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Primary polyarteritis nodosa

MedGen UID:
891641
•Concept ID:
CN237623
•
Disease or Syndrome
Synonyms: Primary PAN; primary PAN; Primary periarteritis nodosa; primary periarteritis nodosa; primary polyarteritis nodosa
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:0018593
Orphanet: ORPHA439737

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary polyarteritis nodosa

Professional guidelines

PubMed

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
Treatment of systemic necrotizing vasculitides: recent advances and important clinical considerations.
Pagnoux C, Mendel A
Expert Rev Clin Immunol 2019 Sep;15(9):939-949. Epub 2019 Aug 26 doi: 10.1080/1744666X.2019.1656527. PMID: 31414912
Classification and classification criteria for vasculitis: achievements, limitations and prospects.
Mahr A, de Menthon M
Curr Opin Rheumatol 2015 Jan;27(1):1-9. doi: 10.1097/BOR.0000000000000134. PMID: 25415531

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
Extrahepatic autoimmune diseases in primary biliary cholangitis: Prevalence and significance for clinical presentation and disease outcome.
Efe C, Torgutalp M, Henriksson I, Alalkim F, Lytvyak E, Trivedi H, Eren F, Fischer J, Chayanupatkul M, Coppo C, Purnak T, Muratori L, Werner M, Muratori P, Rorsman F, Onnerhag K, Nilsson E, Heurgué-Berlot A, Demir N, Semela D, Kıyıcı M, Schiano TD, Montano-Loza AJ, Berg T, Ozaslan E, Yoshida EM, Bonder A, Marschall HU, Beretta-Piccoli BT, Wahlin S
J Gastroenterol Hepatol 2021 Apr;36(4):936-942. Epub 2020 Aug 23 doi: 10.1111/jgh.15214. PMID: 32790935
The heart in vasculitis.
Miloslavsky E, Unizony S
Rheum Dis Clin North Am 2014 Feb;40(1):11-26. doi: 10.1016/j.rdc.2013.10.006. PMID: 24268007
CNS vasculitis.
Kelley RE
Front Biosci 2004 Jan 1;9:946-55. doi: 10.2741/1259. PMID: 14766421
Vasculitis.
Watts RA, Scott DG
Baillieres Clin Rheumatol 1995 Aug;9(3):529-54. doi: 10.1016/s0950-3579(05)80257-2. PMID: 7497537

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
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
Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever.
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
Pulmonary involvement in primary systemic vasculitides.
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
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

Therapy

Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever.
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
Nonatherosclerotic Abdominal Vasculopathies.
Skeik N, Hyde JR, Olson SL, Thaler CM, Abuatiyeh W, Ahmed AK, Lyon DR, Witt DR, Garberich R, Sullivan T
Ann Vasc Surg 2019 Oct;60:128-146. Epub 2019 Jun 12 doi: 10.1016/j.avsg.2019.04.004. PMID: 31200053
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
Vasculitis in children.
Eleftheriou D, Brogan PA
Best Pract Res Clin Rheumatol 2009 Jun;23(3):309-23. doi: 10.1016/j.berh.2009.02.001. PMID: 19508940Free PMC Article
Vasculitic neuropathies.
Burns TM, Schaublin GA, Dyck PJ
Neurol Clin 2007 Feb;25(1):89-113. doi: 10.1016/j.ncl.2006.11.002. PMID: 17324722

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
Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever.
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
Extrahepatic autoimmune diseases in primary biliary cholangitis: Prevalence and significance for clinical presentation and disease outcome.
Efe C, Torgutalp M, Henriksson I, Alalkim F, Lytvyak E, Trivedi H, Eren F, Fischer J, Chayanupatkul M, Coppo C, Purnak T, Muratori L, Werner M, Muratori P, Rorsman F, Onnerhag K, Nilsson E, Heurgué-Berlot A, Demir N, Semela D, Kıyıcı M, Schiano TD, Montano-Loza AJ, Berg T, Ozaslan E, Yoshida EM, Bonder A, Marschall HU, Beretta-Piccoli BT, Wahlin S
J Gastroenterol Hepatol 2021 Apr;36(4):936-942. Epub 2020 Aug 23 doi: 10.1111/jgh.15214. PMID: 32790935
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.
Bonsib SM
Semin Diagn Pathol 2001 Feb;18(1):14-23. PMID: 11296989

Clinical prediction guides

Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever.
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
Pulmonary involvement in primary systemic vasculitides.
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
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
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
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

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
Haemophagocytic syndrome in rheumatic patients. A systematic review.
Atteritano M, David A, Bagnato G, Beninati C, Frisina A, Iaria C, Bagnato G, Cascio A
Eur Rev Med Pharmacol Sci 2012 Oct;16(10):1414-24. PMID: 23104659

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