Diagnostic Images of Pulmonary Alveolar Microlithiasis: A Rare, Autosomal Recessive Disorder

Fabia Cruz; Diogo  Batista; Cátia Pereira; Vera Durão; Rita Macedo; Richard  Staats; Paula Pinto; Cristina  Bárbara

doi:10.12890/2022_003575

Fabia Cruz
Internal Medicine Service, Hospital Amato Lusitano, ULSCB, Castelo Branco, Portugal https://orcid.org/0000-0002-7952-0046
Diogo Batista
Pneumology Service, Hospital Santa Maria, CHULN, Lisbon, Portugal
Cátia Pereira
Pneumology Service, Hospital Santa Maria, CHULN, Lisbon, Portugal https://orcid.org/0000-0001-9247-2571
Vera Durão
Pneumology Service, Hospital Santa Maria, CHULN, Lisbon, Portugal https://orcid.org/0000-0003-1199-3741
Rita Macedo
Pneumology Service, Hospital Santa Maria, CHULN, Lisbon, Portugal https://orcid.org/0000-0002-3969-0200
Richard Staats
Pneumology Service, Hospital Santa Maria, CHULN, Lisbon, Portugal; Faculty of Medicine of Lisbon, ISAMB, Lisbon, Portugal https://orcid.org/0000-0003-2218-8537
Paula Pinto
Pneumology Service, Hospital Santa Maria, CHULN, Lisbon, Portugal; Faculty of Medicine of Lisbon, ISAMB, Lisbon, Portugal https://orcid.org/0000-0003-2301-2752
Cristina Bárbara
Pneumology Service, Hospital Santa Maria, CHULN, Lisbon, Portugal; Faculty of Medicine of Lisbon, ISAMB, Lisbon, Portugal https://orcid.org/0000-0003-0915-4105

Keywords

Pulmonary alveolar microlithiasis, parenchymal calcification, clinical–radiological dissociation

Abstract

Pulmonary alveolar microlithiasis (PAM) is a genetic lung disorder that is characterized by the accumulation of calcium phosphate deposits in the alveolar spaces of the lung. PAM is discovered incidentally on radiographs performed for other purposes, and the typical disease course is characterized by slowly progressive respiratory failure over decades. Treatment remains supportive. A 62-year-old woman presented in the emergency department with dyspnoea and fatigue. On physical examination she had crackles on pulmonary auscultation and digital clubbing. A CT scan of the chest showed multiple high-density areas throughout the lung parenchyma, suggesting the presence of alveolar microlithiasis. This CT finding is the typical radiological presentation of PAM, while the hallmark presentation is clinical–radiological dissociation.

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References

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Published: 2022-10-18
Issue: 2022: Vol 9 No 10 (view)

How to cite:

1.

Cruz F, Batista D, Pereira C, Durão V, Macedo R, Staats R, Pinto P, Bárbara C. Diagnostic Images of Pulmonary Alveolar Microlithiasis: A Rare, Autosomal Recessive Disorder. EJCRIM 2022;9 doi:10.12890/2022_003575.

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