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

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 usi...

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Detalles Bibliográficos
Autores principales: Koshiba, Keiko, Muraoka, Sei, Nanki, Toshihiro, Komatsumoto, Satoru
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2018
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6232042/
https://www.ncbi.nlm.nih.gov/pubmed/29877285
http://dx.doi.org/10.2169/internalmedicine.0931-18
Descripción
Sumario: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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