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Current Rheumatology Reports

Erschienen in:

01.07.2021 | Vasculitis (C Dejaco and C Duftner, Section Editors)

IgA Vasculitis in Adults: a Rare yet Challenging Disease

verfasst von: Kinanah Yaseen, Leal C Herlitz, Alexandra Villa-Forte

Erschienen in: Current Rheumatology Reports | Ausgabe 7/2021

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Abstract

Purpose of Review

IgA vasculitis (IgAV) is a rare and poorly understood systemic vasculitis in adults. Its diagnosis and treatment remain a challenge. Herein, we review the clinical manifestations, diagnosis, management, and prognosis of IgAV in adults.

Recent Findings

The clinical course of IgAV in adults appears to be different from pediatric IgAV, especially due to its higher risk of evolving into end-stage renal disease. Rising awareness and interest in adult-onset IgA vasculitis has resulted in recent increasing number of publications on different treatment experiences. However, there is still controversy over the role of glucocorticoid (GC) and different immunosuppressive therapies such as cyclophosphamide, rituximab, and mychophenolate mofetil for more severe IgAV. Data regarding potential benefits of targeting the mucosal immune system, toll-like receptors, complements, and tyrosine kinase inhibitors in the treatment of IgA nephropathy are emerging.

Summary

High quality evidence or guidelines in the treatment of severe IgAV are lacking and there is still a great need for controlled trials.

Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR. Incidence of Henoch-Schonlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet. 2002;360(9341):1197–202. https://doi.org/10.1016/S0140-6736(02)11279-7.CrossRefPubMed

Watts RA, Lane S, Scott DGI. What is known about the epidemiology of the vasculitides? Best Pract Res Clin Rheumatol. 2005;19(2):191–207. https://doi.org/10.1016/j.berh.2004.11.006.CrossRefPubMed

García-Porrúa C, Calviño MC, Llorca J, Couselo JM, González-Gay MA. Henoch-Schönlein purpura in children and adults: clinical differences in a defined population. Semin Arthritis Rheum. 2002;32(3):149–56. https://doi.org/10.1053/sarh.2002.33980.CrossRefPubMed

Schonlein J. Allgemeine und specielle pathologie und therapie : Nach Seinen Vorlesungen Niedergeschrieben von Einigen Seiner Zuhörer Und Nicht Autorisiert Herausgegeben. 5th ed: Etlinger; 1832.

Henoch E. Uber eine eigentumliche form von purpura. Klin Wochenschr. 1874;11:641.

Heineke MH, Ballering AV, Jamin A, Ben Mkaddem S, Monteiro RC, Van Egmond M. New insights in the pathogenesis of immunoglobulin A vasculitis (Henoch-Schönlein purpura). Autoimmun Rev. 2017;16(12):1246–53. https://doi.org/10.1016/j.autrev.2017.10.009.CrossRefPubMed

Pillebout E. Henoch-Schonlein purpura in adults: outcome and prognostic factors. J Am Soc Nephrol. 2002;13(5):1271–8. https://doi.org/10.1097/01.ASN.0000013883.99976.22.CrossRefPubMed

Mills JA, Michel BA, Bloch DA, Calabrese LH, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of henoch-schönlein purpura. Arthritis Rheum. 2010;33(8):1114–21. https://doi.org/10.1002/art.1780330809.CrossRef

Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, et al. Nomenclature of systemic vasculitides. Arthritis Rheum. 1994;37(2):187–92. https://doi.org/10.1002/art.1780370206.CrossRefPubMed

10.

Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1–11. https://doi.org/10.1002/art.37715.CrossRefPubMed

11.

Ortiz-Sanjuán F, Blanco R, Hernández JL, et al. Applicability of the 2006 European League Against Rheumatism (EULAR) criteria for the classification of Henoch-Schönlein purpura. An analysis based on 766 patients with cutaneous vasculitis. Clin Exp Rheumatol. 2015;33(2 Suppl 89):S-44–7.

12.

Hočevar A, Rotar Z, Jurčić V, Pižem J, Čučnik S, Vizjak A, et al. IgA vasculitis in adults: the performance of the EULAR/PRINTO/PRES classification criteria in adults. Arthritis Res Ther. 2016;18(1):58. https://doi.org/10.1186/s13075-016-0959-4.CrossRefPubMedPubMedCentral

13.

