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Clin Exp Pediatr > Accepted Articles
DOI: https://doi.org/10.3345/kjp.2019.00514    [Accepted]
Published online September 24, 2019.
Treatment of Refractory Henoch-Schonlein Purpura (HSP) with Dapsone: A Systematic Review
Keum Hwa Lee1,2  , Sung Hwi Hong3, Jinhae Jun3, Youngheun Jo3, Woogyeong Jo3, Dayeon Choi3, Jeongho Joo3, Guhyun Jung3, Sunghee Ahn3, Andreas Kronbichler4, Michael Eisenhut5, Jae Il Shin1,2,6 
1Department of Pediatrics, Yonsei University College of Medicine, Republic of Korea, Seoul, Korea
2Division of Pediatric Nephrology, Severance Children’s Hospital, Seoul , Korea
3Yonsei University College of Medicine, Seoul, Republic of Korea, Seoul , Korea
4Department of Internal Medicine IV (Nephrology and Hypertension), Medical University of Innsbruck, Innsbruck, Austria
5Luton & Dunstable University Hospital NHS Foundation Trust, Luton, United Kingdom
6Institute of Kidney Disease Research, Yonsei University College of Medicine, Seoul , Korea
Jae Il Shin, Tel: +82-2-2228-2050, Fax: +82-2-393-9118, Email: shinji@yuhs.ac
Received: 15 May 2019   • Revised: 12 September 2019   • Accepted: 23 September 2019
Henoch-Schonlein Purpura (HSP) is a systemic IgA-mediated vasculitis of small vessels common in children. The natural history of HSP is generally self-limiting, however, one third of patients experience recurrence of symptoms showing refractory courses. Our objective was to generate a systematic review of the use of dapsone in refractory HSP. A literature search of PubMed and MEDLINE databases was performed and 13 articles were finally identified published until June 14, 2018. The most common clinical feature was a palpable rash which was seen in all patients (100%) and joint pain was the following (69.2%). Treatment response within 1-2 days was observed in 6 of the 26 (23.1%) patients and 17 (65.4%) patients responded within 3 to 7 days. Relapse after discontinuation of treatment was reported in 17 patients (65.4%), whereas 3 patients (11.5%) reported no relapse of symptoms. Four of the 26 (15.4%) patients reported adverse effects of dapsone. The adverse effects included arthralgia (7.7%), rash (7.7%) and dapsone hypersensitivity syndrome (3.8%). This systematic study suggests that dapsone has an effect on refractory HSP. To determine the standard dosage of dapsone at initial treatment or tapering of treatment in HSP patients and evaluate whether dapsone has a significant benefit compared with steroids or other medication, multicenter, randomized placebo controlled clinical trials are necessary.
Key Words: Henoch-Schonlein Purpura (HSP), Dapsone, Systematic Review

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