Tolle Leslie B
Department of Pulmonary Medicine and Critical Care Medicine, Cleveland Clinic, Cleveland, OH, USA.
Case Rep Pulmonol. 2022 Feb 15;2022:9942432. doi: 10.1155/2022/9942432. eCollection 2022.
Interstitial lung diseases (ILDs) are heterogeneous in their clinical presentation. Making a differential diagnosis of ILD requires a thorough medical history, clinical examination, serologies, high-resolution computed tomography (CT) scan, and, in some cases, bronchoalveolar lavage or surgical lung biopsy. Multidisciplinary discussion is recommended to improve diagnostic confidence. ILDs have a variable and unpredictable clinical course. Patients should be closely monitored to ensure that progression of ILD is detected promptly. This involves regular assessment of symptoms, lung function, and, where appropriate, high-resolution CT. Patients with some fibrosing ILDs may respond well to immunosuppressants, but even patients who respond well to immunosuppressants initially may later show deterioration despite appropriate management. The tyrosine kinase inhibitor nintedanib has been approved for the treatment of idiopathic pulmonary fibrosis, other chronic fibrosing ILDs with a progressive phenotype, and systemic sclerosis-associated ILD. The three case studies described in this article illustrate the challenges in the diagnosis and management of patients with fibrosing ILDs and the importance of taking a multidisciplinary and individualized approach to care, including regular monitoring and consideration of whether a patient's drug regimen needs to be changed when there is evidence of disease progression.
间质性肺疾病（ILDs）在临床表现上具有异质性。对ILDs进行鉴别诊断需要全面的病史、临床检查、血清学检查、高分辨率计算机断层扫描（CT），在某些情况下还需要进行支气管肺泡灌洗或外科肺活检。建议进行多学科讨论以提高诊断的可信度。ILDs具有多变且不可预测的临床病程。应密切监测患者，以确保及时发现ILDs的进展。这包括定期评估症状、肺功能，并在适当情况下进行高分辨率CT检查。一些纤维化ILDs患者可能对免疫抑制剂反应良好，但即使最初对免疫抑制剂反应良好的患者，尽管进行了适当的管理，后期仍可能出现病情恶化。酪氨酸激酶抑制剂尼达尼布已被批准用于治疗特发性肺纤维化、其他具有进行性表型的慢性纤维化ILDs以及系统性硬化症相关的ILD。本文所述的三个病例研究说明了纤维化ILDs患者诊断和管理中的挑战，以及采取多学科和个体化护理方法的重要性，包括定期监测以及当有疾病进展证据时考虑是否需要改变患者的药物治疗方案。
