Brillet P Y, Mama N, Nunes H, Uzunhan Y, Abbad S, Brauner M W
Service de Radiologie, Hôpital Avicenne, 125, route de Stalingrad, 93009 Bobigny cedex, France.
J Radiol. 2009 Nov;90(11 Pt 2):1854-68. doi: 10.1016/s0221-0363(09)73288-3.
Connective tissue disorders correspond to a heterogeneous group of inflammatory diseases characterized by abnormal immune system activity leading to connective tissue alterations in multiple parts of the body. In adults, connective tissue disorders include rheumatoid arthritis, progressive systemic sclerosis, Sjögren syndrome, systemic lupus erythematosus, dermatomyositis and polymyositis, ankylosing spondylitis, and mixed connective tissue disease. Broncho-pulmonary involvement may be variable with involvement of all anatomical components of the lung. Involvement of other intrathoracic structures (pleura, respiratory muscles, heart, rib cage) is frequent. The most specific manifestations include interstitial lung diseases and pulmonary hypertension. During follow-up, progressive respiratory diseases may occur due to the treatment, infections, pulmonary embolism or neoplasms.
结缔组织疾病是一组异质性炎症性疾病，其特征是免疫系统活动异常，导致身体多个部位的结缔组织发生改变。在成年人中，结缔组织疾病包括类风湿性关节炎、进行性系统性硬化症、干燥综合征、系统性红斑狼疮、皮肌炎和多发性肌炎、强直性脊柱炎以及混合性结缔组织病。支气管 - 肺部受累情况可能各不相同，肺部所有解剖结构均可受累。其他胸内结构（胸膜、呼吸肌、心脏、胸廓）受累也很常见。最具特异性的表现包括间质性肺疾病和肺动脉高压。在随访期间，由于治疗、感染、肺栓塞或肿瘤，可能会发生进行性呼吸系统疾病。
