A 31-year-old female from a non-consanguineous family presented to PUMCH in April 2015 due to an abnormal appearance on a chest X-ray observed during a medical examination 1 year prior. The patient did not present with any respiratory symptoms such as dyspnea on exertion or a cough. Physical examination revealed decreased pulmonary sounds in the lower zones and P2>A2 was heard during heart auscultation. The HRCT revealed diffuse, scattered, hyperdense micronodules throughout the whole lungs, accompanied by interlobular septal and bilateral pleural calcific thickening, subpleural cysts between the ribs and calcified parenchyma, referred to as the 'black pleura' sign (Fig. 2A and B). The BAFL examination (performed as above) revealed that the classification of inflammatory cells was normal and the ratio of cluster of differentiation (CD)4+/CD8+ T cells was 0.9 (reference range, 0.9-2.0), but a calcified body was detected (Fig. 2C). ABG measured pH 7.378, PaCO2 37.0 mmHg, PaO2 88.1 mmHg, SO2 96.4% and HCO3 22 mmol/l of room air (data not shown). Lung tissue pathology and SLC34A2 gene testing were lacking as a positive diagnosis of PMA had been reached based on the tests performed making invasive surgery unnecessary Gene testing was not performed on this patient due to economic constraints. A 99mTc-MDP bone scan revealed a diffusely increased intake of radiation in the lungs. An echocardiograph revealed mild enlargement of the right ventricle (anteroposterior diameter, 32 mm), with mild pulmonary hypertension (pulmonary systolic pressure, 37 mmHg) (data not shown). PFT and routine blood biochemistry were normal (data not shown). In the patient's local hospital they were misdiagnosed with pulmonary tuberculosis (TB) due to the appearance of miliary nodules on HRCT imaging. Following a positive diagnosis for PAM no medication was prescribed. At the 3 year follow-up the clinical course of the patient's disease remained stable.
Diagnostic imaging and pathological features of case 2. (C) Hematoxylin and eosin staining of bronchoalveolar lavage fluid demonstrating a lamellar calcified body (magnification, x400). HRCT, high resolution computed tomography.