Motegi M, Suzuki Y, Takayanagi N, Sando Y, Ubukata M, Aihara T, Imai S, Suzuki T
Second Department of Internal Medicine, Gunma University School of Medicine, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1994 Oct;32(10):1016-21.
A 58-year-old female, who had complained of dryness of the conjunctiva and mouth for nine years, was admitted to our hospital because chest X-ray films revealed increases in both the size and the number of pulmonary nodules in comparison to four years earlier. Histological findings of the salivary gland biopsy confirmed a diagnosis of Sjögren's syndrome. An open lung biopsy was performed. Histologically, the pulmonary nodules consisted of homogeneous, acellular eosinophilic materials, which were identified as amyloid by Congo red staining. Immunohistochemical examination showed the amyloid to be composed of AA protein. There were no deposits in other organs. Peribronchial lymphocytic infiltration associated with Sjögren's syndrome was present. Nodular pulmonary amyloidosis with Sjögren's syndrome is very rare condition. To our knowledge, this case is only the ninth to be reported in the literature.
一名58岁女性,主诉结膜和口腔干燥9年,因胸部X线片显示肺结节的大小和数量较4年前增加而入住我院。唾液腺活检的组织学结果确诊为干燥综合征。进行了开放性肺活检。组织学上,肺结节由均匀的无细胞嗜酸性物质组成,经刚果红染色鉴定为淀粉样蛋白。免疫组织化学检查显示淀粉样蛋白由AA蛋白组成。其他器官无沉积。存在与干燥综合征相关的支气管周围淋巴细胞浸润。合并干燥综合征的结节性肺淀粉样变性是一种非常罕见的疾病。据我们所知,该病例是文献中报道的第九例。