Follow-Up and Community Care of the Child With a Chronic Disease

EnglishTürkçe

CHAPTER


DOI :10.26650/B/CH32.2024.010.022   IUP :10.26650/B/CH32.2024.010.022    Full Text (PDF)

Follow-up and Community Care of the Child with the Diagnosis of IgA Vasculitis (Henoch Schönlein Purpura)

Gülşah Kavrul Kayaalp, Nuray Aktay Ayaz

IgA vasculitis (IgAV) is the most common vasculitis in childhood, occurring most frequently between the ages of 3 and 15. However, its etiology has not been fully elucidated. Characterized by the accumulation of IgA-dominated immune complexes in the small vessel walls and leukocytoclastic vasculitis, IgAV most frequently affects the skin, presenting as a symmetrical, palpable purpura rash, particularly on the lower extremities, as well as the gastrointestinal tract. Kidney involvement primarily determines long-term mortality and morbidity. Arthritis usually presents as selflimiting that involves large joints. Central nervous system vasculitis, pulmonary hemorrhage, interstitial pneumonia, epistaxis, carditis, adrenal hemorrhage, and testicular involvement rarely occur. Although a skin biopsy may be performed in cases where the rash is atypical to rule out other conditions, it is essential to monitor all patients with IgAV for organ involvement. For renal involvement, regular follow-ups including full urinalysis, renal function assessment, and blood pressure measurements for at least 2 years are recommended. For gastrointestinal involvement, abdominal pain, hematemesis, hematochezia, and melena should be closely monitored. Supportive treatment is indicated for patients presenting with a rash, arthritis, and arthralgia. Meanwhile, hospitalization and close monitoring are necessary for patients with severe abdominal pain, severe kidney involvement, neurological findings, or very severe skin manifestations. Steroids are used for severe gastrointestinal and renal involvement. For severe cases, immunosuppressive agents (cyclophosphamide or cyclosporine) should be considered. Long-term follow-up is necessary due to recurrence; for example, patients with severe renal involvement are more prone to renal recurrences. A healthy diet and low-sodium intake are recommended for patients using steroids, calcium and vitamin D supplementation for those receiving long-term steroid therapy, and continuation of appropriate physical activity for all patients. Importantly, as immunization guidelines should be adhered to, vaccination status of all patients should be checked.


Keywords: IgA vasculitis, child, Henoch Schönlein Purpura
DOI :10.26650/B/CH32.2024.010.022   IUP :10.26650/B/CH32.2024.010.022    Full Text (PDF)

IgA Vasküli̇ti̇ (Henoch Schönlein Purpurasi) Olan Çocuğun İzlemi̇ ve Toplumda Bakımı

Gülşah Kavrul Kayaalp, Nuray Aktay Ayaz

IgA vasküliti çocukluk çağında en sık görülen vaskülittir. En sık 3-15 yaşları arasında görülür. Etiyolojisi tam olarak aydınlatılamamıştır. Küçük damar duvarlarında IgA baskın immün komplekslerin birikimi ve lökositoklastik vaskülit ile karakterizedir. En sık etkilenen organ deridir. Tipik döküntü, alt ekstremitelerde daha belirgin olmak üzere simetrik, palpabl purpuradır. Gastrointestinal tutulum sıktır. Uzun dönem mortalite ve morbiditeyi belirleyen en önemli organ tutulumu böbrek tutulumudur. Renal tutulum değişken bir spektrumda görülebilmektedir. Artrit genellikle büyük eklemleri tutan kendi kendini sınırlayan artrit şeklinde görülür. IgA vasküliti olan çocuklarda nadiren merkezi sinir sistemi vasküliti, pulmoner hemoraji, interstisyel pnömoni, epistaksis, kardit, adrenal kanama ve testis tutulumu görülebilir. Tanı klinik olarak konulmaktadır. Döküntünün atipik olduğu durumlarda diğer tanıları dışlamak için deri biyopsisi yapılabilir. IgA vasküliti olan çocuklar organ tutulumu açısından izlenmelidir. Böbrek tutulumu açısından tüm çocuklar en az iki yıl boyunca tam idrar tahlili, böbrek fonksiyonu ve kan basıncı ölçümleri ile takip edilmelidir. Gastrointestinal tutulum açısından karın ağrısı, hematemez, hematokezya ve melena varlığı takip edilmelidir. Döküntü, artrit ve artralji varlığında destek tedavisi verilir. Şiddetli karın ağrısı, şiddetli böbrek tutulumu, nörolojik bulgular veya çok şiddetli cilt bulguları varlığında yatırılarak izlenir. Ciddi gastrointestinal tutulum ve böbrek tutulumu varlığında steroidler kullanılır. Şiddetli vakalarda, siklofosfamid veya siklosporin gibi immünsüpresif ilaçlar düşünülmelidir. Nüks görülebileceğinden uzun süreli takip gerekmektedir. Başlangıçta, şiddetli böbrek tutulumu olan çocuklarda renal nüksler daha sık görülmektedir. Takipte sağlıklı beslenme, steroid kullanmakta olan çocuklarda düşük sodyumlu diyet, uzun süreli steroid tedavisi alanlarda kalsiyum ve D vitamini desteği, tüm olgularda uygun fiziksel aktivitenin desteklenmesi ve bağışıklama diğer önemli faktörlerdir. Tüm çocukların aşılanma durumu kontrol edilmelidir.