Zurada JM, Ward KM, Grossman ME. Henoch-Schönlein purpura associated with malignancy in adults. J Am Acad Dermatol. 2006;55(5):S65–70. https://doi.org/10.1016/j.jaad.2005.10.011.CrossRefPubMed

14.

Audemard-Verger A, Pillebout E, Guillevin L, Thervet E, Terrier B. IgA vasculitis (Henoch–Shönlein purpura) in adults: diagnostic and therapeutic aspects. Autoimmun Rev. 2015;14(7):579–85. https://doi.org/10.1016/j.autrev.2015.02.003.CrossRefPubMed

15.

Kang Y, Park J, Ha Y-J, Kang MI, Park HJ, Lee SW, et al. Differences in clinical manifestations and outcomes between adult and child patients with Henoch-Schönlein purpura. J Korean Med Sci. 2014;29(2):198–203. https://doi.org/10.3346/jkms.2014.29.2.198.CrossRefPubMedPubMedCentral

16.

Blanco R, Martínez-Taboada VM, Rodríguez-Valverde V, García-Fuentes M, Gonzalez-Gay MA. Henoch-Schönlein purpura in adulthood and childhood. Two different expressions of the same syndrome. Arthritis Rheum. 1997;40(5):859–64. https://doi.org/10.1002/art.1780400513.CrossRefPubMed

17.

Hitomi K, Izaki S, Teraki Y, Aso Y, Yokoyama M, Takamura S, et al. Characterization of adult-type IgA vasculitis (Henoch-Schönlein Purpura): a retrospective study of 122 cases. Open Dermatol J. 2014;8(1):51–9. https://doi.org/10.2174/1874372201408010051.CrossRef

18.

Abbara S, Grateau G, Ducharme-Bénard S, Saadoun D, Georgin-Lavialle S. Association of vasculitis and familial Mediterranean fever. Front Immunol. 2019;10:763. https://doi.org/10.3389/fimmu.2019.00763.CrossRefPubMedPubMedCentral

19.

Villatoro-Villar M, Crowson C, Makol A, Ytterberg S, Warrington K, Koster M. 170. IGA vasculitis associated with inflammatory bowel disease. A retrospective study. Rheumatology. 2019;58(Supplement_2). https://doi.org/10.1093/rheumatology/kez059.047.

20.

Gong EJ, Kim DH, Chun JH, Ahn JY, Choi KS, Jung KW, et al. Endoscopic findings of upper gastrointestinal involvement in primary vasculitis. Gut Liver. 2016;10(4):542–8. https://doi.org/10.5009/gnl15198.CrossRefPubMedPubMedCentral

21.

Roberts PF, Waller TA, Brinker TM, Riffe IZ, Sayre JW, Bratton RL. Henoch-Schönlein purpura: a review article. South Med J. 2007;100(8):821–4. https://doi.org/10.1097/SMJ.0b013e3180f62d0f.CrossRefPubMed

22.

Pillebout E, Niaudet P. Henoch-Schönlein purpura. Rev Prat. 2008;58(5):507–11.PubMed

23.

Chao HC, Kong MS, Lin SJ. Hepatobiliary involvement of Henoch-Schönlein purpura in children. Acta Paediatr Taiwan. 2000;41(2):63–8.PubMed

24.

Shrestha S. Henoch Schonlein purpura with nephritis in adults: adverse prognostic indicators in a UK population. QJM. 2006;99(4):253–65. https://doi.org/10.1093/qjmed/hcl034.CrossRefPubMed

25.

Kanaan N, Mourad G, Thervet E, Peeters P, Hourmant M, Vanrenterghem Y, et al. Recurrence and graft loss after kidney transplantation for Henoch–Schönlein purpura nephritis: a multicenter analysis. Clin J Am Soc Nephrol. 2011;6(7):1768–72. https://doi.org/10.2215/CJN.00520111.CrossRefPubMed

26.

