Abstract
Background
IgA vasculitis (IgAV) is the most common vasculitis in children. IgAV long-term prognosis depends on kidney involvement or IgA vasculitis with nephritis (IgAVN). To date, steroid treatment (oral steroids or methylprednisolone pulses) has not proven to be formally efficient. This study aimed to assess the role of steroids on IgAVN outcome.
Methods
All children with IgAVN diagnosed 2000–2019 in 14 French pediatric nephrology units with minimal follow-up of 6 months were retrospectively included. Outcomes of patients treated with steroids were compared with those of a control group of untreated patients matched for age, sex, proteinuria, eGFR, and histological features. The primary endpoint was IgAVN remission defined as urine protein-to-creatinine ratio < 20 mg/mmol without impaired eGFR one year after disease onset.
Results
A total of 359 patients with IgAVN were included with a median follow-up time of 249 days (range 43–809). One hundred eight (30%) patients received oral steroids alone, 207 (51%) patients received three methylprednisolone pulses followed by oral steroids, and 44 patients (12.5%) did not receive steroids. Thirty-two children treated with oral steroids alone were compared with 32 matched control patients who did not receive steroids. One year after disease onset, IgAVN remission proportion was not different between these two groups: 62% versus 68%, respectively. Ninety-three children treated with oral steroids alone were compared with 93 matched patients treated with three methylprednisolone pulses followed by oral corticosteroids. IgAVN remission proportion was not different between these two groups: 77% versus 73%, respectively.
Conclusion
The benefit of oral steroids alone and methylprednisolone pulses could not be established based on this observational study. Randomized controlled trials are thus required to determine the efficacy of steroids in IgAVN.
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References
Jennette JC, Falk RJ, Bacon PA, Basu N et al (2013) 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Arthritis Rheum 65:1–11. https://doi.org/10.1002/art.37715
Chartapisak W, Opastirakul S, Hodson EM, Willis NS et al (2009) Interventions for preventing and treating kidney disease in Henoch-Schönlein Purpura (HSP). Cochrane Database Syst Rev (3):CD005128
Stewart M, Savage JM, Bell B, McCord B (1988) Long term renal prognosis of Henoch-Schönlein purpura in an unselected childhood population. Eur J Pediatr 147:113–115
Ronkainen J, Ala-Houhala M, Huttunen NP, Jahnukainen T et al (2003) Outcome of Henoch-Schoenlein nephritis with nephrotic-range proteinuria. Clin Nephrol 60:80–84
Goldstein AR, White RH, Akuse R, Chantler C (1992) Long-term follow-up of childhood Henoch-Schönlein nephritis. Lancet 339:280–282
Ronkainen J, Koskimies O, Ala-Houhala M, Antikainen M et al (2006) Early prednisone therapy in Henoch-Schönlein purpura: a randomized, double-blind, placebo-controlled trial. J Pediatr 149:241–247. https://doi.org/10.1016/j.jpeds.2006.03.024
Niaudet P, Habib R (1998) Methylprednisolone pulse therapy in the treatment of severe forms of Schönlein-Henoch purpura nephritis. Pediatr Nephrol 12:238–243
Zaffanello M, Fanos V (2009) Treatment-based literature of Henoch-Schönlein purpura nephritis in childhood. Pediatr Nephrol 24:1901–1911. https://doi.org/10.1007/s00467-008-1066-9
(2012) Chapter 11: Henoch-Schönlein purpura nephritis. Kidney Int Suppl 2:218–220. https://doi.org/10.1038/kisup.2012.24
Ozen S, Pistorio A, Iusan SM, Bakkaloglu A et al (2010) 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 69:798–806. https://doi.org/10.1136/ard.2009.116657
Clavé S, Sordet M, Tsimaratos M, Decramer S et al (2021) Association of kidney biopsy findings with short- and medium-term outcomes in children with moderate-to-severe IgA vasculitis nephritis. Eur J Pediatr 180:3209–3218. https://doi.org/10.1007/s00431-021-04065-4
Delbet J-D, Geslain G, Auger M, Hogan J et al (2020) Histological prognostic factors in children with Henoch-Schönlein purpura nephritis. Pediatr Nephrol 35:313–320. https://doi.org/10.1007/s00467-019-04363-y
Counahan R, Winterborn MH, White RH, Heaton JM et al (1977) Prognosis of Henoch-Schönlein nephritis in children. Br Med J 2:11–14. https://doi.org/10.1136/bmj.2.6078.11
Wakaki H, Ishikura K, Hataya H, Hamasaki Y et al (2011) Henoch-Schönlein purpura nephritis with nephrotic state in children: predictors of poor outcomes. Pediatr Nephrol 26:921–925. https://doi.org/10.1007/s00467-011-1827-8
Pillebout E, Thervet E, Hill G, Alberti C et al (2002) Henoch-Schönlein Purpura in adults: outcome and prognostic factors. J Am Soc Nephrol 13:1271–1278. https://doi.org/10.1097/01.asn.0000013883.99976.22
Rauen T, Eitner F, Fitzner C, Sommerer C et al (2015) Intensive supportive care plus immunosuppression in IgA nephropathy. N Engl J Med 373:2225–2236. https://doi.org/10.1056/NEJMoa1415463
Ronkainen J, Nuutinen M, Koskimies O (2002) The adult kidney 24 years after childhood Henoch-Schönlein purpura: a retrospective cohort study. Lancet 360:666–670. https://doi.org/10.1016/S0140-6736(02)09835-5
Coppo R, Mazzucco G, Cagnoli L, Lupo A et al (1997) Long-term prognosis of Henoch-Schönlein nephritis in adults and children. Italian Group of Renal Immunopathology Collaborative Study on Henoch-Schönlein purpura. Nephrol Dial Transplant 12:2277–2283
EdströmHalling S, Söderberg MP, Berg UB (2010) Predictors of outcome in Henoch-Schönlein nephritis. Pediatr Nephrol 25:1101–1108. https://doi.org/10.1007/s00467-010-1444-y
Mir S, Yavascan O, Mutlubas F, Yeniay B et al (2007) Clinical outcome in children with Henoch-Schönlein nephritis. Pediatr Nephrol 22:64–70. https://doi.org/10.1007/s00467-006-0278-0
Hennies I, Gimpel C, Gellermann J, Möller K et al (2018) Presentation of pediatric Henoch-Schönlein purpura nephritis changes with age and renal histology depends on biopsy timing. Pediatr Nephrol 33:277–286. https://doi.org/10.1007/s00467-017-3794-1
Davin J-C, Coppo R (2013) Pitfalls in recommending evidence-based guidelines for a protean disease like Henoch-Schönlein purpura nephritis. Pediatr Nephrol 28:1897–1903. https://doi.org/10.1007/s00467-013-2550-4
Soylemezoglu O, Ozkaya O, Ozen S, Bakkaloglu A et al (2009) Henoch-Schönlein nephritis: a nationwide study. Nephron Clin Pract 112:c199-204. https://doi.org/10.1159/000218109
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Mary, AL., Clave, S., Rousset-Rouviere, C. et al. Outcome of children with IgA vasculitis with nephritis treated with steroids: a matched controlled study. Pediatr Nephrol 38, 3317–3326 (2023). https://doi.org/10.1007/s00467-023-05981-3
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DOI: https://doi.org/10.1007/s00467-023-05981-3