Sakamoto Noriho, Ishimatsu Yuji, Kakugawa Tomoyuki, Hara Atsuko, Hara Shintaro, Amenomori Misato, Fujita Hanako, Mukae Hiroshi, Kohno Shigeru
The Second Department of Internal Medicine, Nagasaki University School of Medicine.
Intern Med. 2010;49(15):1609-11. doi: 10.2169/internalmedicine.49.3457. Epub 2010 Aug 2.
A 73-year-old woman who had been diagnosed with systemic sclerosis was admitted for further examination of bilateral hilar lymphadenopathy. Sarcoidosis was confirmed based on elevated serum levels of angiotensin-converting enzyme, a high proportion of lymphocytes and a high CD4/CD8 ratio in bronchoalveolar lavage fluid, abnormal (67)Gallium uptake in the mediastinum and noncaseating granulomas in skin biopsy specimens. In addition, high levels of antimitochondrial M2 antibodies and alkaline phosphatase indicated primary biliary cirrhosis (PBC). Here we describe a rare triplex of sarcoidosis, SSc and PBC. Although the etiology of this complex remains unknown, these three diseases might share some pathogenesis.
一名73岁被诊断为系统性硬化症的女性因双侧肺门淋巴结病接受进一步检查。根据血清血管紧张素转换酶水平升高、支气管肺泡灌洗液中淋巴细胞比例高和CD4/CD8比值高、纵隔镓摄取异常（67Ga）以及皮肤活检标本中的非干酪样肉芽肿，确诊为结节病。此外，抗线粒体M2抗体和碱性磷酸酶水平高提示原发性胆汁性肝硬化（PBC）。在此，我们描述了一种罕见的结节病、系统性硬化症和原发性胆汁性肝硬化三联征。尽管这种综合征的病因尚不清楚，但这三种疾病可能有一些共同的发病机制。
