Severe gastrointestinal vasculitis in Henoch-Schoenlein purpura: pathophysiologic mechanisms, the diagnostic value of factor XIII, and therapeutic options

Smıth, Lee; Lee, Jae Seung

doi:10.1007/s00431-007-0415-1

Cited by 15 publications

(7 citation statements)

References 9 publications

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“…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. Corticosteroids have various side effects; corticosteroid-resistant patients with IgA vasculitis have been treated effectively with mizoribine or cyclosporine A.…”

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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“…Since Factor XIII levels are found to be low in HSP patients and correlate with the severity of gastrointestinal symptoms, Factor XIII replacement has been advocated as an adjunctive therapy [24]. …”

Section: Review Of Literaturementioning

confidence: 99%

Henoch-Schonlein Purpura—A Case Report and Review of the Literature

Sohagia

Gunturu

Tong

et al. 2010

Gastroenterology Research and Practice

135

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We describe a case of an adolescent male with Henoch-Schonlein purpura (HSP), presenting with cutaneous and gastrointestinal manifestations. Endoscopy revealed diffuse ulcerations in the stomach, duodenum, and right colon. Biopsies revealed a leukocytoclastic vasculitis in the skin and gastrointestinal tract. Steroid therapy led to complete resolution of the symptoms. HSP is the most common childhood vasculitis, and is characterized by the classic tetrad of nonthrombocytopenic palpable purpura, arthritis or arthralgias, gastrointestinal and renal involvement. It is a systemic disease where antigen-antibody (IgA) complexes activate the alternate complement pathway, resulting in inflammation and small vessel vasculitis. Mild disease resolves spontaneously, and symptomatic treatment alone is sufficient. Systemic steroids are recommended for moderate to severe HSP. The prognosis depends upon the extent of renal involvement, which requires close followup. Early recognition of multiorgan involvement, especially outside of the typical age group, as in our adolescent patient, and appropriate intervention can mitigate the disease and limit organ damage.

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“…Von einzelnen Autoren wird die Bestimmung und Gabe von F XIII bei steroidtherapierefraktären Bauchschmerzen berichtet (Evidenz IIIB; [25,35,45,46]). …”

Section: Tabelle 1 Klassi Kationskriterien Der Purpura Schönlein-heunclassified

Häufigste Vaskulitis des Kindes- und Jugendalters

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Huppertz

2013

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Severe gastrointestinal vasculitis in Henoch-Schoenlein purpura: pathophysiologic mechanisms, the diagnostic value of factor XIII, and therapeutic options

Cited by 15 publications

References 9 publications

Treatment of refractory IgA vasculitis with dapsone: a systematic review

Treatment of refractory IgA vasculitis with dapsone: a systematic review

Henoch-Schonlein Purpura—A Case Report and Review of the Literature

Häufigste Vaskulitis des Kindes- und Jugendalters

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