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Pulmonary Alveolar Microlithiass: Accidentally Discovered FREE TO VIEW

Asem Hewidy, MD; Mohammad El Badrawy, MD
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Mansoura Faculty of Medicine, Mansoura, Egypt

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;149(4_S):A191. doi:10.1016/j.chest.2016.02.198
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SESSION TITLE: Diffuse Lung Disease

SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, April 17, 2016 at 11:45 AM - 12:45 PM

INTRODUCTION: Pulmonary alveolar microlithiasis is rare autosomal recessive disorder characterized by intra-alveolar accumulation of microliths, may be sporadic or familial and may affect people of any age, diagnosis is sometimes based on radiological findings;sandstorm appearance is the typical X-ray finding, the diagnosis is confirmed by HRCT and transbronchial biopsy (1)

CASE PRESENTATION: Female patient 32 years old presented 2 years ago productive cough, developed fever, severe dyspnes at rest. There was loss of appetite and loss of weight. No family history of similar condition. On general examination she had orthopnea, central cyanosis, 2nd degree clubbing. On auscultation; bilateral inspiratory fine crepitations. The routine blood examination was normal. PaO2 55 mmhg on room air, spirometry showed mild restriction, echocardiography normal. Chest X-ray showed diffuse scattered small nodules involving both lung more in basal parts. HRCT of the chest showed diffuse groundglass attenuation, small nodules, calcifications along the interlobular septa and subpleural regions. Fiberoptic bronchoscope revealed hyperemic mucosa and transbronchial biopsy showed intra-alveolar lamellar microliths confirming PAM diagnosis

DISCUSSION: Homozygous mutations in the gene of solute carrier family 34 (sodiumphosphate), member 2 (SLC34A2) are significantly, as this gene encodes a type IIb sodium-dependent phosphate transporter (NaPi-IIb) (1) Patients are mostly asymptomatic and are diagnosed incidentally on radiology by numerous sand-like microliths diffusely scattered in bilateral lung fields predominantly in the lower two-thirds. On HRCT, the most common findings are diffuse groundglass attenuation and subpleural linear calcifications more in basal and medial aspects of the lungs (2) Confirmatory diagnosis can be established by open lung or transbronchial biopsy, showing characteristic intra-alveolar lamellar microliths. Currently, there is no specific medical therapy for PAM and as patients suffer from respiratory insufficiency they needlung transplantation (3)

CONCLUSIONS: PAM is a rare lung disease discovered accidentally diagnosed radiologically by sandstorm pattern and diffuse calcifications on HRCT, confirmed by transbronchial or open lung biopsy with intra-alveolar lamellar microliths. No specific treatment lung transplantation is the only hope

Reference #1: Mariotta S, Ricci A, Papale M et al Pulmonary alveolar microlithiasis: report on 576 cases published in the literature. Sarcoidosis Vasc Diffuse Lung Dis 2004;21(3):173e81

Reference #2: Schmidt H, Lo¨rcher U, Kitz R, et al. Pulmonary alveolar microlithiasis in children. Pediatr Radiol 1996;26 (1):33e6

Reference #3: Jackson KB, Modry DL, Halenar J, et Single lung transplantation for pulmonary alveolar microlithiasis. J Heart Lung Transplant. 2001;20:226.

DISCLOSURE: The following authors have nothing to disclose: Asem Hewidy, Mohammad El Badrawy

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