Journal of Vascular Surgery Cases and Innovative Techniques (Sep 2019)

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

  • Kuniyasu Ikeoka, MD, PhD,
  • Tetsuya Watanabe, MD, PhD,
  • Toshika Ohkawa, MD, PhD,
  • Takashi Tsuzuki, MD,
  • Yoshihiko Ikeda, MD, PhD,
  • Masashi Takeda, MD, PhD,
  • Shiro Hoshida, MD, PhD

Journal volume & issue
Vol. 5, no. 3
pp. 289 – 292

Abstract

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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. Keywords: Cerebral infarction, Endovascular therapy, IgG4-related disease, Mikulicz’s disease, Small-sized vasculitis