Department of Immunology and Rheumatology, Unit of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Japan.
Department of Physical Therapy, Nagasaki University Graduate School of Biomedical Sciences, Japan.
Intern Med. 2023 Jun 15;62(12):1849-1855. doi: 10.2169/internalmedicine.0453-22. Epub 2022 Nov 9.
We encountered a 78-year-old Japanese man with IgG4-related sialoadenitis complicated with marked eosinophilia. We diagnosed him with IgG4-RD (related disease) with a submandibular gland tumor, serum IgG4 elevation, IgG4-positive plasma cell infiltration, and storiform fibrosis. During follow-up after total incision of the submandibular gland, the peripheral eosinophil count was markedly elevated to 29,480/μL. The differential diagnosis of severe eosinophilia without IgG4-RD was excluded. The patient exhibited a prompt response to corticosteroid therapy. His peripheral blood eosinophil count was the highest ever reported among similar cases. We also review previous cases of IgG4-RD with severe eosinophilia.
我们遇到了一位 78 岁的日本男性，患有 IgG4 相关唾液腺炎并伴有明显的嗜酸性粒细胞增多症。我们诊断他患有 IgG4-RD（相关疾病）伴颌下腺肿瘤、血清 IgG4 升高、IgG4 阳性浆细胞浸润和席纹状纤维化。在颌下腺全切除术后随访期间，外周血嗜酸性粒细胞计数显著升高至 29,480/μL。排除了无 IgG4-RD 的严重嗜酸性粒细胞增多症的鉴别诊断。患者对皮质类固醇治疗有迅速反应。他的外周血嗜酸性粒细胞计数是此类病例中报告的最高值。我们还回顾了以前 IgG4-RD 伴严重嗜酸性粒细胞增多症的病例。
