Abstract：
While idiopathic interstitial pneumonia (IIP) centering on idiopathic pulmonary fibrosis (IPF) is the most prevalent interstitial lung disease (ILD), especially in the older adult population, connective tissue disease (CTD)-related ILD is the second most prevalent ILD. The pathogenesis of IPF is primarily fibrosis, whereas that of other ILDs, particularly CTD-ILD, is mainly inflammation. Therefore, a precise diagnosis is crucial for selecting appropriate treatments, such as antifibrotic or immunosuppressive agents. In addition, some patients with IIP have CTD-related features, such as arthritis and skin eruption, but do not meet the criteria for any CTD, this is referred to as interstitial pneumonia with autoimmune features (IPAF). IPAF is closely associated with idiopathic nonspecific interstitial pneumonia (iNSIP) and cryptogenic organizing pneumonia (COP). Furthermore, patients with iNSIP or those with NSIP with OP overlap frequently develop polymyositis/dermatomyositis after the diagnosis of IIP. Acute exacerbation of ILD, the most common cause of death, occurs more frequently in patients with IPF than in those with other ILDs. Although acute exacerbation of CTD-ILD occurs at a low rate of incidence, patients with rheumatoid arthritis, microscopic polyangiitis, or systemic sclerosis experience more acute exacerbation of CTD-ILD than those with other CTD. In this review, the features of each IIP, focusing on CTD-related signatures, are summarized, and the pathogenesis and appropriate treatments to improve the prognoses of patients with various ILDs are discussed.
摘要：
虽然以特发性肺纤维化（IPF）为中心的特发性间质性肺炎（IIP）是最常见的间质性肺病（ILD），尤其是在老年人口，结缔组织病(CTD)相关的ILD是第二普遍的ILD。IPF的发病机制主要是纤维化,而其他ILD，特别是CTD-ILD，主要是炎症。因此,准确的诊断对于选择合适的治疗方法至关重要，如抗纤维化或免疫抑制剂。此外,一些IIP患者具有CTD相关特征，比如关节炎和皮肤出疹，但不符合任何CTD的标准，这被称为具有自身免疫特征的间质性肺炎(IPAF).IPAF与特发性非特异性间质性肺炎（iNSIP）和隐源性机化性肺炎（COP）密切相关。此外，iNSIP或NSIP伴OP重叠的患者在诊断IIP后经常发生多发性肌炎/皮肌炎.ILD急性加重，最常见的死因,IPF患者的发病率高于其他ILD患者。尽管CTD-ILD的急性加重发生率较低，类风湿性关节炎患者，显微镜下多血管炎，与其他CTD相比，系统性硬化症或CTD-ILD的急性加重。在这次审查中,每个IIP的特征，专注于CTD相关的签名，总结，并讨论了各种ILD患者的发病机制和改善预后的适当治疗方法。