Han SS, Sun H-K, Lee JP, Ha JW, Kim SJ, Kim YS. Outcome of renal allograft in patients with Henoch-Schönlein nephritis: single-center experience and systematic review. Transplantation. 2010;89(6):721–6. https://doi.org/10.1097/TP.0b013e3181c9cc4a.CrossRefPubMed

27.

Nadrous HF, Yu AC, Specks U, Ryu JH. Pulmonary…Henoch-Schönlein purpura. Mayo Clin Proc. 2004;79(9):1151–7. https://doi.org/10.4065/79.9.1151.CrossRefPubMed

28.

Polizzotto MN, Gibbs SDJ, Beswick W, Seymour JF. Cardiac involvement in Henoch-Schonlein purpura. Intern Med J. 2006;36(5):328–31. https://doi.org/10.1111/j.1445-5994.2006.01061.x.CrossRefPubMed

29.

Modi S, Mohan M, Jennings A. Acute scrotal swelling in Henoch-Schonlein purpura: case report and review of the literature. Urol Case Rep. 2016;6:9–11. https://doi.org/10.1016/j.eucr.2016.01.004.CrossRefPubMedPubMedCentral

30.

Garzoni L, Vanoni F, Rizzi M, Simonetti GD, Simonetti BG, Ramelli GP, et al. Nervous system dysfunction in Henoch-Schonlein syndrome: systematic review of the literature. Rheumatology. 2009;48(12):1524–9. https://doi.org/10.1093/rheumatology/kep282.CrossRefPubMed

31.

Tayabali S, Andersen K, Yoong W. Diagnosis and management of Henoch-Schönlein purpura in pregnancy: a review of the literature. Arch Gynecol Obstet. 2012;286(4):825–9. https://doi.org/10.1007/s00404-012-2468-2.CrossRefPubMed

32.

Lin Q, Min Y, Li Y, Zhu Y, Song X, Xu Q, et al. Henoch–Schönlein purpura with hypocomplementemia. Pediatr Nephrol. 2012;27(5):801–6. https://doi.org/10.1007/s00467-011-2070-z.CrossRefPubMed

33.

Calviño MC, Llorca J, García-Porrúa C, Fernández-Iglesias JL, Rodriguez-Ledo P, González-Gay MA. Henoch-Schönlein purpura in children from Northwestern Spain: a 20-year epidemiologic and clinical study. Medicine (Baltimore). 2001;80(5):279–90. https://doi.org/10.1097/00005792-200109000-00001.CrossRef

34.

Sais G, Vidaller A, Jucglà A, Servitje O, Condom E, Peyrí J. Prognostic factors in leukocytoclastic vasculitis: a clinicopathologic study of 160 patients. Arch Dermatol. 1998;134(3):309–15. https://doi.org/10.1001/archderm.134.3.309.CrossRefPubMed

35.

Linskey KR, Kroshinsky D, Mihm MC, Hoang MP. Immunoglobulin-A–associated small-vessel vasculitis: a 10-year experience at the Massachusetts General Hospital. J Am Acad Dermatol. 2012;66(5):813–22. https://doi.org/10.1016/j.jaad.2011.06.012.CrossRefPubMed

36.

Jelusic M, Sestan M, Cimaz R, Ozen S. Different histological classifications for Henoch-Schönlein purpura nephritis: which one should be used? Pediatr Rheumatol Online J. 2019;17(1):10. https://doi.org/10.1186/s12969-019-0311-z.CrossRefPubMedPubMedCentral

37.

Trimarchi H, Barratt J, Cattran DC, Cook HT, Coppo R, Haas M, et al. Oxford Classification of IgA nephropathy 2016: an update from the IgA Nephropathy Classification Working Group. Kidney Int. 2017;91(5):1014–21. https://doi.org/10.1016/j.kint.2017.02.003.CrossRefPubMed

38.

Yun D, Kim DK, Oh K-H, Joo KW, Moon KC, Kim YS, et al. MEST-C pathological score and long-term outcomes of child and adult patients with Henoch-Schönlein purpura nephritis. BMC Nephrol. 2020;21(1):33. https://doi.org/10.1186/s12882-020-1691-5.CrossRefPubMedPubMedCentral

39.

