Ceyhun AÇARI, Meral BAYRAM, Gizem YILDIZ, Salih KAVUKÇU, Alper SOYLU

IgA Vaskülitli çocuk hastaların demografik, klinik, laboratuvar bulguları ve tedavi sonuçları: tek merkez deneyimi

Amaç: Henoch-Schönlein purpurası (HSP) olarak adlandırılan İmmünoglobin A (IgA) vasküliti (IgAV), damar duvarlarında IgA baskın immün birikimi ile karakterize küçük damar vaskülitidir. Bu çalışmada merkezimizde IgAV/HSP tanısı almış hastaların demografik özelliklerini, klinik ve laboratuvar bulgularını tedavilerini ve tedavi yanıtlarını değerlendirmeyi amaçladık.Gereç ve yöntem: Çocuk romatoloji ve nefroloji kliniğinde takipli 201 IgAV/HSP hastasının kayıtları retrospektif olarak değerlendirildi. Bulgular: Kız ve erkekler arasında hastalık eşit sıklıkta görüldü. Hastaların tamamında purpura varken diğer bulgular sıklık sırasına göre gastrointestinal, eklem, renal, subkutan ödem, testiküler tutulum idi. İnvajinasyon gelişen hastaların oranı %2,5’tu ve hiçbirinde cerrahi tedavi gerekmedi. Persistan proteinüri veya hematürisi olan hastalara biyopsi uygulandı. Histopatolojik tanıları sırayla mezengial proliferasyon, kresent ve minimal değişiklik idi. Döküntü ile relaps gelişen olgularda renal tutulum oranı yüksek iken (p=0,046) gastrointestinal ve eklem tutulum oranlarında fark yoktu. Renal tutulum olan erkeklerin böbrek histopatolojik bulgularında kresent kızlara göre daha yüksek oranda görüldü (p=0,017). Sonuç: IgAV/HSP, genel olarak, iyi prognoza sahiptir, ancak renal tutulumdan muzdarip hastalar vardır. Çalışmamızda renal tutulum gerçekleşen olgularda böbrek histopatolojisi kızlarda erkeklere göre daha hafif bulgular gösterirken diğer bulgularda fark yoktu. Relaps gelişen olgularda renal tutulum daha fazla idi.

Anahtar Kelimeler:

IgA vasküliti, renal tutulum, Henoch-Schonlein purpurası, renal histopatoloji

Demographics, clinical, laboratory findings and treatment results of pediatric patients with IgA Vasculitis: Single-center experience

Objective: Immunoglobulin A (IgA) vasculitis (IgAV), also known as Henoch-Schönlein purpura (HSP), is a vasculitis characterized by the accumulation of IgA in the vessel walls. In this study, we purposed to evaluate the demographics, clinical and laboratory findings, and treatments and responses of patients diagnosed with IgAV/HSP in our center.Materials and methods: The records of 201 IgAV/HSP patients who were followed up in the pediatric nephrology-rheumatology clinic were evaluated retrospectively.Results: It was seen with the equal frequency between girls and boys. While all patients had purpura, other findings were gastrointestinal, joint, renal, subcutaneous edema, and testicular involvement, in order of frequency. The rate of patients who developed intussusception was 2.5%, and none required surgical treatment. Biopsy was performed in patients with persistent proteinuria or hematuria. Histopathological diagnoses were mesangial proliferation, crescent, and minimal change, respectively. While the rate of renal involvement was high in cases with rash and relapse (p=0.046), there was no difference in gastrointestinal and joint involvement. In the histopathological findings of the boys, the crescent was higher than in the girls (p=0.017).Conclusion: IgAV/HSP generally has a good prognosis, but some patients suffer from renal involvement. In our study, renal histopathology in cases with renal involvement showed milder findings in girls than in boys, but there was no difference in other findings. Renal involvement was higher in relapsed patients.

