Antoniou K M, Margaritopoulos G, Economidou F, Siafakas N M
Dept. of Thoracic Medicine, Medical School, University of Crete, Heraklion, Greece.
Eur Respir J. 2009 Apr;33(4):882-96. doi: 10.1183/09031936.00152607.
The connective tissue disorders (CTDs), also called collagen vascular diseases (CVDs), represent a heterogeneous group of immunologically mediated inflammatory disorders with a large variety of affected organs. Individuals with a CTD (rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, Sjögren's syndrome, polymyositis/dermatomyositis and mixed connective tissue disease) are susceptible to respiratory involvement. When the lungs are affected, an increasing mortality and morbidity in CVDs occurs. Interstitial lung disease (ILD) is established as a clinical corollary across the spectrum of CTDs, with an overall incidence estimated at 15%. Therefore, pivotal clinical dilemmas remain in the evaluation and management of ILD involvement in CVDs. Critical questions are the presence of fibrosis and whether the disease is clinically significant. Moreover, the clinician has to decide if treatment is warranted and which is the best therapeutic approach. The use of additional tests, such as pulmonary function tests, high-resolution computed tomography scan, bronchoalveolar lavage fluid and surgical lung biopsy, deserves better discussion. The present review focuses on establishing the diagnosis of ILD in CTD, and on evaluating disease activity and prognosis. This will provide the basis for therapeutic decisions that will be discussed, including an overview of recent advances.
结缔组织病（CTD），也称为胶原血管病（CVD），是一组异质性的免疫介导的炎症性疾病，累及多种器官。患有CTD（类风湿性关节炎、系统性红斑狼疮、系统性硬化症、干燥综合征、多发性肌炎/皮肌炎和混合性结缔组织病）的个体易发生呼吸系统受累。当肺部受到影响时，CVD的死亡率和发病率会增加。间质性肺病（ILD）在各种CTD中均被确认为临床必然结果，总体发病率估计为15%。因此，在评估和管理CVD中ILD的受累情况时，关键的临床难题仍然存在。关键问题是纤维化的存在以及该疾病在临床上是否具有重要意义。此外，临床医生必须决定是否需要治疗以及哪种是最佳治疗方法。对于使用其他检查，如肺功能测试、高分辨率计算机断层扫描、支气管肺泡灌洗和外科肺活检，值得进行更深入的讨论。本综述重点在于确定CTD中ILD的诊断，以及评估疾病活动度和预后。这将为后续讨论的治疗决策提供依据，包括近期进展的概述。