Keywords: IgA vasküliti, çocuk, Henoch Schönleın Purpurası

References

  • 1. Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, et al. Nomenclature of systemic vascu-litides. Proposal of an international consensus conference. Arthritis Rheum. 1994;37(2):187-92. google scholar
  • 2. Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, et al. EULAR/PRINTO/PRES crite-ria for Henoch-Schonlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis. 2010;69(5):798-806. google scholar
  • 3. Piram M, Mahr A. Epidemiology of immunoglobulin A vasculitis (Henoch-Schonlein): current state of knowledge. Curr Opin Rheumatol. 2013;25(2):171-8. google scholar
  • 4. Piram M, Maldini C, Biscardi S, De Suremain N, Orzechowski C, Georget E, et al. Incidence of IgA vasculitis in children estimated by four-source capture-recapture analysis: a population-based study. Rheu-matology (Oxford). 2017;56(8):1358-66. google scholar
  • 5. Ostini A, Simonetti GD, Pellanda G, Bianchetti MG, Ferrarini A, Milani GP. Familial Henoch-Schonlein Syndrome. J Clin Rheumatol. 2016;22(2):80-1. google scholar
  • 6. Rigante D, Castellazzi L, Bosco A, Esposito S. Is there a crossroad between infections, genetics, and Henoch-Schonlein purpura? Autoimmun Rev. 2013;12(10):1016-21. google scholar
  • 7. Song Y, Huang X, Yu G, Qiao J, Cheng J, Wu J, et al. Pathogenesis of IgA Vasculitis: An Up-To-Date Review. Front Immunol. 2021;12:771619. google scholar
  • 8. Suzuki H, Yasutake J, Makita Y, Tanbo Y, Yamasaki K, Sofue T, et al. IgA nephropathy and IgA vasculitis with nephritis have a shared feature involving galactose-deficient IgA1-oriented pathogenesis. Kidney Int. 2018;93(3):700-5. google scholar
  • 9. Du L, Wang P, Liu C, Li S, Yue S, Yang Y. Multisystemic manifestations of IgA vasculitis. Clin Rheumatol. 2021;40(1):43-52. google scholar
  • 10. Hetland LE, Susrud KS, Lindahl KH, Bygum A. Henoch-Schonlein Purpura: A Literature Review. Acta Derm Venereol. 2017;97(10):1160-6. google scholar
  • 11. Jauhola O, Ronkainen J, Koskimies O, Ala-Houhala M, Arikoski P, Holtta T, et al. Clinical course of extrarenal symptoms in Henoch-Schonlein purpura: a 6-month prospective study. Arch Dis Child. 2010;95(11):871-6. google scholar
  • 12. Karadag SG, Tanatar A, Sonmez HE, Cakmak F, Kiyak A, Yavuz S, et al. The clinical spectrum of Heno-ch-Schonlein purpura in children: a single-center study. Clin Rheumatol. 2019;38(6):1707-14. google scholar
  • 13. Trapani S, Micheli A, Grisolia F, Resti M, Chiappini E, Falcini F, et al. Henoch Schonlein purpura in chil-dhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of literature. Semin Arthritis Rheum. 2005;35(3):143-53. google scholar
  • 14. Saulsbury FT. Clinical update: Henoch-Schonlein purpura. Lancet. 2007;369(9566):976-8. google scholar
  • 15. Halling SF, Soderberg MP, Berg UB. Henoch Schonlein nephritis: clinical findings related to renal function and morphology. Pediatr Nephrol. 2005;20(1):46-51. google scholar
  • 16. Brogan P, Nott KA. Immune Complex Small Vessel Vasculitis, IgA Vasculitis (Henoch-Schönlein Purpura) and Hypersensitivity Vasculitis. In: Petty RE, Laxer ML, Lindsley CB, Wedderburn LR, Mellins ED, Fuh-lbrigge RC (eds). Textbook of Pediatric Rheumatology. 8th ed. Philadelphia: Elsevier, 2021. P. 456-464. google scholar
