肺泡蛋白沉积症的诊治及长期随访

Diagnosis,Treatment,and Long-term Follow-up of Patients with Pulmonary Alveolar Proteinosis

目的探讨肺泡蛋白沉积症(PAP)的临床表现、诊断、治疗及长期疗效。方法选取2000年1月至2014年12月在北京大学第一医院诊治的PAP患者11例,所有患者均采用纤维支气管镜检查,经支气管肺泡灌洗和(或)支气管镜肺活检确诊,具有完整的病例资料。结果 11例PAP患者均为成人,平均确诊周期达14.5个月,仅2例患者没有误诊。临床主要表现为活动后气促及咳嗽,双肺斑片状或大片状磨玻璃样阴影。患者存在不同程度低氧血症及呼吸衰竭9例(81.8%),肺功能表现为不同程度的限制性通气功能障碍伴弥散障碍。其中9例患者诊断为原发性PAP,2例患者诊断为继发性PAP。8例(72.7%)患者至少接受了1次全肺灌洗治疗,1例继发性PAP患者在根治原发病后PAP缓解。长期随访的10例患者中,仅1例因晚期乳癌病故,其余患者日常生活无呼吸困难,胸部影像学、血气分析及肺功能稳定。结论全肺灌洗是原发性PAP患者的主要治疗方法,预后良好。继发性PAP以原发病治疗或去除诱发因素治疗为主,诊断PAP时应注意有无继发因素。

Objective To describe the clinical characteristics of pulmonary alveolar proteinosis （PAP） , evaluate the methods of diagnosis and treatment, and analyze treatment outcomes and prognosis. Methods Data were accumulated from a cohort of 11 patients with PAP between 2000 and 2014. All the patients were examined by fiber bronchoscopy and confirmed by bronchoalveolar lavage and/or transbronchial lung biopsy. Their medical records were complete. Results In the present study, all the patients were adults. The mean confirmation duration of the disease was 14.5 months, and only two patients were not misdiagnosed. The main clinical manifestations were shortness of breath and cough after activities and flake or ground glass opacities affecting both lungs. Nine （ 81.8% ） patients had hyoxemia of different degrees and respiratory failure, and the examination of their lung functions showed restrictive ventilatory dysfunction complicated by diffusion impairment. Nine patients were diagnosed as primary PAP and two as secondary PAP. Eight patients underwent whole lung lavage at least once. One patient with secondary PAP was improved after radical treatment of underlying diseases. Among 10 patients during long-term follow-up, one was died of advanced breast cancer, and the rest were stable in chest imaging, blood gas analysis and lung functions and showed no dyspnea in dally life. Conclusion Whole lung lavage is the standard treatment for primary PAP, with good prognoses. The treatment for secondary PAP focuses on the therapy used for underlying diseases and eliminating inducing factors. Clinicians should pay attention to the presence of secondary factors when treating PAP.