Lefebvre C, Tousignant J, Chartier S, Demers D
Service de Dermatologie, Centre Hospitalier Universitaire Notre-Dame, Montréal, P. Québec, Canada.
Ann Dermatol Venereol. 1993;120(4):293-5.
The association of cancer and connective tissue disease is well known, the most frequent being certainly with dermatomyositis. The association cancer and PSS is more controversial. The incidence of neoplasia in that group seems to be comparable with the general population but the proportion of certain types of cancer is different, and the temporal relationship with the apparition of symptoms of PSS is stunning. The hypothesis actually in favor is an imbalance of the immune system, which cause the diminution of the immune surveillance and the apparition of cancer and a concomitant dysregulation of the system, causing the fibrosis of the PSS. We describe a 75 year-old white female who developed a colic adenocarcinoma; she also had, concomitantly, a systemic scleroderma, with sclerodactyly and pulmonary fibrosis. The patient corresponded to the criteria of the American Rheumatism Association for progressive systemic scleroderma (PSS). The prognosis of patients with PSS depends on their systemic involvement but also, we believe, in the more aged group, on the apparition of a neoplasia.
癌症与结缔组织病之间的关联众所周知，其中最常见的无疑是与皮肌炎的关联。癌症与系统性硬化症（PSS）的关联则更具争议性。该组中肿瘤形成的发生率似乎与普通人群相当，但某些类型癌症的比例有所不同，并且与PSS症状出现的时间关系令人惊讶。目前受到支持的假说是免疫系统失衡，这导致免疫监视功能减弱以及癌症出现，同时系统失调导致PSS的纤维化。我们描述了一名75岁的白人女性，她患了结肠腺癌；同时，她还患有系统性硬化症，伴有指（趾）硬皮病和肺纤维化。该患者符合美国风湿病协会关于进行性系统性硬化症（PSS）的标准。PSS患者的预后取决于其全身受累情况，但我们认为，在年龄较大的人群中，还取决于肿瘤的出现。
