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Immunoglobulin A vasculitis

MedGen UID:
48265
•Concept ID:
C0034152
•
Disease or Syndrome
Synonyms: Anaphylactoid purpura; Henoch-Schoenlein purpura; Henoch-Schonlein purpura; Vascular purpura
SNOMED CT: Henoch-Schönlein purpura (191306005); Henoch-Schonlein purpura (191306005); Henoch's purpura (191306005); Autoimmune purpura (191306005); Spring fever (191306005); Acute vascular purpura (191306005); HSP - Henoch-Schonlein purpura (191306005); Henoch-Schoenlein vasculitis (191306005); Henoch-Schoenlein purpura (191306005); Anaphylactoid purpura (191306005); Immunoglobulin A vasculitis (191306005); Purpura rheumatica (191306005)
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:0019167
Orphanet: ORPHA761

Definition

A rare, small-vessel vasculitis characterized by skin purpura, arthritis, abdominal and/or renal involvement, IgA tissue deposits (arterioles, capillaries, and venules) and circulating IgA immune complexes. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVImmunoglobulin A vasculitis

Professional guidelines

PubMed

European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis-the SHARE initiative.
Ozen S, Marks SD, Brogan P, Groot N, de Graeff N, Avcin T, Bader-Meunier B, Dolezalova P, Feldman BM, Kone-Paut I, Lahdenne P, McCann L, Pilkington C, Ravelli A, van Royen A, Uziel Y, Vastert B, Wulffraat N, Kamphuis S, Beresford MW
Rheumatology (Oxford) 2019 Sep 1;58(9):1607-1616. doi: 10.1093/rheumatology/kez041. PMID: 30879080
Annular Lesions: Diagnosis and Treatment.
Trayes KP, Savage K, Studdiford JS
Am Fam Physician 2018 Sep 1;98(5):283-291. PMID: 30216021
IgA Vasculitis: Genetics and Clinical and Therapeutic Management.
González-Gay MA, López-Mejías R, Pina T, Blanco R, Castañeda S
Curr Rheumatol Rep 2018 Apr 2;20(5):24. doi: 10.1007/s11926-018-0735-3. PMID: 29611051

Recent clinical studies

Etiology

IgA vasculitis with nephritis in children.
Dyga K, Szczepańska M
Adv Clin Exp Med 2020 Apr;29(4):513-519. doi: 10.17219/acem/112566. PMID: 32356414
European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis-the SHARE initiative.
Ozen S, Marks SD, Brogan P, Groot N, de Graeff N, Avcin T, Bader-Meunier B, Dolezalova P, Feldman BM, Kone-Paut I, Lahdenne P, McCann L, Pilkington C, Ravelli A, van Royen A, Uziel Y, Vastert B, Wulffraat N, Kamphuis S, Beresford MW
Rheumatology (Oxford) 2019 Sep 1;58(9):1607-1616. doi: 10.1093/rheumatology/kez041. PMID: 30879080
Immunoglobulin A Nephropathy and Immunoglobulin A Vasculitis.
Nicoara O, Twombley K
Pediatr Clin North Am 2019 Feb;66(1):101-110. doi: 10.1016/j.pcl.2018.08.008. PMID: 30454736
Genetics of vasculitis.
Carmona FD, Martín J, González-Gay MA
Curr Opin Rheumatol 2015 Jan;27(1):10-7. doi: 10.1097/BOR.0000000000000124. PMID: 25405820
Overview of the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides.
Jennette JC
Clin Exp Nephrol 2013 Oct;17(5):603-606. Epub 2013 Sep 27 doi: 10.1007/s10157-013-0869-6. PMID: 24072416Free PMC Article

Diagnosis

Multisystemic manifestations of IgA vasculitis.
Du L, Wang P, Liu C, Li S, Yue S, Yang Y
Clin Rheumatol 2021 Jan;40(1):43-52. Epub 2020 Jun 16 doi: 10.1007/s10067-020-05166-5. PMID: 32557258
European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis-the SHARE initiative.
Ozen S, Marks SD, Brogan P, Groot N, de Graeff N, Avcin T, Bader-Meunier B, Dolezalova P, Feldman BM, Kone-Paut I, Lahdenne P, McCann L, Pilkington C, Ravelli A, van Royen A, Uziel Y, Vastert B, Wulffraat N, Kamphuis S, Beresford MW
Rheumatology (Oxford) 2019 Sep 1;58(9):1607-1616. doi: 10.1093/rheumatology/kez041. PMID: 30879080
Annular Lesions: Diagnosis and Treatment.
Trayes KP, Savage K, Studdiford JS
Am Fam Physician 2018 Sep 1;98(5):283-291. PMID: 30216021
IgA Vasculitis: Genetics and Clinical and Therapeutic Management.
González-Gay MA, López-Mejías R, Pina T, Blanco R, Castañeda S
Curr Rheumatol Rep 2018 Apr 2;20(5):24. doi: 10.1007/s11926-018-0735-3. PMID: 29611051
Overview of the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides.
Jennette JC
Clin Exp Nephrol 2013 Oct;17(5):603-606. Epub 2013 Sep 27 doi: 10.1007/s10157-013-0869-6. PMID: 24072416Free PMC Article

