Khan A, Agarwal R, Aggarwal A N, Bal Amanjit, Sen Indu, Yaddanapuddi L N, Puri G D
Department of Pulmonary Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Indian J Chest Dis Allied Sci. 2012 Apr-Jun;54(2):91-7.
Pulmonary alveolar proteinosis (PAP) is a disorder characterised by accumulation of lipids and proteins in the alveoli, with the resultant symptoms ranging from indolent subclinical disease to progressive respiratory failure.
We retrospectively studied five patients with PAP managed at our center between January 2007 and April 2010, with whole lung lavage (WLL) and/or subcutaneous granulocyte macrophage-colony stimulating factor (GM-CSF) therapy. Patients undergoing WLL under general anaesthesia were supplemented with three months of GM-CSF therapy. Pre- and post-lavage symptom assessment was performed with a 10-point, symptom-based visual analogue scale.
Their mean age was 37.6-7.0 years; there were four males. Diagnosis of PAP [idiopathic (n=3); secondary to Nocardia (n=1)] was established by surgical lung biopsy in four patients who presented with respiratory failure. Three patients with idiopathic PAP (n=3) were treated with a combination of GM-CSF and WLL; one patient with secondary PAP was treated with antibiotics alone. In another patient transbronchial lung biopsy was used to diagnose PAP and GM-CSF alone was administered. All patients were followed up for a median period of two years (range 0.5-3 years). Significant improvement was achieved in all the patients with therapeutic WLL and/or GM-CSF.
Whole lung lavage appeared to be an effective and safe therapy in patients with PAP. Efficacy of simultaneous administration of GM-CSF and WLL in the treatment of PAP merits further study.
肺泡蛋白沉积症（PAP）是一种以肺泡内脂质和蛋白质积聚为特征的疾病，其症状从隐匿的亚临床疾病到进行性呼吸衰竭不等。
我们回顾性研究了2007年1月至2010年4月在我们中心接受治疗的5例PAP患者，采用全肺灌洗（WLL）和/或皮下注射粒细胞巨噬细胞集落刺激因子（GM-CSF）治疗。在全身麻醉下接受WLL的患者补充3个月的GM-CSF治疗。灌洗前后的症状评估采用基于症状的10分视觉模拟量表进行。
他们的平均年龄为37.6±7.0岁；有4名男性。4例出现呼吸衰竭的患者通过手术肺活检确诊为PAP[特发性（n=3）；继发于诺卡菌（n=1）]。3例特发性PAP患者（n=3）接受GM-CSF和WLL联合治疗；1例继发性PAP患者仅接受抗生素治疗。另一例患者经支气管肺活检诊断为PAP，仅给予GM-CSF治疗。所有患者的中位随访期为两年（范围0.5 - 3年）。所有接受治疗性WLL和/或GM-CSF的患者均取得了显著改善。
全肺灌洗似乎是PAP患者一种有效且安全的治疗方法。GM-CSF与WLL联合应用治疗PAP的疗效值得进一步研究。
