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Medium vessel vasculitis

MedGen UID:
1054158
•Concept ID:
CN377488
•
Finding
Synonym: Medium-size-vessel vasculitis
 
HPO: HP:6000658

Definition

A type of vasculitis (inflammation of blood vessel walls) that affects medium-sized blood vessels. By common usage, vasculitis predominantly refers to arterial disease. Medium-sized vasculitis is observed in diseases including polyarteritis nodosa, Kawasaki disease, and cutaneous polyarteritis nodosa. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMedium vessel vasculitis

Professional guidelines

PubMed

2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis.
Robson JC, Grayson PC, Ponte C, Suppiah R, Craven A, Judge A, Khalid S, Hutchings A, Watts RA, Merkel PA, Luqmani RA; DCVAS Investigators
Ann Rheum Dis 2022 Mar;81(3):315-320. Epub 2022 Feb 2 doi: 10.1136/annrheumdis-2021-221795. PMID: 35110333
2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis.
Suppiah R, Robson JC, Grayson PC, Ponte C, Craven A, Khalid S, Judge A, Hutchings A, Merkel PA, Luqmani RA, Watts RA; DCVAS INVESTIGATORS
Ann Rheum Dis 2022 Mar;81(3):321-326. Epub 2022 Feb 2 doi: 10.1136/annrheumdis-2021-221796. PMID: 35110332
EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis.
Yates M, Watts RA, Bajema IM, Cid MC, Crestani B, Hauser T, Hellmich B, Holle JU, Laudien M, Little MA, Luqmani RA, Mahr A, Merkel PA, Mills J, Mooney J, Segelmark M, Tesar V, Westman K, Vaglio A, Yalçındağ N, Jayne DR, Mukhtyar C
Ann Rheum Dis 2016 Sep;75(9):1583-94. Epub 2016 Jun 23 doi: 10.1136/annrheumdis-2016-209133. PMID: 27338776

Recent clinical studies

Etiology

2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis.
Grayson PC, Ponte C, Suppiah R, Robson JC, Craven A, Judge A, Khalid S, Hutchings A, Luqmani RA, Watts RA, Merkel PA; DCVAS Study Group
Ann Rheum Dis 2022 Mar;81(3):309-314. Epub 2022 Feb 2 doi: 10.1136/annrheumdis-2021-221794. PMID: 35110334
2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis.
Robson JC, Grayson PC, Ponte C, Suppiah R, Craven A, Judge A, Khalid S, Hutchings A, Watts RA, Merkel PA, Luqmani RA; DCVAS Investigators
Ann Rheum Dis 2022 Mar;81(3):315-320. Epub 2022 Feb 2 doi: 10.1136/annrheumdis-2021-221795. PMID: 35110333
Kawasaki disease in siblings in close temporal proximity to each other-what are the implications?
Banday AZ, Bhattacharya D, Pandiarajan V, Singh S
Clin Rheumatol 2021 Mar;40(3):849-855. Epub 2020 Aug 10 doi: 10.1007/s10067-020-05328-5. PMID: 32776314Free PMC Article
Diagnosis of Kawasaki disease.
Singh S, Jindal AK, Pilania RK
Int J Rheum Dis 2018 Jan;21(1):36-44. Epub 2017 Nov 13 doi: 10.1111/1756-185X.13224. PMID: 29131549Free PMC Article
Role of imaging studies in Kawasaki disease.
Tsuda E, Singhal M
Int J Rheum Dis 2018 Jan;21(1):56-63. Epub 2017 Nov 8 doi: 10.1111/1756-185X.13210. PMID: 29115035

Diagnosis

Vasculitis Mimics and Other Related Conditions.
Springer JM, Villa-Forte A
Rheum Dis Clin North Am 2023 Aug;49(3):617-631. Epub 2023 Apr 15 doi: 10.1016/j.rdc.2023.03.008. PMID: 37331736
2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis.
Grayson PC, Ponte C, Suppiah R, Robson JC, Craven A, Judge A, Khalid S, Hutchings A, Luqmani RA, Watts RA, Merkel PA; DCVAS Study Group
Ann Rheum Dis 2022 Mar;81(3):309-314. Epub 2022 Feb 2 doi: 10.1136/annrheumdis-2021-221794. PMID: 35110334
Diagnosis of Kawasaki disease.
Singh S, Jindal AK, Pilania RK
Int J Rheum Dis 2018 Jan;21(1):36-44. Epub 2017 Nov 13 doi: 10.1111/1756-185X.13224. PMID: 29131549Free PMC Article
Role of imaging studies in Kawasaki disease.
Tsuda E, Singhal M
Int J Rheum Dis 2018 Jan;21(1):56-63. Epub 2017 Nov 8 doi: 10.1111/1756-185X.13210. PMID: 29115035
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