Keywords:

IgA vasculitis, renal involvement, Henoch-Schonlein purpura, renal histopathology IgA vasküliti, renal tutulum, Henoch-Schonlein purpurası, renal histopatoloji,

PDF

___

1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised ınternational chapel hill consensus conference nomenclature of vasculitides. Arthritis Rheum 2013;65:1-11 https://doi.org/10.1002/art.37715
2. Song Y, Huang X, Yu G, et al. Pathogenesis of IgA vasculitis: an Up-To-Date review. Front Immunol 2021;12:771619. https://doi.org/10.3389/fimmu.2021.7716
3. Türe E, Yazar A. Çocuk acil kliniğinde Ig-A vasküliti (Henoch-Schönlein purpurası) tanısı alan çocuklarda trombosit indekslerinin klinik önemi. J Contemp Med 2018;8:98-102. https://doi.org/10.16899/gopctd.387725
4. Levy M, Broyer M, Arsan A, Levy Bentolila D, Habib R. Anaphylactoid purpura nephritis in childhood: natural history and immunopathology. Adv Nephrol Necker Hosp 1976;183-228.
5. Hetland LE, Susrud KS, Lindahl KH, Bygum A. Henoch-Schonlein purpura: a literature review. Acta Derm Venereol 2017;97:1160-1166. https://doi.org/10.2340/00015555-2733
6. Demir S, Kaplan O, Celebier M, et al. Predictive biomarkers of IgA vasculitis with nephritis by metabolomic analysis. Semin Arthritis Rheum 2020;50:1238-1244. https://doi.org/10.1016/j.semarthrit.2020.09.006
7. Mills JA, Michel BA, Bloch DA, et al. The American College of rheumatology 1990 criteria for the classification of Henoch-Schonlein purpura. Arthritis Rheum 1990;33:1114-1121. https://doi.org/10.1002/art.1780330809
8. Ozen S, Pistorio A, Iusan SM, et al. EULAR/PRINTO/PRES criteria 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;69:798-806. https://doi.org/10.1136/ard.2009.116657
9. Brogan P, Bagga A. Leukocytoclastic vasculitis. In: Petty RE, Laser RM, Lindsley CB, Wedderburn LR, eds. Textbook of Pediatric Rheumatology. 7th ed. Philadelphia; Elsevier, 2016;452-460.
10. Gardner Medwin JMM, Dolezalova P, Cummins C, Southwood TR. Incidence of Henoch-Schonlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet 2002;360:1197-1202. https://doi.org/10.1016/S0140-6736(02)11279-7
11. Yang YH, Hung CF, Hsu CR, et al. A nationwide survey on epidemiological characteristics of childhood Henoch-Schonlein purpura in Taiwan. Rheumatology (Oxford) 2005;44:618-622. https://doi.org/10.1093/rheumatology/keh544
12. Karadağ ŞG, Tanatar A, Sönmez HE, et al. The clinical spectrum of Henoch–Schönlein purpura in children: a single-center study. Clinical Rheumatol 2019;38:1707-1714. https://doi.org/10.1007/s10067-019-04460-1
13. Batu ED, Sarı A, Erden A, et al. Comparing immunoglobulin A vasculitis (Henoch–Schönlein purpura) in children and adults: a single-centre study from Turkey. Scand J Rheumatol 2018;47:481-486. https://doi.org/10.1080/03009742.2018.1448111
14. Kisla Ekinci RM, Balci S, Gokay Sarı S, et al. Do practical laboratory indices predict the outcomes of children with Henoch-Schönlein purpura? Postgrad Med 2019;131:295-298 https://doi.org/10.1080/00325481.2019.1609814
15. Weiss PF, Klink AJ, Luan X, Feudtner C. Temporal associationof Streptococcus, Staphylococcus, and parainfluenza pediatric hospitalizations and hospitalized cases of Henoch-Schonlein purpura. J Rheumatol 2010;37:2587-2594. https://doi.org/10.3899/jrheum.100364
