Motamedi Melika, Ferrara Giovanni, Yacyshyn Elaine, Osman Mohammed, Abril Andy, Rahman Samia, Netchiporouk Elena, Gniadecki Robert
Division of Dermatology, University of Alberta, Edmonton, Alberta, Canada.
Division of Pulmonary Medicine, University of Alberta, Edmonton, Alberta, Canada.
J Am Acad Dermatol. 2023 Apr;88(4):751-764. doi: 10.1016/j.jaad.2022.10.001. Epub 2022 Oct 11.
Numerous inflammatory, neoplastic, and genetic skin disorders are associated with interstitial lung disease (ILD), the fibrosing inflammation of lung parenchyma that has significant morbidity and mortality. Therefore, the dermatologist plays a major role in the early detection and appropriate referral of patients at risk for ILD. Part 1 of this 2-part CME outlines the pathophysiology of ILD and focuses on clinical screening and therapeutic principles applicable to dermatological patients who are at risk for ILD. Patients with clinical symptoms of ILD should be screened with pulmonary function tests and high-resolution chest computed tomography. Screening for pulmonary hypertension should be considered in high-risk patients. Early identification and elimination of pulmonary risk factors, including smoking and gastroesophageal reflux disease, are essential in improving respiratory outcomes. First-line treatment interventions for ILD in a dermatological setting include mycophenolate mofetil, but the choice of therapeutic agents depends on the nature of the primary disease, the severity of ILD, and comorbidities and should be the result of a multidisciplinary assessment. Better awareness of ILD among medical dermatologists and close interdisciplinary collaborations are likely to prevent treatment delays improving long-term outcomes.
许多炎症性、肿瘤性和遗传性皮肤病都与间质性肺疾病(ILD)相关,ILD是一种肺实质的纤维化炎症,具有较高的发病率和死亡率。因此,皮肤科医生在早期发现以及对有ILD风险的患者进行适当转诊方面起着主要作用。这个两部分的继续医学教育(CME)的第1部分概述了ILD的病理生理学,并重点关注适用于有ILD风险的皮肤科患者的临床筛查和治疗原则。有ILD临床症状的患者应通过肺功能测试和高分辨率胸部计算机断层扫描进行筛查。高危患者应考虑筛查肺动脉高压。早期识别和消除肺部危险因素,包括吸烟和胃食管反流病,对改善呼吸结局至关重要。皮肤科环境中ILD的一线治疗干预措施包括霉酚酸酯,但治疗药物的选择取决于原发性疾病的性质、ILD的严重程度和合并症,应该是多学科评估的结果。皮肤科医生对ILD有更好的认识以及密切的跨学科合作可能会防止治疗延误,改善长期结局。