|Year : 2021 | Volume
| Issue : 1 | Page : 51-52
Pulmonary alveolar microlithiasis
Sudheer Tale1, Vipul Prakash1, Mihir Gangakhedkar1, Mrudula Kolli1, Anil Kumar Garbhapu2, Girish Sindhwani1
1 Department of Pulmonary Medicine, All Institute of Medical Sciences, Rishikesh, Uttarakhand, India
2 Department of Pediatric Surgery, RML Hospital, New Delhi, India
|Date of Submission||25-Jun-2020|
|Date of Decision||30-Jul-2020|
|Date of Acceptance||05-Aug-2020|
|Date of Web Publication||15-Feb-2021|
Dr. Sudheer Tale
Department of Pulmonary Medicine, AIIMS, Rishikesh
Source of Support: None, Conflict of Interest: None
Pulmonary alveolar microlithiasis is a rare genetic disorder and the affected people often presented with shortness of breath on exertion and dry cough. Chest radiology shows diffuse micronodules with calcifications that are easily visible on noncontrast computed tomography scans. It is progressive disease and treatment is only supportive.
Keywords: Alveolar mircolithiasis, calcification, genetics
|How to cite this article:|
Tale S, Prakash V, Gangakhedkar M, Kolli M, Garbhapu AK, Sindhwani G. Pulmonary alveolar microlithiasis. J Assoc Chest Physicians 2021;9:51-2
A 24-year-old male patient presented to the outpatient department with progressively increasing dyspnea and nonproductive cough for the preceding 1 year. He was a nonsmoker and had no addictions. He had previously received a full course of antitubercular drugs that had been advised based solely on chest radiograph findings, with no significant improvement in symptoms. His examination was significant for digital clubbing, tachypnea, and fine basal crepitations. High-resolution computed tomography (HRCT) of the chest showed widespread micronodules throughout the bilateral lung fields with ground glass opacities and septal thickening [Figure 1]. Mediastinal window sections of the HRCT revealed diffuse nodular calcifications along the interlobar septa and in subpleural regions [Figure 2]. In cytospin smears of bronchoalveolar lavage, round to oval microliths were seen, suggesting the diagnosis of pulmonary alveolar microlithiasis (PAM).
|Figure 1 HRCT image of chest showing diffuse micronodules, groundglass opacities and septal thickening|
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|Figure 2 Mediastinal window of the chest showing subpleural calcifications, nodules and septal thickening|
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| Discussion|| |
PAM is a rare genetic disorder with an autosomal recessive pattern of transmission characterized by deposition of calcium phosphate crystals within the alveoli known as microliths due to mutations in the solute family carrier gene SLC34A2, causing loss of function in the sodium phosphate cotransporter type IIb. This results in intra-alveolar accumulation of phosphate, creating a nidus for microlith formation. There are very few cases in the literature with reported prevalence of 500 cases worldwide. Typical radiological findings are diffuse micronodules in bilateral lung fields, ground glass opacities, and subpleural calcification as seen in our case. Diagnosis is made by bronchoalveolar lavage or lung biopsy, which demonstrates microliths within the alveoli. There is no definitive treatment for this condition except for lung transplantation and the posttransplant recurrence of this condition is not reported in the literature.
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Conflicts of Interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]