Therapy

Adult-onset IgA vasculitis (Henoch-Schönlein): Update on therapy.
Maritati F, Canzian A, Fenaroli P, Vaglio A
Presse Med 2020 Oct;49(3):104035. Epub 2020 Jul 6 doi: 10.1016/j.lpm.2020.104035. PMID: 32645417
European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis-the SHARE initiative.
Ozen S, Marks SD, Brogan P, Groot N, de Graeff N, Avcin T, Bader-Meunier B, Dolezalova P, Feldman BM, Kone-Paut I, Lahdenne P, McCann L, Pilkington C, Ravelli A, van Royen A, Uziel Y, Vastert B, Wulffraat N, Kamphuis S, Beresford MW
Rheumatology (Oxford) 2019 Sep 1;58(9):1607-1616. doi: 10.1093/rheumatology/kez041. PMID: 30879080
Immunoglobulin A Nephropathy and Immunoglobulin A Vasculitis.
Nicoara O, Twombley K
Pediatr Clin North Am 2019 Feb;66(1):101-110. doi: 10.1016/j.pcl.2018.08.008. PMID: 30454736
IgA Vasculitis: Genetics and Clinical and Therapeutic Management.
González-Gay MA, López-Mejías R, Pina T, Blanco R, Castañeda S
Curr Rheumatol Rep 2018 Apr 2;20(5):24. doi: 10.1007/s11926-018-0735-3. PMID: 29611051
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

Prognosis

Multisystemic manifestations of IgA vasculitis.
Du L, Wang P, Liu C, Li S, Yue S, Yang Y
Clin Rheumatol 2021 Jan;40(1):43-52. Epub 2020 Jun 16 doi: 10.1007/s10067-020-05166-5. PMID: 32557258
Adult-onset IgA vasculitis (Henoch-Schönlein): Update on therapy.
Maritati F, Canzian A, Fenaroli P, Vaglio A
Presse Med 2020 Oct;49(3):104035. Epub 2020 Jul 6 doi: 10.1016/j.lpm.2020.104035. PMID: 32645417
IgA vasculitis with nephritis in children.
Dyga K, Szczepańska M
Adv Clin Exp Med 2020 Apr;29(4):513-519. doi: 10.17219/acem/112566. PMID: 32356414
Measuring Vasculitis with Numbers: Outcome Scores.
Batu ED, Ozen S
Curr Rheumatol Rev 2020;16(1):21-28. doi: 10.2174/1573397115666191126093927. PMID: 31804163
IgA Vasculitis: Genetics and Clinical and Therapeutic Management.
González-Gay MA, López-Mejías R, Pina T, Blanco R, Castañeda S
Curr Rheumatol Rep 2018 Apr 2;20(5):24. doi: 10.1007/s11926-018-0735-3. PMID: 29611051

Clinical prediction guides

Immunoglobulin A Vasculitis Following COVID-19: A French Multicenter Case Series.
Ramdani Y, Galempoix JM, Augusto JF, Dekmeer E, Perard L, Ferreira N, Bigot A, Magnant J, Jobard S, Diot E, Besse MC, Henrique H, Maillot F, Audemard-Verger A
J Rheumatol 2022 Dec;49(12):1390-1394. Epub 2022 Sep 1 doi: 10.3899/jrheum.220503. PMID: 36243405
IgA vasculitis with nephritis in children.
Dyga K, Szczepańska M
Adv Clin Exp Med 2020 Apr;29(4):513-519. doi: 10.17219/acem/112566. PMID: 32356414
Measuring Vasculitis with Numbers: Outcome Scores.
Batu ED, Ozen S
Curr Rheumatol Rev 2020;16(1):21-28. doi: 10.2174/1573397115666191126093927. PMID: 31804163
IgA Vasculitis: Genetics and Clinical and Therapeutic Management.
González-Gay MA, López-Mejías R, Pina T, Blanco R, Castañeda S
Curr Rheumatol Rep 2018 Apr 2;20(5):24. doi: 10.1007/s11926-018-0735-3. PMID: 29611051
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

Recent systematic reviews

Autoimmune markers and vascular immune deposits in Finkelstein-Seidlmayer vasculitis: Systematic literature review.
Bronz G, Gianini J, Passi AG, Rizzi M, Bergmann MM, Milani GP, Lava SAG, Bianchetti MG, Terziroli Beretta-Piccoli B, Vanoni F
J Autoimmun 2023 Apr;136:103002. Epub 2023 Feb 22 doi: 10.1016/j.jaut.2023.103002. PMID: 36822150
Venous sinus thrombosis in a case of immunoglobulin A vasculitis and a systemic review of literature.
Zheng Q, He Q, Huang H, Lu M
Int J Rheum Dis 2023 Mar;26(3):539-543. Epub 2022 Dec 11 doi: 10.1111/1756-185X.14529. PMID: 36502505
Coronavirus disease 2019, vaccination against coronavirus and immunoglobulin A-mediated diseases: systematic literature review.
Bronz G, Faré PB, Lava SAG, Bianchetti MG, Simonetti GD, Scoglio M, Beretta-Piccoli BT, Agostoni C, Milani GP
J Autoimmun 2022 Oct;132:102899. Epub 2022 Aug 22 doi: 10.1016/j.jaut.2022.102899. PMID: 36108473Free PMC Article
COVID-19 and New Onset IgA Vasculitis: A Systematic Review of Case Reports.
Messova A, Pivina L, Muzdubayeva Z, Sanbayev D, Urazalina Z, Adams A
J Emerg Nurs 2022 Jul;48(4):348-365. Epub 2022 May 13 doi: 10.1016/j.jen.2022.05.002. PMID: 35691763Free PMC Article
Rituximab treatment for IgA vasculitis: A systematic review.
Hernández-Rodríguez J, Carbonell C, Mirón-Canelo JA, Diez-Ruiz S, Marcos M, Chamorro AJ
Autoimmun Rev 2020 Apr;19(4):102490. Epub 2020 Feb 13 doi: 10.1016/j.autrev.2020.102490. PMID: 32062030

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