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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
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
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
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
Pediatric vasculitis.
Weiss PF
Pediatr Clin North Am 2012 Apr;59(2):407-23. Epub 2012 Apr 6 doi: 10.1016/j.pcl.2012.03.013. PMID: 22560577Free PMC Article

Diagnosis

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 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.
Micheroli R, Distler O
Rheumatology (Oxford) 2018 Apr 1;57(4):670. doi: 10.1093/rheumatology/kex327. PMID: 28968697
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
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
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.
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
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: State of the art.
Puéchal X
Joint Bone Spine 2022 Jul;89(4):105320. Epub 2021 Dec 11 doi: 10.1016/j.jbspin.2021.105320. PMID: 34902576
Clusters in Pediatric Rheumatic Diseases.
Sag E, Demir S, Ozen S
Curr Rheumatol Rep 2020 May 20;22(7):28. doi: 10.1007/s11926-020-00908-5. PMID: 32436006
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
Drug-induced vasculitis.
Cuellar ML
Curr Rheumatol Rep 2002 Feb;4(1):55-9. doi: 10.1007/s11926-002-0024-y. PMID: 11798983

Clinical prediction guides

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
Thrombosis in vasculitis.
Springer J, Villa-Forte A
Curr Opin Rheumatol 2013 Jan;25(1):19-25. doi: 10.1097/BOR.0b013e32835ad3ca. PMID: 23143223
Intravenous immunoglobulin-associated cranial pachymeningitis.
Marie I, Hervé F, Lahaxe L, Robaday S, Gerardin E, Levesque H
J Intern Med 2006 Aug;260(2):164-7. doi: 10.1111/j.1365-2796.2006.01676.x. PMID: 16882281
Vasculitic neuropathy.
Said G
Baillieres Clin Neurol 1995 Nov;4(3):489-503. PMID: 8599722
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
DADA2 diagnosed in adulthood versus childhood: A comparative study on 306 patients including a systematic literature review and 12 French cases.
Fayand A, Chasset F, Boutboul D, Queyrel V, Tieulié N, Guichard I, Dupin N, Franck N, Cohen P, Bessis D, Guenno GL, Koné-Paut I, Belot A, Bonhomme A, Ducharme-Bénard S, Grateau G, Sarrabay G, Touitou I, Boursier G, Georgin-Lavialle S
Semin Arthritis Rheum 2021 Dec;51(6):1170-1179. Epub 2021 Sep 16 doi: 10.1016/j.semarthrit.2021.09.001. PMID: 34571400
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
Mortality in systemic vasculitis: a systematic review.
Phillip R, Luqmani R
Clin Exp Rheumatol 2008 Sep-Oct;26(5 Suppl 51):S94-104. PMID: 19026150

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