Castellana Giuseppe, Castellana Roberto, Fanelli Carmela, Lamorgese Vito, Florio Carlo
Unità Operativa di Pneumologia, ASL BA/5, Putignano (Bari).
Radiol Med. 2003 Sep;106(3):160-8.
Pulmonary alveolar microlithiasis (PAM) is a rare chronic disease of unknown etiology and pathogenesis. The disease is characterized by the diffuse presence in the alveoli of minute calcific deposits known as microliths. In most cases patients have mild clinical symptoms, contrasting with the severe radiographic appearance: this is a typical feature that should raise the suspicion of PAM. The first to describe the clinical and radiographic aspects of the disease, as well as the first case series, was the radiologist Sosman. In recent years, high resolution computed tomography (HRCT) has made it possible to define the extent and severity of the disease more precisely, and has demonstrated calcifications in anatomical sites that could not be shown by conventional radiology. The present paper describes the radiological evolution of the disease, and suggests a classification based on the radiographic and HRCT follow-up of the three clinical cases: two young patients followed up for 24 and 11 years and one elderly man who is still alive and is the PAM case with the longest survival since diagnosis, over 50 years, to be reported in the international literature.
肺泡微石症(PAM)是一种病因和发病机制不明的罕见慢性疾病。该疾病的特征是在肺泡中弥漫存在称为微石的微小钙化沉积物。在大多数情况下,患者临床症状较轻,与严重的影像学表现形成对比:这是一个应引起对PAM怀疑的典型特征。放射科医生索斯曼首次描述了该疾病的临床和影像学方面以及首个病例系列。近年来,高分辨率计算机断层扫描(HRCT)使更精确地确定疾病的范围和严重程度成为可能,并显示出传统放射学无法显示的解剖部位的钙化。本文描述了该疾病的放射学演变,并基于对三例临床病例的影像学和HRCT随访提出了一种分类方法:两名年轻患者分别随访了24年和11年,一名老年男性仍然健在,是国际文献中报道的自诊断以来存活时间最长(超过50年)的PAM病例。