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http://dx.doi.org/10.3345/kjp.2019.00514

Treatment of refractory IgA vasculitis with dapsone: a systematic review  

Lee, Keum Hwa (Department of Pediatrics, Yonsei University College of Medicine)
Hong, Sung Hwi (Yonsei University College of Medicine)
Jun, Jinhae (Yonsei University College of Medicine)
Jo, Youngheun (Yonsei University College of Medicine)
Jo, Woogyeong (Yonsei University College of Medicine)
Choi, Dayeon (Yonsei University College of Medicine)
Joo, Jeongho (Yonsei University College of Medicine)
Jung, Guhyun (Yonsei University College of Medicine)
Ahn, Sunghee (Yonsei University College of Medicine)
Kronbichler, Andreas (Department of Internal Medicine IV (Nephrology and Hypertension), Medical University of Innsbruck)
Eisenhut, Michael (Luton & Dunstable University Hospital NHS Foundation Trust)
Shin, Jae Il (Department of Pediatrics, Yonsei University College of Medicine)
Publication Information
Clinical and Experimental Pediatrics / v.63, no.5, 2020 , pp. 158-163 More about this Journal
Abstract
IgA vasculitis, formerly known as Henoch-Schönlein purpura, is a systemic IgA-mediated vasculitis of the small vessels commonly seen in children. The natural history of IgA vasculitis is generally self-limiting; however, one-third of patients experience symptom recurrence and a refractory course. This systematic review examined the use of dapsone in refractory IgA vasculitis cases. A literature search of PubMed databases retrieved 13 articles published until June 14, 2018. The most common clinical feature was a palpable rash (100% of patients), followed by joint pain (69.2%). Treatment response within 1-2 days was observed in 6 of 26 patients (23.1%) versus within 3-7 days in 17 patients (65.4%). Relapse after treatment discontinuation was reported in 17 patients (65.4%) but not in 3 patients (11.5 %). Four of the 26 patients (15.4%) reported adverse effects of dapsone including arthralgia (7.7%), rash (7.7%), and dapsone hypersensitivity syndrome (3.8%). Our findings suggest that dapsone may affect refractory IgA vasculitis. Multicenter randomized placebo-controlled trials are necessary to determine the standard dosage of dapsone at initial or tapering of treatment in IgA vasculitis patients and evaluate whether dapsone has a significant benefit versus steroids or other medications.
Keywords
IgA vasculitis; Henoch-Schonlein purpura; Dapsone; Systematic review;
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