Selcan DEMİR, Müferret ERGÜVEN, Cengiz CANDAN, Pınar TURHAN

Henoch Schönlein Purpura / Ig A Vasculitis in Children and Risk Factors for Renal Involvement

Objective: Henoch Schönlein Purpura also known as IgA vasculitis is the most common form of pediatric vasculitis and renal involvement is responsible for the mortality and long-term morbidity. We aimed to describe the epidemiological, clinical, and laboratory characteristics of patients with IgAV and analyze the predicting factors associated with renal involvement. Materials and Methods: This study included188 children diagnosed with IgA vasculitis. Demographical, and clinical data were retrospectively reviewed from the patient files. Results: Of the 188 IgA vasculitis patients, 51.6% were female. The mean±SD age at diagnosis was 8.49±3.35 years, and 66% of them were diagnosed before 10 years of age. All the patients had palpable purpura, 35.6% had arthritis, 34.6% had gastrointestinal system involvement, 12.2% had renal disorders, at the time of diagnosis. Besides 23(12.2%) patients presented with renal involvement, 42(22%) patients developed renal involvement at follow-up. Patients under 10 years of age had significantly more arthritis, patients over 10 years of age had significantly more renal involvement. Among laboratory workup, erythrocyte sedimentation rate levels were found significantly higher in patients with renal involvement. In multivariate analysis, the occurrence of renal involvement was not associated with any of the defined demographic and clinical characteristics of the disease. Although erythrocyte sedimentation rate levels showed a higher risk ratio, it has only borderline significance. Conclusion: Although IgA vasculitis is a self-limiting disease, renal involvement can cause serious complications. In the presented study, being older than 10 years of age and having high levels of erythrocyte sedimentation rate at the time of diagnosis could serve as a possible predictor of renal involvement.

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[1] Gardner-Medwin JM, Dolezalova P, Cummins C, et al. Incidence of Henoch-Schonlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet 2002, 360(9341): 1197-1202.

[2] Piram M, Mahr A. Epidemiology of immunoglobulin A vasculitis (Henoch-Schonlein): current state of knowledge. Curr Opin Rheumatol 2013, 25(2): 171-178.

[3] Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013, 65(1): 1-11.

[4] Demir S, Sonmez HE, Ozen S. Vasculitis: Decade in Review. Curr Rheumatol Rev 2019, 15(1): 14-22.

[5] Bogdanovic R: Henoch-Schonlein purpura nephritis in children: risk factors, prevention and treatment. Acta Paediatr 2009, 98(12): 1882-1889.

[6] Ronkainen J, Nuutinen M, Koskimies O. The adult kidney 24 years after childhood Henoch-Schonlein purpura: a retrospective cohort study. Lancet 2002, 360(9334): 666- 670.

[7] Narchi H. Risk of long term renal impairment and duration of follow up recommended for Henoch-Schonlein purpura with normal or minimal urinary findings: a systematic review. Arch Dis Child 2005, 90(9): 916-920.

[8] de Almeida JL, Campos LM, Paim LB, et al. Renal involvement in Henoch-Schonlein purpura: a multivariate analysis of initial prognostic factors. J Pediatr (Rio J) 2007, 83(3): 259-266.

[9] Kaku Y, Nohara K, Honda S. Renal involvement in HenochSchonlein purpura: a multivariate analysis of prognostic factors. Kidney Int 1998, 53(6):1755-1759.

[10] Chan H, Tang YL, Lv XH, et al. Risk Factors Associated with Renal Involvement in Childhood Henoch-Schonlein Purpura: A Meta-Analysis. PLoS One 2016, 11(11): e0167346.

[11] Sano H, Izumida M, Shimizu H, et al. Risk factors of renal involvement and significant proteinuria in HenochSchonlein purpura. Eur J Pediatr 2002, 161(4): 196-201.

[12] Rigante D, Candelli M, Federico G, et al. Predictive factors of renal involvement or relapsing disease in children with Henoch-Schonlein purpura. Rheumatol Int 2005, 25(1): 45- 48.

[13] 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(5): 798-806.

[14] Karadag SG, Tanatar A, Sonmez HE, et al. The clinical spectrum of Henoch-Schonlein purpura in children: a single-center study. Clin Rheumatol 2019, 38(6): 1707- 1714.

