Calcified pulmonary consolidations in pulmonary alveolar microlithiasis: Uncommon computed tomographic appearance of a rare disease

Gupta, Pawan; Mittal, Richa; Chhabra, Sanjeev

doi:10.4103/0970-2113.205336

Cited by 3 publications

(1 citation statement)

References 11 publications

(21 reference statements)

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“…Diffuse involvement of both lungs is generally more apparent in the middle and lower regions. 14 , 15 High-resolution computed tomography (HRCT) findings can be classified into four stages based on the degree of radiological intensity. The first stage is referred to as pre-calcification because of the small number and size of calcifications.…”

Section: Discussionmentioning

confidence: 99%

Alveolar Microlithiasis with Mild Clinical Symptoms But Severe Imaging Findings: A Case Report

Hoseininia,

Salimi,

Salmani

et al. 2024

Clin Med Insights Case Rep

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Pulmonary alveolar microlithiasis (PAM) is a rare genetic disorder that causes calcium phosphate microliths to form in the alveoli. Symptoms usually appear in a person’s third or fourth decade of life. A definitive diagnosis does not always demand a lung biopsy but can be achieved in families with more than one member with PAM and compatible chest imaging. We present the case of a 47-year-old woman referred to us for shortness of breath. Chest imaging revealed bilateral diffuse ground-glass opacities, interlobar fissure calcification, and subpleural linear calcifications, leading to a diagnosis of PAM. Although there is no specific treatment for this condition, early diagnosis can help prevent it from progressing rapidly by avoiding exposure to risk factors.

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Section: Discussionmentioning

confidence: 99%

Alveolar Microlithiasis with Mild Clinical Symptoms But Severe Imaging Findings: A Case Report

Hoseininia,

Salimi,

Salmani

et al. 2024

Clin Med Insights Case Rep

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Pulmonary alveolar microlithiasis and interstitial pneumonitis: a case report of the west of Iran

Ramezani¹,

Aminparast²,

Sadeghi³

2019

BioMedicine

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Pulmonary alveolar microlithiasis (PAM) is a rare disease with autosomal recessive inheritance. Herein, a 20-year-old lady referred to the hospital with a dry cough for two years. The chest X-ray findings were bilateral reticulonodular opacities in both lungs and honeycomb appearance suspicious for miliary tuberculosis and idiopathic pulmonary fibrosis. A wedge biopsy of lung showed that there were several intraalveolar laminated concretions in the pathology report compatible with pulmonary alveolar microlithiasis and interstitial infiltration of lymphocytes and neutrophils compatible with interstitial pneumonitis. PAM is a rare progressive disease with the production of microliths in pulmonary alveoli. The pathologist, radiologist, and clinician should be familiar with this entity for diagnosis and appropriate management. The family of the patient especially siblings must be evaluated for earlier diagnosis.

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Pulmonary alveolar microlithiasis

et al. 2020

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Pulmonary alveolar microlithiasis (PAM) is a fascinating rare lung disease that is associated with the accumulation of hydroxyapatite microliths within the lumen of the alveolar spaces. In most patients, PAM is discovered incidentally on radiographs performed for other purposes, and the typical disease course is characterised by slowly progressive respiratory insufficiency over decades. Recent genetic analyses that have revealed that the deficiency of the sodium-phosphate cotransporter NPT2B is the cause of PAM have enabled the development of powerful animal models that inform our approach to disease management and treatment. Here we review the epidemiology and molecular pathophysiology of PAM, as well as the diagnostic approach, clinical manifestations, radiographic and pathologic features, and clinical management of the disease. Although there are no proven treatments for PAM, progress in our understanding of disease pathogenesis is providing insights that suggest strategies for trials.

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Calcified pulmonary consolidations in pulmonary alveolar microlithiasis: Uncommon computed tomographic appearance of a rare disease

Cited by 3 publications

References 11 publications

Alveolar Microlithiasis with Mild Clinical Symptoms But Severe Imaging Findings: A Case Report

Alveolar Microlithiasis with Mild Clinical Symptoms But Severe Imaging Findings: A Case Report

Pulmonary alveolar microlithiasis and interstitial pneumonitis: a case report of the west of Iran

Pulmonary alveolar microlithiasis

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