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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.
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
Management of cutaneous vasculitis.
Micheletti RG, Pagnoux C
Presse Med 2020 Oct;49(3):104033. Epub 2020 Jul 6 doi: 10.1016/j.lpm.2020.104033. PMID: 32645416

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
Deficiency of Adenosine Deaminase 2 (DADA2): Updates on the Phenotype, Genetics, Pathogenesis, and Treatment.
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
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.
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.
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
Diagnosis and classification of polyarteritis nodosa.
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

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
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
Management of cutaneous vasculitis.
Micheletti RG, Pagnoux C
Presse Med 2020 Oct;49(3):104033. Epub 2020 Jul 6 doi: 10.1016/j.lpm.2020.104033. PMID: 32645416
Polyarteritis nodosa.
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
Diagnosis and classification of polyarteritis nodosa.
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

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 Neurologic Manifestations.
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
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

Clinical prediction guides

Clinical Characteristics and Outcomes of Polyarteritis Nodosa: An International Study.
Karadag O, Bolek EC, Ayan G, Mohammad AJ, Grayson PC, Pagnoux C, Martín-Nares E, Monti S, Abe Y, Alberici F, Alibaz-Oner F, Cuthbertson D, Dagna L, Direskeneli H, Khalidi NA, Koening C, Langford CA, McAlear CA, Monach PA, Moroni L, Padoan R, Seo P, Warrington KJ, Hocevar A, Hinojosa-Azaola A, Furuta S, Emmi G, Ozen S, Jayne D, Merkel PA; GLOBAL‐PAN Collaborators
Arthritis Rheumatol 2024 Jul;76(7):1120-1129. Epub 2024 Feb 22 doi: 10.1002/art.42817. PMID: 38343337Free PMC Article
Diagnosis and management of ADA2 deficient polyarteritis nodosa.
Human A, Pagnoux C
Int J Rheum Dis 2019 Jan;22 Suppl 1:69-77. Epub 2018 Apr 6 doi: 10.1111/1756-185X.13283. PMID: 29624883
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
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
Medium-size-vessel vasculitis.
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

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
Therapeutic options for cutaneous polyarteritis nodosa: a systematic review.
Papachristodoulou E, Kakoullis L, Tiniakou E, Parperis K
Rheumatology (Oxford) 2021 Sep 1;60(9):4039-4047. doi: 10.1093/rheumatology/keab402. PMID: 33944902
Systematic review of childhood-onset polyarteritis nodosa and DADA2.
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
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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