Inagaki K, Kaihan AB, Hachiya A, Ozeki T, Ando M, Kato S, et al. Clinical impact of endocapillary proliferation according to the Oxford Classification among adults with Henoch-Schönlein purpura nephritis: a multicenter retrospective cohort study. BMC Nephrol. 2018;19(1):208. https://doi.org/10.1186/s12882-018-1009-z.CrossRefPubMedPubMedCentral

40.

Ho Kim C, Jin Lim B, Sung Bae Y, Eun Kwon Y, Ly Kim Y, Heon Nam K, et al. Using the Oxford Classification of IgA nephropathy to predict long-term outcomes of Henoch–Schönlein purpura nephritis in adults. Mod Pathol. 2014;27(7):972–82. https://doi.org/10.1038/modpathol.2013.222.CrossRef

41.

Roman C, Dima B, Muyshont L, Schurmans T, Gilliaux O. Indications and efficiency of dapsone in IgA vasculitis (Henoch-Schonlein purpura): case series and a review of the literature. Eur J Pediatr. 2019;178(8):1275–81. https://doi.org/10.1007/s00431-019-03409-5.CrossRefPubMed

42.

Ronkainen J, Koskimies O, Ala-Houhala M, Antikainen M, Merenmies J, Rajantie J, et al. Early prednisone therapy in Henoch-Schönlein purpura: a randomized, double-blind, placebo-controlled trial. J Pediatr. 2006;149(2):241–7. https://doi.org/10.1016/j.jpeds.2006.03.024.CrossRefPubMed

43.

Rauen T, Eitner F, Fitzner C, Sommerer C, Zeier M, Otte B, et al. Intensive supportive care plus immunosuppression in IgA nephropathy. N Engl J Med. 2015;373(23):2225–36. https://doi.org/10.1056/NEJMoa1415463.CrossRefPubMed

44.

Lv J, Zhang H, Wong MG, Jardine MJ, Hladunewich M, Jha V, et al. Effect of oral methylprednisolone on clinical outcomes in patients with IgA nephropathy: the TESTING randomized clinical trial. JAMA. 2017;318(5):432–42. https://doi.org/10.1001/jama.2017.9362.CrossRefPubMedPubMedCentral

45.

Pillebout E, Alberti C, Guillevin L, Ouslimani A, Thervet E. The CESAR study group. Addition of cyclophosphamide to steroids provides no benefit compared with steroids alone in treating adult patients with severe Henoch Schönlein purpura. Kidney Int. 2010;78(5):495–502. https://doi.org/10.1038/ki.2010.150.CrossRefPubMed

46.

Audemard-Verger A, Terrier B, Dechartres A, Chanal J, Amoura Z, le Gouellec N, et al. Characteristics and management of IgA vasculitis (Henoch-Schönlein) in adults: data from 260 patients included in a French multicenter retrospective survey: IgA vasculitis in adults. Arthritis Rheumatol. 2017;69(9):1862–70. https://doi.org/10.1002/art.40178.CrossRefPubMed

47.

Maritati F, Fenoglio R, Pillebout E, Emmi G, Urban ML, Rocco R, et al. Brief report: rituximab for the treatment of adult-onset IgA vasculitis (Henoch-Schönlein). Arthritis Rheumatol. 2018;70(1):109–14. https://doi.org/10.1002/art.40339.CrossRefPubMed

48.

Lafayette RA, Canetta PA, Rovin BH, Appel GB, Novak J, Nath KA, et al. A randomized, controlled trial of rituximab in IgA nephropathy with proteinuria and renal dysfunction. J Am Soc Nephrol. 2017;28(4):1306–13. https://doi.org/10.1681/ASN.2016060640.CrossRefPubMed

49.

Hernández-Rodríguez J, Carbonell C, Mirón-Canelo J-A, Diez-Ruiz S, Marcos M, Chamorro AJ. Rituximab treatment for IgA vasculitis: a systematic review. Autoimmun Rev. 2020;19(4):102490. https://doi.org/10.1016/j.autrev.2020.102490.CrossRefPubMed

50.

Ren P, Han F, Chen L, Xu Y, Wang Y, Chen J. The combination of mycophenolate mofetil with corticosteroids induces remission of Henoch-Schönlein purpura nephritis. Am J Nephrol. 2012;36(3):271–7. https://doi.org/10.1159/000341914.CrossRefPubMed

51.