Therapy

Kawasaki like illness due to COVID-19: a review of the literature.
Fang Y, Aravamudan VM, Sridharan GK, Mehta KK, Sekhar R, Senguttuvan NB, Venkatachalam I, Abid MB
J Infect Dev Ctries 2021 May 31;15(5):630-638. doi: 10.3855/jidc.14185. PMID: 34106885
Adult-onset Kawasaki disease: A great masquerader.
Datta AK, Debnath P, Chaudhuri J, Mukherjee A
BMJ Case Rep 2021 Mar 16;14(3) doi: 10.1136/bcr-2020-239746. PMID: 33727292Free PMC Article
EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis.
Yates M, Watts RA, Bajema IM, Cid MC, Crestani B, Hauser T, Hellmich B, Holle JU, Laudien M, Little MA, Luqmani RA, Mahr A, Merkel PA, Mills J, Mooney J, Segelmark M, Tesar V, Westman K, Vaglio A, Yalçındağ N, Jayne DR, Mukhtyar C
Ann Rheum Dis 2016 Sep;75(9):1583-94. Epub 2016 Jun 23 doi: 10.1136/annrheumdis-2016-209133. PMID: 27338776
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

Prognosis

Markers of Endothelial Dysfunction in Kawasaki Disease: An Update.
Kumrah R, Goyal T, Rawat A, Singh S
Clin Rev Allergy Immunol 2024 Feb;66(1):99-111. Epub 2024 Mar 11 doi: 10.1007/s12016-024-08985-5. PMID: 38462555
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
Diagnosis of Kawasaki disease.
Singh S, Jindal AK, Pilania RK
Int J Rheum Dis 2018 Jan;21(1):36-44. Epub 2017 Nov 13 doi: 10.1111/1756-185X.13224. PMID: 29131549Free PMC Article
Role of imaging studies in Kawasaki disease.
Tsuda E, Singhal M
Int J Rheum Dis 2018 Jan;21(1):56-63. Epub 2017 Nov 8 doi: 10.1111/1756-185X.13210. PMID: 29115035
EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis.
Yates M, Watts RA, Bajema IM, Cid MC, Crestani B, Hauser T, Hellmich B, Holle JU, Laudien M, Little MA, Luqmani RA, Mahr A, Merkel PA, Mills J, Mooney J, Segelmark M, Tesar V, Westman K, Vaglio A, Yalçındağ N, Jayne DR, Mukhtyar C
Ann Rheum Dis 2016 Sep;75(9):1583-94. Epub 2016 Jun 23 doi: 10.1136/annrheumdis-2016-209133. PMID: 27338776

Clinical prediction guides

Markers of Endothelial Dysfunction in Kawasaki Disease: An Update.
Kumrah R, Goyal T, Rawat A, Singh S
Clin Rev Allergy Immunol 2024 Feb;66(1):99-111. Epub 2024 Mar 11 doi: 10.1007/s12016-024-08985-5. PMID: 38462555
2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis.
Grayson PC, Ponte C, Suppiah R, Robson JC, Craven A, Judge A, Khalid S, Hutchings A, Luqmani RA, Watts RA, Merkel PA; DCVAS Study Group
Ann Rheum Dis 2022 Mar;81(3):309-314. Epub 2022 Feb 2 doi: 10.1136/annrheumdis-2021-221794. PMID: 35110334
2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis.
Robson JC, Grayson PC, Ponte C, Suppiah R, Craven A, Judge A, Khalid S, Hutchings A, Watts RA, Merkel PA, Luqmani RA; DCVAS Investigators
Ann Rheum Dis 2022 Mar;81(3):315-320. Epub 2022 Feb 2 doi: 10.1136/annrheumdis-2021-221795. PMID: 35110333
Diagnosis of Kawasaki disease.
Singh S, Jindal AK, Pilania RK
Int J Rheum Dis 2018 Jan;21(1):36-44. Epub 2017 Nov 13 doi: 10.1111/1756-185X.13224. PMID: 29131549Free PMC Article
Role of imaging studies in Kawasaki disease.
Tsuda E, Singhal M
Int J Rheum Dis 2018 Jan;21(1):56-63. Epub 2017 Nov 8 doi: 10.1111/1756-185X.13210. PMID: 29115035

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
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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