16. Shim JO, Han K, Park S, Kim GH, Ko JS, Chung JY. Ten-year nationwide population-based survey on the characteristics of children with Henoch-Schnlein purpura in Korea. J Korean Med Sci 2018;33:e174. https://doi.org/10.3346/jkms.2018.33.e174
17. Wang K, Sun X, Cao Y, et al. Risk factors for renal involvement and severe kidney disease in 2731 Chinese children with Henoch-Schönlein purpura: a retrospective study. Medicine (Baltimore) 2018;97:e12520. https://doi.org/10.1097/MD.0000000000012520
18. Fretzayas A, Sionti I, Moustaki M, Papadimitriou A, Nicolaidou P. Henoch-Schonlein purpura: a long-term prospective study in Greek children. J Clin Rheumatol 2008;14:324-331. https://doi.org/10.1097/RHU.0b013e31817a240a
19. Oni L, Sampath S. Childhood IgA Vasculitis (Henoch Schonlein Purpura)-Advances and Knowledge Gaps. Front Pediatr 2019;7:257. https://doi.org/10.3389/fped.2019.00257
20. Ebert EC. Gastrointestinal manifestations of Henoch-Schonlein Purpura. Dig Dis Sci 2008;53:2011-2019. https://doi.org/10.1007/s10620-007-0147-0
21. Calvo Rio V, Loricera J, Mata C, et al. Henoch-Schonlein purpura in northern Spain: clinical spectrum of the disease in 417 patients from a single center. Medicine (Baltimore) 2014;93:106-113. https://doi.org/10.1097/MD.0000000000000019
22. Piram M, Maldini C, Biscardi S, et al. Incidence of IgA vasculitis in children estimated by four-source capture-recapture analysis: a population-based study. Rheumatology (Oxford) 2017;56:1358-1366. https://doi.org/10.1093/rheumatology/kex158
23. Trapani S, Micheli A, Grisolia F, et al. Henoch Schonlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of literature. Semin Arthritis Rheum 2005;35:143-153. https://doi.org/10.1016/j.semarthrit.2005.08.007 24. Buscatti IM, Casella BB, Aikawa NE, et al. Henoch-Schonlein purpura nephritis: initial risk factors and outcomes in a Latin American tertiary center. Clin Rheumatol 2018;37:1319-1324. https://doi.org/10.1007/s10067-017-3972-3
25. Jauhola O, Ronkainen J, Koskimies O, et al. Renal manifestations of Henoch-Schonlein purpura in a 6-month prospective study of 223 children. Arch Dis Child 2010;95:877-882. https://doi.org/10.1136/adc.2009.182394
26. Ozcakar ZB, Yalcinkaya F, Cakar N, et al. MEFV mutations modify the clinical presentation of Henoch-Schonlein purpura. J Rheumatol 2008;35:2427-2429. https://doi.org/10.3899/jrheum.080405
27. Bayram C, Demircin G, Erdogan O, Bulbul M, Caltik A, Akyuz SG. Prevalence of MEFV gene mutations and their clinical correlations in Turkish children with Henoch-Schonlein purpura. Acta Paediatr 2011;100:745-749. https://doi.org/10.1111/j.1651-2227.2011.02143.x
28. Gershoni Baruch R, Broza Y, Brik R. Prevalence and significance of mutations in the familial Mediterranean fever gene in Henoch-Schonlein purpura. J Pediatr 2003;143:658-661. https://doi.org/10.1067/S0022-3476(03)00502-X
29. Teng MC, Wang LC, Yu HH, Lee JH, Yang YH, Chiang BL. Kawasaki disease and Henoch-Schonlein purpura—10 years’ experience of childhood vasculitis at a university hospital in Taiwan. J Microbiol Immunol Infect 2012;45:22-30. https://doi.org/10.1016/j.jmii.2011.09.024