Caparros D, Steenhouwer F, Marquette C H, Carpentier F, Foulet A
Département de Pneumologie, Hôpital V.-Provo, Roubaix.
Rev Mal Respir. 1994;11(1):63-6.
Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the accumulation of lipoproteinaceous material within the alveoli. Though many studies addressed biochemical data in bronchoalveolar lavage (BAL) effluent, poor attention was payed to cell profile in PAP. We report the case of a 40 year old woman, presenting with progressive respiratory failure and bilateral pulmonary infiltrates. BAL and transbronchial biopsy confirmed PAP. Therapeutic whole lung lavage led to full clinical and radiological remission. Sequential BAL follow-up was started over a 4-year period. As previously reported, lymphocytes were increased prior to whole lung lavage. This pattern subsequently remained, whereas recurrence or superinfections did not occur. The mechanism of this disorder is unclear and deserves further investigation.
肺泡蛋白沉积症(PAP)是一种罕见疾病,其特征是肺泡内蓄积脂蛋白样物质。尽管许多研究涉及支气管肺泡灌洗(BAL)流出液中的生化数据,但对PAP患者的细胞特征关注较少。我们报告一例40岁女性病例,该患者表现为进行性呼吸衰竭和双侧肺部浸润。BAL和经支气管活检确诊为PAP。治疗性全肺灌洗导致临床和影像学完全缓解。在4年期间开始进行序贯BAL随访。如先前报道,全肺灌洗前淋巴细胞增多。这种模式随后持续存在,而未发生复发或重叠感染。这种疾病的机制尚不清楚,值得进一步研究。