显示间质性肺疾病(ILD)特征的结缔组织疾病(CTD)包括系统性红斑狼疮(SLE),类风湿性关节炎(RA),系统性硬化症(SSc),皮肌炎(DM)和多发性肌炎(PM),强直性脊柱炎(AS),干燥综合征(SS),和混合性结缔组织病(MCTD)。关于CTD相关ILD(CTD-ILD)的肺活检的组织病理学,多部门参与是一个重要线索,当存在时,应将CTD置于病因鉴别诊断的首位。不同的组织学模式,包括非特异性间质性肺炎(NSIP),普通间质性肺炎(UIP),机化肺炎,根尖纤维化,弥漫性肺泡损伤,在CTD-ILD患者的组织学上可以看到淋巴样间质性肺炎。尽管ILD的比例各不相同,NSIP模式占很大比例,尤其是在SSc中,DM和/或PM和MCTD,其次是UIP模式。在RA患者中,据报道,间质性肺异常(ILA)发生在大约20-60%的个体中,其中35-45%将具有CT异常的进展。胸膜下分布和更大的基线ILA受累是与疾病进展相关的危险因素。无症状的CTD-ILD或ILA患者肺功能正常且没有疾病进展的证据,无需治疗即可随访。用于症状性和/或纤维化CTD-ILD的免疫抑制或抗纤维化药物可用于需要治疗的患者。
Connective tissue diseases (CTDs) demonstrating features of interstitial lung disease (ILD) include systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), systemic sclerosis (SSc), dermatomyositis (DM) and polymyositis (PM), ankylosing spondylitis (AS), Sjogren syndrome (SS), and mixed connective tissue disease (MCTD). On histopathology of lung biopsy in CTD-related ILDs (CTD-ILDs), multi-compartment involvement is an important clue, and when present, should bring CTD to the top of the list of etiologic differential diagnoses. Diverse histologic patterns including nonspecific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), organizing pneumonia, apical fibrosis, diffuse alveolar damage, and lymphoid interstitial pneumonia can be seen on histology in patients with CTD-ILDs. Although proportions of ILDs vary, the NSIP pattern accounts for a large proportion, especially in SSc, DM and/or PM and MCTD, followed by the UIP pattern. In RA patients, interstitial lung abnormality (ILA) is reported to occur in approximately 20-60% of individuals of which 35-45% will have progression of the CT abnormality. Subpleural distribution and greater baseline ILA involvement are risk factors associated with disease progression. Asymptomatic CTD-ILDs or ILA patients with normal lung function and without evidence of disease progression can be followed without treatment. Immunosuppressive or antifibrotic agents for symptomatic and/or fibrosing CTD-ILDs can be used in patients who require treatment.