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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); IgA (immunoglobulin A) vasculitis (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

Management of Primary Small-Vessel Vasculitis.
Nwannunu CE, Shah R, Limmer AL
Skin Therapy Lett 2020 May;25(3):5-8. PMID: 32510891
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

Recent clinical studies

Etiology

BAFF, APRIL and BAFFR on the pathogenesis of Immunoglobulin-A vasculitis.
Prieto-Peña D, Genre F, Remuzgo-Martínez S, Pulito-Cueto V, Atienza-Mateo B, Llorca J, Sevilla-Pérez B, Ortego-Centeno N, Lera-Gómez L, Leonardo MT, Peñalba A, Narváez J, Martín-Penagos L, Rodrigo E, Miranda-Filloy JA, Caminal-Montero L, Collado P, Pérez JS, de Argila D, Rubio E, Luque ML, Blanco-Madrigal JM, Galíndez-Agirregoikoa E, Gualillo O, Martín J, Castañeda S, Blanco R, González-Gay MA, López-Mejías R
Sci Rep 2021 Jun 1;11(1):11510. doi: 10.1038/s41598-021-91055-z. PMID: 34075170Free PMC Article
Management of Primary Small-Vessel Vasculitis.
Nwannunu CE, Shah R, Limmer AL
Skin Therapy Lett 2020 May;25(3):5-8. PMID: 32510891
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

Diagnosis

Renal Medullary Angiitis Associated with Cutaneous Leukocytoclastic Vasculitis.
Miura S, Katayama K, Joh K, Fujimoto M, Yamakawa M, Akiyama E, Nishida J, Yasutomi M, Ishikawa E, Dohi K
Intern Med 2024 Jun 15;63(12):1757-1763. Epub 2023 Nov 6 doi: 10.2169/internalmedicine.2679-23. PMID: 37926537Free PMC Article
Interleukin -17 and oxidative stress in children with immunoglobulin A vasculitis.
Kisaoglu H, Misir S, Aliyazicioglu Y, Kalyoncu M
Scand J Rheumatol 2022 Jul;51(4):309-314. Epub 2021 Jun 25 doi: 10.1080/03009742.2021.1926319. PMID: 34169806
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
Vasculitis Pathogenesis: Can We Talk About Precision Medicine?
Ozen S, Batu ED
Front Immunol 2018;9:1892. Epub 2018 Aug 14 doi: 10.3389/fimmu.2018.01892. PMID: 30154798Free PMC Article

Therapy

Renal Medullary Angiitis Associated with Cutaneous Leukocytoclastic Vasculitis.
Miura S, Katayama K, Joh K, Fujimoto M, Yamakawa M, Akiyama E, Nishida J, Yasutomi M, Ishikawa E, Dohi K
Intern Med 2024 Jun 15;63(12):1757-1763. Epub 2023 Nov 6 doi: 10.2169/internalmedicine.2679-23. PMID: 37926537Free PMC Article
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
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

Blood transthyretin for predicting immunoglobulin A vasculitis nephritis outcome in children.
Ma X, Sun Y, Wang D, Lin Y, Chang H
Int Immunopharmacol 2022 Jul;108:108765. Epub 2022 Apr 6 doi: 10.1016/j.intimp.2022.108765. PMID: 35397389
Impact of aging on phenotype and prognosis in IgA vasculitis.
Audemard-Verger A, Pillebout E, Baldolli A, Gouellec NL, Augusto JF, Jourde-Chiche N, Raffray L, Thervet E, Deroux A, Goutte J, Hummel A, Lioger B, Sanges S, Cacoub P, Amoura Z, Moulis G, Maurier F, Lavigne C, Urbanski G, Chanal J, Faguer S, Deriaz S, Feirreira-Maldent N, Diot E, Maillot F, Guillevin L, Terrier B
Rheumatology (Oxford) 2021 Sep 1;60(9):4245-4251. doi: 10.1093/rheumatology/keaa921. PMID: 33410479
Biopsy-proven Henoch-Schönlein purpura nephritis: a single center experience.
Kurt-Şükür ED, Sekar T, Tullus K
Pediatr Nephrol 2021 May;36(5):1207-1215. Epub 2020 Oct 21 doi: 10.1007/s00467-020-04809-8. PMID: 33089378Free PMC Article
Comorbidities and phenotype-genotype correlation in children with familial Mediterranean fever.
Ayaz NA, Tanatar A, Karadağ ŞG, Çakan M, Keskindemirci G, Sönmez HE
Rheumatol Int 2021 Jan;41(1):113-120. Epub 2020 Apr 28 doi: 10.1007/s00296-020-04592-7. PMID: 32347339
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

Clinical prediction guides

Blood transthyretin for predicting immunoglobulin A vasculitis nephritis outcome in children.
Ma X, Sun Y, Wang D, Lin Y, Chang H
Int Immunopharmacol 2022 Jul;108:108765. Epub 2022 Apr 6 doi: 10.1016/j.intimp.2022.108765. PMID: 35397389
Interleukin -17 and oxidative stress in children with immunoglobulin A vasculitis.
Kisaoglu H, Misir S, Aliyazicioglu Y, Kalyoncu M
Scand J Rheumatol 2022 Jul;51(4):309-314. Epub 2021 Jun 25 doi: 10.1080/03009742.2021.1926319. PMID: 34169806
Role of the IL33 and IL1RL1 pathway in the pathogenesis of Immunoglobulin A vasculitis.
Prieto-Peña D, Remuzgo-Martínez S, Genre F, Pulito-Cueto V, Atienza-Mateo B, Llorca J, Sevilla-Pérez B, Ortego-Centeno N, Marquez A, Lera-Gómez L, Leonardo MT, Peñalba A, Narváez J, Martín-Penagos L, Rodrigo E, Miranda-Filloy JA, Caminal-Montero L, Collado P, Sánchez Pérez J, de Argila D, Rubio E, León Luque M, Blanco-Madrigal JM, Galíndez-Agirregoikoa E, Gualillo O, Martín J, Castañeda S, Blanco R, González-Gay MA, López-Mejías R
Sci Rep 2021 Aug 9;11(1):16163. doi: 10.1038/s41598-021-95762-5. PMID: 34373564Free PMC Article
BAFF, APRIL and BAFFR on the pathogenesis of Immunoglobulin-A vasculitis.
Prieto-Peña D, Genre F, Remuzgo-Martínez S, Pulito-Cueto V, Atienza-Mateo B, Llorca J, Sevilla-Pérez B, Ortego-Centeno N, Lera-Gómez L, Leonardo MT, Peñalba A, Narváez J, Martín-Penagos L, Rodrigo E, Miranda-Filloy JA, Caminal-Montero L, Collado P, Pérez JS, de Argila D, Rubio E, Luque ML, Blanco-Madrigal JM, Galíndez-Agirregoikoa E, Gualillo O, Martín J, Castañeda S, Blanco R, González-Gay MA, López-Mejías R
Sci Rep 2021 Jun 1;11(1):11510. doi: 10.1038/s41598-021-91055-z. PMID: 34075170Free PMC Article
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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