Han F, Chen L, Ren P, le JY, Choong PJ, Wang HJ, et al. Mycophenolate mofetil plus prednisone for inducing remission of Henoch-Schönlein purpura nephritis: a retrospective study. J Zhejiang Univ Sci B. 2015;16(9):772–9. https://doi.org/10.1631/jzus.B1400335.CrossRefPubMedPubMedCentral

52.

Shin JI, Park JM, Shin YH, Kim JH, Lee JS, Kim PK, et al. Can azathioprine and steroids alter the progression of severe Henoch-Schönlein nephritis in children? Pediatr Nephrol. 2005;20(8):1087–92. https://doi.org/10.1007/s00467-005-1869-x.CrossRefPubMed

53.

Kalliakmani P, Benou E, Goumenos DS. Cyclosporin A in adult patients with Henoch-Schönlein purpura nephritis and nephrotic syndrome; 5 case reports. Clin Nephrol. 2011;75(04):380–3. https://doi.org/10.5414/CN106553.CrossRefPubMed

54.

Zhang D-F, Hao G-X, Li C-Z, Yang YJ, Liu FJ, Liu L, et al. Off-label use of tacrolimus in children with Henoch-Schönlein purpura nephritis: a pilot study. Arch Dis Child. 2018;103(8):772–5. https://doi.org/10.1136/archdischild-2017-313788.CrossRefPubMed

55.

Floege J, Barbour SJ, Cattran DC, Hogan JJ, Nachman PH, Tang SCW, et al. Management and treatment of glomerular diseases (part 1): conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney Int. 2019;95(2):268–80. https://doi.org/10.1016/j.kint.2018.10.018.CrossRefPubMed

56.

Liu L-J, Yang Y, Shi S-F, Bao YF, Yang C, Zhu SN, et al. Effects of hydroxychloroquine on proteinuria in IgA nephropathy: a randomized controlled trial. Am J Kidney Dis. 2019;74(1):15–22. https://doi.org/10.1053/j.ajkd.2019.01.026.CrossRefPubMed

57.

Kiryluk K, Novak J, Gharavi AG. Pathogenesis of immunoglobulin A nephropathy: recent insight from genetic studies. Annu Rev Med. 2013;64(1):339–56. https://doi.org/10.1146/annurev-med-041811-142014.CrossRefPubMed

58.

Ring T, Pedersen BB, Salkus G, Goodship THJ. Use of eculizumab in crescentic IgA nephropathy: proof of principle and conundrum? Clin Kidney J. 2015;8(5):489–91. https://doi.org/10.1093/ckj/sfv076.CrossRefPubMedPubMedCentral

59.

Rosenblad T, Rebetz J, Johansson M, Békássy Z, Sartz L, Karpman D. Eculizumab treatment for rescue of renal function in IgA nephropathy. Pediatr Nephrol. 2014;29(11):2225–8. https://doi.org/10.1007/s00467-014-2863-y.CrossRefPubMed

60.

National Library of Medicine. Safety and efficacy study of fostamatinib to treat immunoglobulin A (IgA) nephropathy.; 2014. https://ClinicalTrials.gov/show/NCT02112838.

61.

National Library of Medicine. Efficacy of colchicine to prevent skin relapses in adult’s IgA vasculitis. https://ClinicalTrials.gov/show/NCT04008316.

62.

Byun J-W, Song H-J, Kim L, Shin J-H, Choi G. Predictive factors of relapse in adult with Henoch-Schönlein purpura. Am J Dermatopathol. 2012;34(2):139–44. https://doi.org/10.1097/DAD.0b013e3182157f90.CrossRefPubMed

Titel: IgA Vasculitis in Adults: a Rare yet Challenging Disease
verfasst von: Kinanah Yaseen
Leal C Herlitz
Alexandra Villa-Forte
Publikationsdatum: 01.07.2021
Verlag: Springer US
Erschienen in: Current Rheumatology Reports / Ausgabe 7/2021
Print ISSN: 1523-3774
Elektronische ISSN: 1534-6307
DOI: https://doi.org/10.1007/s11926-021-01013-x

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