IgG4-related small-sized occlusive vasculitis in Mikulicz's disease

Mikulicz's disease is one of the IgG4-related diseases (IgG4-RDs) that involves the cardiovascular system; however, small-sized vasculitis is rare in IgG4-related diseases. A 64-year-old man presented with distal occlusive disease and developed left cerebrovascular infarction with occlusion of...

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Detalles Bibliográficos
Autores principales: Ikeoka, Kuniyasu, Watanabe, Tetsuya, Ohkawa, Toshika, Tsuzuki, Takashi, Ikeda, Yoshihiko, Takeda, Masashi, Hoshida, Shiro
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2019
Materias:
Case report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6614702/
https://www.ncbi.nlm.nih.gov/pubmed/31334401
http://dx.doi.org/10.1016/j.jvscit.2019.03.019
Descripción
Sumario:Mikulicz's disease is one of the IgG4-related diseases (IgG4-RDs) that involves the cardiovascular system; however, small-sized vasculitis is rare in IgG4-related diseases. A 64-year-old man presented with distal occlusive disease and developed left cerebrovascular infarction with occlusion of the middle cerebral artery and diseased temporal artery branches. He underwent superficial temporal artery-middle cerebral artery anastomosis surgery. Histology of the temporal artery biopsy showed smooth muscle cell proliferation with many IgG4-positive plasma cells. He then developed salivary gland inflammation, and Mikulicz's disease was diagnosed. Small-sized occlusive vasculitis was observed in this IgG4-RD. Low-dose corticosteroid therapy is effective in preventing progressive occlusive disease.

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