[15] Ekinci RMK, Balci S, Melek E, et al. Clinical manifestations and outcomes of 420 children with Henoch Schonlein Purpura from a single referral center from Turkey: A threeyear experience. Mod Rheumatol 2020, 30(6): 1039-1046.

[16] Ozturk K, Cakan M. Initial manifestations and short term follow-up results of Henoch-Schonlein purpura in children: A report from two centers. North Clin Istanb 2020, 7(4): 341-347.

[17] Hocevar A, Rotar Z, Ostrovrsnik J, et al. Incidence of IgA vasculitis in the adult Slovenian population. Br J Dermatol 2014, 171(3): 524-527.

[18] 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(3): 143-153.

[19] Calvino MC, Llorca J, Garcia-Porrua C, et al. HenochSchonlein purpura in children from northwestern Spain: a 20-year epidemiologic and clinical study. Medicine (Baltimore) 2001, 80(5): 279-290.

[20] Yang YH, Hung CF, Hsu CR, et al. A nationwide survey on epidemiological characteristics of childhood HenochSchonlein purpura in Taiwan. Rheumatology (Oxford) 2005, 44(5): 618-622.

[21] Chen O, Zhu XB, Ren P, et al. Henoch Schonlein Purpura in children: clinical analysis of 120 cases. Afr Health Sci 2013, 13(1): 94-99.

[22] 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(2): 106-113.

[23] Shim JO, Han K, Park S, et al.Ten-year Nationwide Population-based Survey on the Characteristics of Children with Henoch-Schnlein Purpura in Korea. J Korean Med Sci 2018, 33(25):e174.

[24] Cassidy J, Petty RE, Laxer RM, et al. Textbook of Pediatric Rheumatology. Elsevier Saunders, Philadelphia, 2005; 492- 496.

[25] Akgün C AS, Kaya A, Temel H et al. Çocukluk Çağı HenochSchönlein Purpuralı Hastaların Klinik Analizleri. Türkderm 2012; 46: 73-7.

[26] Uppal SS, Hussain MA, Al-Raqum HA, et al. HenochSchonlein’s purpura in adults versus children/adolescents: A comparative study. Clin Exp Rheumatol 2006, 24(2 Suppl 41): S26-30.

[27] Acar BÇ, Dallar Y et al. Çocukluk çağında henoch schönlein purpurası tanısı ile izlenen 168 olgunun sistem tutulumlarının değerlendirilmesi. Ege Tıp Dergisi 49 (1): 7-12, 2010.

[28] Jauhola O, Ronkainen J, Koskimies O, et al. Clinical course of extrarenal symptoms in Henoch-Schonlein purpura: a 6-month prospective study. Arch Dis Child 2010, 95(11): 871-876.

[29] Nong BR, Huang YF, Chuang CM, et al. Fifteen-year experience of children with Henoch-Schonlein purpura in southern Taiwan, 1991-2005. J Microbiol Immunol Infect 2007, 40(4): 371-376.

[30] Chang WL, Yang YH, Lin YT, et al. Gastrointestinal manifestations in Henoch-Schonlein purpura: a review of 261 patients. Acta Paediatr 2004, 93(11): 1427-1431.

[31] Lucas Garcia J, Alvarez Blanco O, Sanahuja Ibanez MJ, et al. [Outcome of Henoch-Schonlein nephropathy in pediatric patients. Prognostic factors]. Nefrologia 2008, 28(6): 627- 632.

[32] Coppo R, Mazzucco G, Cagnoli L, et al. Long-term prognosis of Henoch-Schonlein nephritis in adults and children. Italian Group of Renal Immunopathology Collaborative Study on Henoch-Schonlein purpura. Nephrol Dial Transplant 1997, 12(11): 2277-2283.

[33] Demircioglu Kilic B, Kasap Demir B: Determination of Risk Factors in Children Diagnosed With Henoch-Schonlein Purpura. Arch Rheumatol 2018, 33(4): 395-401.

[34] Wang K, Sun X, Cao Y, et al. Risk factors for renal involvement and severe kidney disease in 2731 Chinese children with Henoch-Schonlein purpura: A retrospective study. Medicine (Baltimore) 2018, 97(38): e12520.

[35] Scharer K, Krmar R, Querfeld U, et al. Clinical outcome of Schonlein-Henoch purpura nephritis in children. Pediatr Nephrol 1999, 13(9): 816-823.