Successful Treatment of IgA Vasculitis Complicated with Bowel Perforation and Crescentic Glomerulonephritis by Combination Therapy of Glucocorticoid, Cyclosporine and Factor XIII Replacement

Keiko Koshiba; Sei Muraoka; Toshihiro Nanki; Satoru Komatsumoto

doi:10.2169/internalmedicine.0931-18

This article has now been updated. Please use the final version.

Successful Treatment of IgA Vasculitis Complicated with Bowel Perforation and Crescentic Glomerulonephritis by Combination Therapy of Glucocorticoid, Cyclosporine and Factor XIII Replacement

Keiko Koshiba, Sei Muraoka, Toshihiro Nanki, Satoru Komatsumoto

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Keywords: IgA vasculitis, Henoch-Schönlein purpura, cyclosporine, coagulation factor XIII, and bowel perforation

JOURNAL OPEN ACCESS Advance online publication

Article ID: 0931-18

DOI https://doi.org/10.2169/internalmedicine.0931-18

The final version of this article is now available: Vol. 57 (2018), No. 20 pp. 3035-3040

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Abstract

We report the findings of an 18-year-old boy with immunoglobulin A vasculitis (IgAV) complicated with bowel perforation and nephritis. He presented with abdominal pain, arthralgia and palpable purpura. Massive proteinuria developed during his clinical course. The patient was treated successfully using combination therapy of glucocorticoid (GC), cyclosporine (CYA) and factor XIII (F XIII) replacement. A standard treatment strategy for severe IgAV patients has not been established due to its rarity. Combination therapy using GC, CYA and F XIII replacement should be considered for severe IgAV patients.

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