Sakata Komei, Kikuchi Jun, Emoto Katsura, Kotaki Tomomi, Ota Yuichiro, Nishina Naoshi, Hanaoka Hironari, Otomo Kotaro, Suzuki Katsuya, Kaneko Yuko, Takeuchi Tsutomu
Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Japan.
Division of Diagnostic Pathology, Keio University School of Medicine, Japan.
Intern Med. 2021 Jul 1;60(13):2135-2143. doi: 10.2169/internalmedicine.6313-20. Epub 2021 Feb 1.
We herein report a rare case of a 66-year-old man with refractory chylothorax. Although he had been treated with moderate doses of prednisolone (PSL) on suspicion of pleuritis with Sjögren syndrome, the pleural effusion expanded after the reduction of PSL. Further workup including histopathological examinations of pleura led to the diagnosis of IgG4-RD with bilateral chylothorax without any leakage from the thoracic duct. Combination therapy with high-dose PSL plus rituximab successfully decreased the pleural effusion. This is a very rare case of IgG4-related pleuritis with chylothorax and the first report of its successful treatment with rituximab.
我们在此报告一例罕见的66岁男性难治性乳糜胸病例。尽管因怀疑干燥综合征合并胸膜炎而给予中等剂量泼尼松龙（PSL）治疗，但在PSL减量后胸腔积液仍增多。包括胸膜组织病理学检查在内的进一步检查确诊为IgG4相关性疾病伴双侧乳糜胸，胸导管无任何渗漏。大剂量PSL联合利妥昔单抗的联合治疗成功减少了胸腔积液。这是一例非常罕见的IgG4相关性胸膜炎合并乳糜胸病例，也是首例使用利妥昔单抗成功治疗的报告。
