美国胸科学会指南推荐进行呼吸困难评分、FVC、肺容积、肺弥散量测定、动脉血气分析、心肺运动试验用于测定气体交换,和(或)每3~6个月进行HRCT检查。上述检查并不总是具有可操作性。[1]American Thoracic Society; European Respiratory Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. Am J Respir Crit Care Med. 2000;161:646-664.http://www.atsjournals.org/doi/full/10.1164/ajrccm.161.2.ats3-00#.UmEPiNglgZkhttp://www.ncbi.nlm.nih.gov/pubmed/10673212?tool=bestpractice.com简单且廉价的6分钟步行试验,可以提供与心肺运动试验相关的疾病进展的资料。[112]Flaherty KR, Andrei AC, Murray S, et al. Idiopathic pulmonary fibrosis: prognostic value of changes in physiology and six-minute-walk test. Am J Respir Crit Care Med. 2006;174:803-809.http://www.atsjournals.org/doi/full/10.1164/rccm.200604-488OC#.UmEQmtglgZkhttp://www.ncbi.nlm.nih.gov/pubmed/16825656?tool=bestpractice.com[113]Eaton T, Young P, Milne D, et al. Six-minute walk, maximal exercise tests: reproducibility in fibrotic interstitial pneumonia. Am J Respir Crit Care Med. 2005;171:1150-1157.http://www.atsjournals.org/doi/full/10.1164/rccm.200405-578OC#.UmEP_tglgZkhttp://www.ncbi.nlm.nih.gov/pubmed/15640367?tool=bestpractice.com连续的肺量测定法也非常易于操作,且可以提供有用的预后信息。[92]Collard HR, King TE Jr, Bartelson BB, et al. Changes in clinical and physiologic variables predict survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2003;168:538-542.http://www.atsjournals.org/doi/full/10.1164/rccm.200211-1311OC#.UmERpNglgZkhttp://www.ncbi.nlm.nih.gov/pubmed/12773325?tool=bestpractice.com[94]Flaherty KR, Mumford JA, Murray S, et al. Prognostic implications of physiologic and radiographic changes in idiopathic interstitial pneumonia. Am J Respir Crit Care Med. 2003;168:543-548.http://www.atsjournals.org/doi/full/10.1164/rccm.200209-1112OC#.UmERTNglgZkhttp://www.ncbi.nlm.nih.gov/pubmed/12773329?tool=bestpractice.com[114]Hanson D, Winterbauer RH, Kirtland SH, et al. Changes in pulmonary function test results after 1 year of therapy as predictors of survival in patients with idiopathic pulmonary fibrosis. Chest. 1995;108:305-310.http://www.ncbi.nlm.nih.gov/pubmed/7634857?tool=bestpractice.com[115]du Bois RM, Weycker D, Albera C, et al. Forced vital capacity in patients with idiopathic pulmonary fibrosis: test properties and minimal clinically important difference. Am J Respir Crit Care Med. 2011;184:1382-1389.http://www.atsjournals.org/doi/full/10.1164/rccm.201105-0840OC#.UmESStglgZkhttp://www.ncbi.nlm.nih.gov/pubmed/21940789?tool=bestpractice.com因此,患者每6个月进行随访,做6分钟步行试验,以及进行肺量计系列测定是较为合理的。临床情况变化或者出现新的症状时,需要增加测定频率。
此外,如果患者给予药物治疗,监测应该包括治疗相关不良反应的监测。尽管给予治疗,患者病情仍然进展,肺功能下降,或者出现明显药物毒性损害的患者,治疗药物应当减量和停用。尽管患者对药物治疗的主观感受很重要,考虑到糖皮质激素可能引起患者轻度兴奋,这不应该作为继续治疗的唯一的依据。如果持续应用中-高剂量的糖皮质激素,应该考虑预防治疗耶氏肺孢子虫感染(例如,甲氧苄啶/磺胺甲恶唑)。 这些患者也应该进行骨密度测定,检查是否患有骨质疏松,可以适当补充钙和维生素D,以及双膦酸盐。[116]Fardet L, Kassar A, Cabane J, et al. Corticosteroid-induced adverse events in adults: frequency, screening and prevention. Drug Saf. 2007;30:861-881.http://www.ncbi.nlm.nih.gov/pubmed/17867724?tool=bestpractice.com