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Polyarteritis nodosa

MedGen UID:
14681
Concept ID:
C0031036
Disease or Syndrome
Synonym: Küssmaul-Maier disease
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.
 
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

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
Micheletti RG, Pagnoux C
Presse Med 2020 Oct;49(3):104033. Epub 2020 Jul 6 doi: 10.1016/j.lpm.2020.104033. PMID: 32645416
Meyts I, Aksentijevich I
J Clin Immunol 2018 Jul;38(5):569-578. Epub 2018 Jun 27 doi: 10.1007/s10875-018-0525-8. PMID: 29951947Free PMC Article

Recent clinical studies

Etiology

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, 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
Saadoun D, Vautier M, Cacoub P
Circulation 2021 Jan 19;143(3):267-282. doi: 10.1161/CIRCULATIONAHA.120.046657. PMID: 33464968
Meyts I, Aksentijevich I
J Clin Immunol 2018 Jul;38(5):569-578. Epub 2018 Jun 27 doi: 10.1007/s10875-018-0525-8. PMID: 29951947Free PMC Article
Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, Buoncompagni A, Lazar C, Bilge I, Uziel Y, Rigante D, Cantarini L, Hilario MO, Silva CA, Alegria M, Norambuena X, Belot A, Berkun Y, Estrella AI, Olivieri AN, Alpigiani MG, Rumba I, Sztajnbok F, Tambic-Bukovac L, Breda L, Al-Mayouf S, Mihaylova D, Chasnyk V, Sengler C, Klein-Gitelman M, Djeddi D, Nuno L, Pruunsild C, Brunner J, Kondi A, Pagava K, Pederzoli S, Martini A, Ruperto N; Paediatric Rheumatology International Trials Organisation (PRINTO)
Ann Rheum Dis 2010 May;69(5):798-806. doi: 10.1136/ard.2009.116657. PMID: 20413568

Diagnosis

Bilginer Y, Ozen S
Curr Opin Pediatr 2022 Apr 1;34(2):229-233. doi: 10.1097/MOP.0000000000001106. PMID: 35081554
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
de Boysson H, Guillevin L
Neurol Clin 2019 May;37(2):345-357. Epub 2019 Mar 16 doi: 10.1016/j.ncl.2019.01.007. PMID: 30952413
Karadag O, Jayne DJ
Clin Exp Rheumatol 2018 Mar-Apr;36 Suppl 111(2):135-142. Epub 2018 Feb 20 PMID: 29465365
Hernández-Rodríguez J, Alba MA, Prieto-González S, Cid MC
J Autoimmun 2014 Feb-Mar;48-49:84-9. Epub 2014 Jan 28 doi: 10.1016/j.jaut.2014.01.029. PMID: 24485157

Therapy

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
Saadoun D, Vautier M, Cacoub P
Circulation 2021 Jan 19;143(3):267-282. doi: 10.1161/CIRCULATIONAHA.120.046657. PMID: 33464968
Micheletti RG, Pagnoux C
Presse Med 2020 Oct;49(3):104033. Epub 2020 Jul 6 doi: 10.1016/j.lpm.2020.104033. PMID: 32645416
Forbess L, Bannykh S
Rheum Dis Clin North Am 2015;41(1):33-46, vii. doi: 10.1016/j.rdc.2014.09.005. PMID: 25399938
Hernández-Rodríguez J, Alba MA, Prieto-González S, Cid MC
J Autoimmun 2014 Feb-Mar;48-49:84-9. Epub 2014 Jan 28 doi: 10.1016/j.jaut.2014.01.029. PMID: 24485157

Prognosis

Springer JM, Byram K
Postgrad Med 2023 Jan;135(sup1):61-68. Epub 2022 Jun 22 doi: 10.1080/00325481.2022.2088940. PMID: 35709399
Bilginer Y, Ozen S
Curr Opin Pediatr 2022 Apr 1;34(2):229-233. doi: 10.1097/MOP.0000000000001106. PMID: 35081554
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
de Boysson H, Guillevin L
Neurol Clin 2019 May;37(2):345-357. Epub 2019 Mar 16 doi: 10.1016/j.ncl.2019.01.007. PMID: 30952413
Karadag O, Jayne DJ
Clin Exp Rheumatol 2018 Mar-Apr;36 Suppl 111(2):135-142. Epub 2018 Feb 20 PMID: 29465365

Clinical prediction guides

Siligato R, Gembillo G, Calabrese V, Conti G, Santoro D
Medicina (Kaunas) 2021 Oct 1;57(10) doi: 10.3390/medicina57101049. PMID: 34684086Free PMC Article
Makhzoum JP, Grayson PC, Ponte C, Robson J, Suppiah R, Watts RA, Luqmani R, Merkel PA, Pagnoux C; DCVAS Collaborators
Rheumatology (Oxford) 2021 Dec 24;61(1):319-330. doi: 10.1093/rheumatology/keab325. PMID: 33788906Free PMC Article
Terrier B, Darbon R, Durel CA, Hachulla E, Karras A, Maillard H, Papo T, Puechal X, Pugnet G, Quemeneur T, Samson M, Taille C, Guillevin L; Collaborators
Orphanet J Rare Dis 2020 Dec 29;15(Suppl 2):351. doi: 10.1186/s13023-020-01621-3. PMID: 33372616Free PMC Article
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
Dillon MJ, Eleftheriou D, Brogan PA
Pediatr Nephrol 2010 Sep;25(9):1641-52. Epub 2009 Nov 28 doi: 10.1007/s00467-009-1336-1. PMID: 19946711Free PMC Article

Recent systematic reviews

Conticini E, d'Alessandro M, Al Khayyat SG, D'Alessandro R, D'Ignazio E, Pata AP, Vallifuoco G, Falsetti P, Baldi C, Bardelli M, Gentileschi S, Fabiani C, Mazzei MA, Guarnieri A, Bargagli E, Cantarini L, Frediani B
Autoimmun Rev 2022 Mar;21(3):103029. Epub 2021 Dec 29 doi: 10.1016/j.autrev.2021.103029. PMID: 34971804
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
Papachristodoulou E, Kakoullis L, Tiniakou E, Parperis K
Rheumatology (Oxford) 2021 Sep 1;60(9):4039-4047. doi: 10.1093/rheumatology/keab402. PMID: 33944902
Kasap Cuceoglu M, Sener S, Batu ED, Kaya Akca U, Demir S, Sag E, Atalay E, Balık Z, Basaran O, Bilginer Y, Ozen S
Semin Arthritis Rheum 2021 Jun;51(3):559-564. Epub 2021 Apr 19 doi: 10.1016/j.semarthrit.2021.04.009. PMID: 33901990
Chan M, Luqmani R
Expert Opin Pharmacother 2009 Jun;10(8):1273-89. doi: 10.1517/14656560902946401. PMID: 19445559

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