Sajadi Yasaman, Mouodi Simin, Chogan Ehsan, Monadi Mahmood
Clinical Research Development Unit of Rouhani Hospital, Rouhani Hospital, Babol University of Medical Sciences, Babol, Iran.
Social Determinants of Health Research Center, Health Research Institute, Babol University of Medical Sciences Babol, Iran.
Caspian J Intern Med. 2025 Apr 1;16(2):362-368. doi: 10.22088/cjim.16.2.362. eCollection 2025 Spring.
Pulmonary alveolar proteinosis (PAP) is a rare disease caused by the accumulation of surfactant in the lung's alveoli, as a result of malfunction in the cleaning function of alveolar macrophages. The major symptoms include cough and dyspnea. Computed tomography scan usually reveals crazy-paving pattern. Lung biopsy confirms the diagnosis by showing accumulations of periodic acid-schiff-positive lipoproteinaceous materials.
In this report, we present a middle-age man with progressive dyspnea on exertion, and frequent cough with no noteworthy medical history. The results of initial examinations and laboratory tests were non-diagnostic, so imaging studies were requested for the patient. After imaging and suspecting PAP as a differential diagnosis due to results of high resolution computed tomography, a transbronchial biopsy was performed to confirm the diagnosis. Then, according to the histopathology reports, the diagnosis of PAP was made. During the biopsy procedure, the patient developed pneumothorax, and accurate treatment approaches were considered for this complication.
In patients with chronic progressive dyspnea and cough, appropriate and timely imaging and other paraclinic investigations must be considered by the physicians.
肺泡蛋白沉积症（PAP）是一种罕见疾病，由于肺泡巨噬细胞清洁功能故障，导致表面活性剂在肺的肺泡中积聚。主要症状包括咳嗽和呼吸困难。计算机断层扫描通常显示铺路石样图案。肺活检通过显示过碘酸-希夫阳性脂蛋白物质的积聚来确诊。
在本报告中，我们介绍了一名中年男性，有进行性劳力性呼吸困难和频繁咳嗽，无显著病史。初步检查和实验室检查结果无诊断意义，因此对该患者进行了影像学检查。在影像学检查后，由于高分辨率计算机断层扫描结果怀疑PAP为鉴别诊断，遂进行经支气管活检以确诊。然后，根据组织病理学报告，作出了PAP的诊断。在活检过程中，患者发生气胸，并针对该并发症考虑了准确的治疗方法。
对于有慢性进行性呼吸困难和咳嗽的患者，医生必须考虑进行适当且及时的影像学检查和其他临床旁检查。
