L, Horisberger A, Moi L, Karampetsou MP, Comte D
Int J Mol Sci 2023 Nov 23;24(23) doi: 10.3390/ijms242316668. PMID: 38068989Free 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
Polyarteritis nodosa.
Bilginer Y, Ozen S
Curr Opin Pediatr 2022 Apr 1;34(2):229-233. doi: 10.1097/MOP.0000000000001106. PMID: 35081554
Polyarteritis nodosa.
Micheroli R, Distler O
Rheumatology (Oxford) 2018 Apr 1;57(4):670. doi: 10.1093/rheumatology/kex327. PMID: 28968697
Polyarteritis nodosa.
Howard T, Ahmad K, Swanson JA, Misra S
Tech Vasc Interv Radiol 2014 Dec;17(4):247-51. Epub 2014 Nov 13 doi: 10.1053/j.tvir.2014.11.005. PMID: 25770638Free PMC Article

Therapy

Polyarteritis Nodosa.
Aoki K, Kojima M
N Engl J Med 2024 May 9;390(18):1711. doi: 10.1056/NEJMicm2309365. PMID: 38718361
Deficiency of Adenosine Deaminase 2: Clinical Manifestations, Diagnosis, and Treatment.
Grim A, Veiga KR, Saad N
Rheum Dis Clin North Am 2023 Nov;49(4):773-787. Epub 2023 Aug 1 doi: 10.1016/j.rdc.2023.06.004. PMID: 37821195
Medium- and Large-Vessel Vasculitis.
Saadoun D, Vautier M, Cacoub P
Circulation 2021 Jan 19;143(3):267-282. doi: 10.1161/CIRCULATIONAHA.120.046657. PMID: 33464968
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
Polyarteritis nodosa.
Pettigrew HD, Teuber SS, Gershwin ME
Compr Ther 2007 Fall;33(3):144-9. doi: 10.1007/s12019-007-0012-2. PMID: 18004029

Prognosis

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
Polyarteritis nodosa.
Bilginer Y, Ozen S
Curr Opin Pediatr 2022 Apr 1;34(2):229-233. doi: 10.1097/MOP.0000000000001106. PMID: 35081554
Clinical Approach to Diagnosis and Therapy of Polyarteritis Nodosa.
Hočevar A, Tomšič M, Perdan Pirkmajer K
Curr Rheumatol Rep 2021 Feb 10;23(3):14. doi: 10.1007/s11926-021-00983-2. PMID: 33569653
Polyarteritis nodosa revisited: a review of historical approaches, subphenotypes and a research agenda.
Karadag O, Jayne DJ
Clin Exp Rheumatol 2018 Mar-Apr;36 Suppl 111(2):135-142. Epub 2018 Feb 20 PMID: 29465365
Polyarteritis nodosa.
Dollery CT
Br Med J 1969 Mar 29;1(5647):827-9. doi: 10.1136/bmj.1.5647.827. PMID: 4388140Free PMC Article

Clinical prediction guides

New-onset autoimmune disease after COVID-19.
Hileman CO, Malakooti SK, Patil N, Singer NG, McComsey GA
Front Immunol 2024;15:1337406. Epub 2024 Feb 8 doi: 10.3389/fimmu.2024.1337406. PMID: 38390319Free PMC Article
Biomarkers in vasculitis.
Hatemi G, Esatoglu SN, Yazici Y
Curr Opin Rheumatol 2018 Jan;30(1):30-35. doi: 10.1097/BOR.0000000000000447. PMID: 28937415
Pictorial essay: Uncommon causes of coronary artery encasement.
Yong YR, Lath N, Cheah FK, Ng YL
J Cardiovasc Comput Tomogr 2016 Sep-Oct;10(5):424-9. Epub 2016 Jul 6 doi: 10.1016/j.jcct.2016.07.004. PMID: 27443748
Early-onset stroke and vasculopathy associated with mutations in ADA2.
Zhou Q, Yang D, Ombrello AK, Zavialov AV, Toro C, Zavialov AV, Stone DL, Chae JJ, Rosenzweig SD, Bishop K, Barron KS, Kuehn HS, Hoffmann P, Negro A, Tsai WL, Cowen EW, Pei W, Milner JD, Silvin C, Heller T, Chin DT, Patronas NJ, Barber JS, Lee CC, Wood GM, Ling A, Kelly SJ, Kleiner DE, Mullikin JC, Ganson NJ, Kong HH, Hambleton S, Candotti F, Quezado MM, Calvo KR, Alao H, Barham BK, Jones A, Meschia JF, Worrall BB, Kasner SE, Rich SS, Goldbach-Mansky R, Abinun M, Chalom E, Gotte AC, Punaro M, Pascual V, Verbsky JW, Torgerson TR, Singer NG, Gershon TR, Ozen S, Karadag O, Fleisher TA, Remmers EF, Burgess SM, Moir SL, Gadina M, Sood R, Hershfield MS, Boehm M, Kastner DL, Aksentijevich I
N Engl J Med 2014 Mar 6;370(10):911-20. Epub 2014 Feb 19 doi: 10.1056/NEJMoa1307361. PMID: 24552284Free PMC Article
Vasculitis.
Fink CW
Pediatr Clin North Am 1986 Oct;33(5):1203-19. doi: 10.1016/s0031-3955(16)36116-8. PMID: 2876408

Recent systematic reviews

Post COVID-19 vaccination medium vessel vasculitis: a systematic review of case reports.
Sanker V, Mylavarapu M, Gupta P, Syed N, Shah M, Dondapati VVK
Infection 2024 Aug;52(4):1207-1213. Epub 2024 Mar 14 doi: 10.1007/s15010-024-02217-w. PMID: 38483787
Clinical characteristics, disease trajectories and management of vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome: a systematic review.
Kouranloo K, Dey M, Almutawa J, Myall N, Nune A
Rheumatol Int 2024 Jul;44(7):1219-1232. Epub 2023 Dec 21 doi: 10.1007/s00296-023-05513-0. PMID: 38129348
2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Polyarteritis Nodosa.
Chung SA, Gorelik M, Langford CA, Maz M, Abril A, 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 M, Lin YC, Springer JM, Turgunbaev M, Villa-Forte A, Turner AS, Mustafa RA
Arthritis Care Res (Hoboken) 2021 Aug;73(8):1061-1070. Epub 2021 Jul 8 doi: 10.1002/acr.24633. PMID: 34235889
Biologic therapy in ANCA-negative vasculitis.
Loricera J, Blanco R, Hernández JL, Pina T, González-Vela MC, González-Gay MA
Int Immunopharmacol 2015 Aug;27(2):213-9. Epub 2015 Mar 28 doi: 10.1016/j.intimp.2015.03.020. PMID: 25828585
Pharmacotherapy of vasculitis.
Chan M, Luqmani R
Expert Opin Pharmacother 2009 Jun;10(8):1273-89. doi: 10.1517/14656560902946401. PMID: 19445559

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