  • 17. Ozen S, Marks SD, Brogan P, Groot N, de Graeff N, Avcin T, et al. European consensus-based recommen-dations for diagnosis and treatment of immunoglobulin A vasculitis-the SHARE initiative. Rheumatology (Oxford). 2019;58(9):1607-16. google scholar
  • 18. Lin Q, Min Y, Li Y, Zhu Y, Song X, Xu Q, et al. Henoch-Schonlein purpura with hypocomplementemia. Pediatr Nephrol. 2012;27(5):801-6. google scholar
  • 19. Narchi H. Risk of long term renal impairment and duration of follow up recommended for Henoch-Schon-lein purpura with normal or minimal urinary findings: a systematic review. Arch Dis Child. 2005;90(9):916-20. google scholar
  • 20. Gershoni-Baruch R, Broza Y, Brik R. Prevalence and significance of mutations in the familial Mediterra-nean fever gene in Henoch-Schonlein purpura. J Pediatr. 2003;143(5):658-61. google scholar
  • 21. Ozcakar ZB, Yalcinkaya F, Cakar N, Acar B, Kasapcopur O, Uguten D, et al. MEFV mutations modify the clinical presentation of Henoch-Schonlein purpura. J Rheumatol. 2008;35(12):2427-9. google scholar
  • 22. Abbara S, Grateau G, Ducharme-Benard S, Saadoun D, Georgin-Lavialle S. Association of Vasculitis and Familial Mediterranean Fever. Front Immunol. 2019;10:763. google scholar
  • 23. Chan H, Tang YL, Lv XH, Zhang GF, Wang M, Yang HP, et al. Risk Factors Associated with Renal Invol-vement in Childhood Henoch-Schonlein Purpura: A Meta-Analysis. PLoS One. 2016;11(11):e0167346. google scholar
  • 24. Coppo R, Mazzucco G, Cagnoli L, Lupo A, Schena FP. Long-term prognosis of Henoch-Schonlein nephri-tis in adults and children. Italian Group of Renal Immunopathology Collaborative Study on Henoch-Schon-lein purpura. Nephrol Dial Transplant. 1997;12(11):2277-83. google scholar
  • 25. Kanaan N, Mourad G, Thervet E, Peeters P, Hourmant M, Vanrenterghem Y, et al. Recurrence and graft loss after kidney transplantation for henoch-schonlein purpura nephritis: a multicenter analysis. Clin J Am Soc Nephrol. 2011;6(7):1768-72. google scholar
  • 26. Jones JT, Lindsley CB. Principles in the Management of Patients with Rheumatic Disease. Petty RE, Laxer ML, Lindsley CB, Wedderburn LR, Mellins ED, Fuhlbrigge RC, editors. Textbook of Pediatric Rheuma-tology (8th ed). Philadelphia: Elsevier; 2021.p.143-150. google scholar
  • 27. Gualano B, Bonfa E, Pereira RMR, Silva CA. Physical activity for paediatric rheumatic diseases: standing up against old paradigms. Nat Rev Rheumatol. 2017;13(6):368-79. google scholar
  • 28. Oliveira DS, Shinjo SK, Silva MG, de Sa-Pinto AL, Lima FR, Roschel H, et al. Exercise in Takayasu Arteritis: Effects on Inflammatory and Angiogenic Factors and Disease-Related Symptoms. Arthritis Care Res (Hoboken). 2017;69(6):892-902. google scholar
  • 29. Jansen MHA, Rondaan C, Legger GE, Minden K, Uziel Y, Toplak N, et al. EULAR/PRES recommendati-ons for vaccination of paediatric patients with autoimmune inflammatory rheumatic diseases: update 2021. Ann Rheum Dis. 2023;82(1):35-47. google scholar


SHARE




Istanbul University Press aims to contribute to the dissemination of ever growing scientific knowledge through publication of high quality scientific journals and books in accordance with the international publishing standards and ethics. Istanbul University Press follows an open access, non-commercial, scholarly publishing.