Yanagisawa Atsushi, Konaka Hachiro, Tanaka Masaki, Ihara Shoichi, Tachibana Isao
Department of Respiratory Medicine and Clinical Immunology, Nippon Life Hospital, Osaka, JPN.
Department of Respiratory Medicine and Clinical Immunology, Osaka University, Osaka, JPN.
Cureus. 2024 Nov 28;16(11):e74669. doi: 10.7759/cureus.74669. eCollection 2024 Nov.
Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the accumulation of surfactants in the alveoli. It has been suggested that immunosuppressants contribute to the development and exacerbation of PAP. Here, we report the case of a 73-year-old man who developed secondary PAP after intensive immunosuppressive treatment for acute exacerbation of interstitial pneumonia (IP). In this case, despite the improvement of the inflammatory response after immunosuppressive treatment, Krebs von den Lungen-6 (KL-6) continued to increase, leading to the diagnosis of PAP. De-escalation of immunosuppressive treatment improved the PAP, allowing him to be discharged from the hospital. Although KL-6 is a useful marker of IP, when IP appears to be refractory and KL-6 increases despite the improvement of other inflammatory markers, physicians should consider the development of PAP and perform proactive bronchoscopic evaluation.
肺泡蛋白沉积症（PAP）是一种罕见疾病，其特征为肺泡内表面活性物质积聚。有观点认为免疫抑制剂会促使PAP的发生和加重。在此，我们报告一例73岁男性病例，该患者在因间质性肺炎（IP）急性加重接受强化免疫抑制治疗后发生了继发性PAP。在该病例中，尽管免疫抑制治疗后炎症反应有所改善，但克雷布斯冯登卢肯-6（KL-6）持续升高，从而确诊为PAP。免疫抑制治疗的降阶梯改善了PAP，使他得以出院。尽管KL-6是IP的一个有用标志物，但当IP似乎难以治愈且尽管其他炎症标志物有所改善但KL-6仍升高时，医生应考虑PAP的发生并积极进行支气管镜评估。
