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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
EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria.
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

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 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
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
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
Vasculitis.
Fink CW
Pediatr Clin North Am 1986 Oct;33(5):1203-19. doi: 10.1016/s0031-3955(16)36116-8. PMID: 2876408

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
The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort.
Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Toumelin PL; French Vasculitis Study Group (FVSG)
Medicine (Baltimore) 2011 Jan;90(1):19-27. doi: 10.1097/MD.0b013e318205a4c6. PMID: 21200183

Clinical prediction guides

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
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
Systemic vasculitis and the gut.
Hatemi I, Hatemi G, Çelik AF
Curr Opin Rheumatol 2017 Jan;29(1):33-38. doi: 10.1097/BOR.0000000000000344. PMID: 27684357
The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort.
Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Toumelin PL; French Vasculitis Study Group (FVSG)
Medicine (Baltimore) 2011 Jan;90(1):19-27. doi: 10.1097/MD.0b013e318205a4c6. PMID: 21200183
Vasculitis.
Watts RA, Scott DG
Baillieres Clin Rheumatol 1995 Aug;9(3):529-54. doi: 10.1016/s0950-3579(05)80257-2. PMID: 7497537

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
Dermatological Manifestations in Pediatric Inflammatory Bowel Disease.
Diaconescu S, Strat S, Balan GG, Anton C, Stefanescu G, Ioniuc I, Stanescu AMA
Medicina (Kaunas) 2020 Aug 23;56(9) doi: 10.3390/medicina56090425. PMID: 32842528Free PMC Article
Association of Vasculitis and Familial Mediterranean Fever.
Abbara S, Grateau G, Ducharme-Bénard S, Saadoun D, Georgin-Lavialle S
Front Immunol 2019;10:763. Epub 2019 Apr 12 doi: 10.3389/fimmu.2019.00763. PMID: 31031761Free PMC Article
Changing etiology and management patterns for spontaneous renal hemorrhage: a systematic review of contemporary series.
Ahn T, Roberts MJ, Navaratnam A, Chung E, Wood S
Int Urol Nephrol 2017 Nov;49(11):1897-1905. Epub 2017 Sep 4 doi: 10.1007/s11255-017-1694-8. PMID: 28871505
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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