Hanioka Yusuke, Yamagami Keiko, Yoshioka Katsunobu, Nakamura Tomomi, Kishida Masatsugu, Nakamura Tomoyuki, Yamaguchi Toshimasa, Koshimo Naomi, Inoue Takeshi, Imanishi Masahito
Department of Internal Medicine, Osaka City General Hospital, Japan.
Intern Med. 2012;51(17):2457-61. doi: 10.2169/internalmedicine.51.7725. Epub 2012 Sep 1.
A case of Churg-Strauss syndrome complicated by chronic symmetrical dacryoadenitis suggestive of Mikulicz's disease is herein presented. A 72-year-old Japanese man, who had been previously diagnosed with asthma, presented with weakness of the left leg and purpura on the lower extremities. A neurological examination showed multiple mononeuropathies and a laboratory examination revealed elevated eosinophil counts, IgE levels and the presence of Myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCAs). Churg-Strauss syndrome was diagnosed, although the patient also exhibited bilateral swelling of the lachrymal glands. Furthermore, elevated serum IgG4 levels, an infiltration of a relatively large number of IgG4-positive plasmacytes in the nasal mucosa and hypocomplementemia were also observed. These findings were consistent with a diagnosis of Mikulicz's disease (MD). Oral prednisolone (30 mg) was administered and the swelling of the lachrymal glands resolved. Churg-Strauss syndrome may be accompanied by Mikulicz's disease (an IgG4-related disease), and common pathogeneses between Churg-Strauss syndrome and IgG4-related disease may exist.
本文报告一例合并慢性对称性泪腺炎（提示米库利奇病）的变应性肉芽肿性血管炎病例。一名72岁的日本男性，既往诊断为哮喘，出现左腿无力和下肢紫癜。神经系统检查显示多发性单神经病，实验室检查显示嗜酸性粒细胞计数、IgE水平升高以及髓过氧化物酶抗中性粒细胞胞浆抗体（MPO-ANCA）阳性。尽管该患者还表现出双侧泪腺肿胀，但仍诊断为变应性肉芽肿性血管炎。此外，还观察到血清IgG4水平升高、鼻黏膜中相对大量IgG4阳性浆细胞浸润以及补体低下。这些发现符合米库利奇病（MD）的诊断。给予口服泼尼松龙（30mg）后，泪腺肿胀消退。变应性肉芽肿性血管炎可能伴有米库利奇病（一种IgG4相关疾病），变应性肉芽肿性血管炎与IgG4相关疾病之间可能存在共同的发病机制。
