Azathioprine therapy for steroid-resistant Henoch-Schönlein purpura: a report of 6 cases

Fotis, Lampros; Tuttle, Paul V.; Baszis, Kevin; Pepmueller, Peri H.; Moore, Terry L.; White, Andrew J.

doi:10.1186/s12969-016-0100-x

Cited by 26 publications

(16 citation statements)

References 32 publications

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“…23) Factor XIII may have diagnostic value in patients with severe gastrointestinal symptoms without cutaneous rash; the infusion of activated factor XIII can be an adjunctive therapy in such patients. 24) Similarly, factor XIII and intravenous immunoglobulin therapy can be effective in IgA vasculitis patients with severe abdominal symptoms. 25,26) Despite various attempts, a standardized treatment for recurrent IgA vasculitis is lacking.…”

Section: F)mentioning

confidence: 99%

Treatment of refractory IgA vasculitis with dapsone: a systematic review

et al. 2020

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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.

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Section: F)mentioning

confidence: 99%

Treatment of refractory IgA vasculitis with dapsone: a systematic review

et al. 2020

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“…Fotis et al successfully used azathioprine in six patients with steroid-resistant IgA vasculitis including patients with persistent gastrointestinal symptoms [ 22 ]. Cyclosporine has also tried for the treatment of resistant nephritis [ 23 ], but its use is associated with several well-known side effects such as hirsutism and profound immunosuppression.…”

Section: Discussionmentioning

confidence: 99%

Intravenous γ Globulin for Intractable Abdominal Pain due to IgA Vasculitis

Naifa

Totikidis

Alexiadou

et al. 2020

Case Reports in Pediatrics

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IgA vasculitis (formerly known as Henoch–Schönlein purpura or anaphylactoid purpura) is a usually benign vasculitis that affects children of school age. The disease is characterized by the tetrad of palpable purpura, arthralgia/arthritis, abdominal pain, and hematuria. Treatment of IgA vasculitis is mainly supportive, with administration of simple analgesics. Corticosteroids have been shown to reduce and/or ameliorate the occurrence of abdominal pain which may be severe. We present two children with IgA vasculitis and severe abdominal pain despite corticosteroid administration, who responded promptly to intravenous γ globulin (IVIg) with complete resolution of their symptoms and review of the relevant medical literature. Given the toxicity and/or need for long-term administration of other second-line immunosuppressive therapies in corticosteroid-resistant IgA vasculitis, such as rituximab, cyclosporine, cyclophosphamide, azathioprine, or colchicine, we propose that IVIg may be a useful and safe treatment option, although randomized controlled clinical trials are needed in order to clarify its role in the treatment of abdominal pain in IgA vasculitis.

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“…Blood puri cation is invasive and expensive, which is not applicable for the treatment of non-life-threatening HSP. In recent years, several immunosuppressants and biological products such as methotrexate, azathioprine, dapsone, colchicine, and rituximab, have also been reported with some successes [9][10][11][12][13].…”

Section: Discussionmentioning

confidence: 99%

Preliminary experience with tacrolimus in managing Henoch-Schönlein purpura featuring extrarenal symptoms refractory to conventional therapy

Chen¹,

Yang²,

Lian³

2020

Preprint

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Background Henoch-Schönlein purpura (HSP) is the commonest vasculitis of childhood, affecting multiple organ systems. The extrarenal manifestations are prominent in some HSP cases, for which no standard treatment is available. It is interesting to examine the value of tacrolimus in such cases. Findings Seventeen children received tacrolimus to manage their refractory extrarenal symptoms of HSP in an open-label prospective study. Tacrolimus was administered at daily dose of 0.05–0.15 mg/kg. Tacrolimus was effective in all cases after treatment for 7–21 days. Tacrolimus treatment resulted in complete resolution of extrarenal symptoms in 15 cases, and partial resolution in the remaining 2 cases. During follow-up, skin purpura reoccurred in 2 cases, abdominal pain and loss of follow-up in 1 case each. Drug-related adverse reaction was not observed in any case. Abnormal renal function test was identified in 7 patients by urinary screening tests before tacrolimus therapy, 4 of them received renal biopsy. Only one patient showed evidence of renal impairment during follow-up. Conclusions Tacrolimus works quickly without drug-related adverse reaction in managing the HSP patients featuring recurrent extrarenal symptoms. Our preliminary experience suggests good clinical efficacy and safety of tacrolimus in such off-label use.

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Azathioprine therapy for steroid-resistant Henoch-Schönlein purpura: a report of 6 cases

Cited by 26 publications

References 32 publications

Treatment of refractory IgA vasculitis with dapsone: a systematic review

Treatment of refractory IgA vasculitis with dapsone: a systematic review

Intravenous γ Globulin for Intractable Abdominal Pain due to IgA Vasculitis

Preliminary experience with tacrolimus in managing Henoch-Schönlein purpura featuring extrarenal symptoms refractory to conventional therapy

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