Pulmonary alveolar microlithiasis diagnosed with radiography, CT, and bone scintigraphy

Alkhankan, Emad; Yamin, Hasan; Bukamur, Hazim; Alkhankan, Fadi; Shweihat, Yousef; Zeid, Fuad

doi:10.1016/j.radcr.2019.03.032

Cited by 9 publications

(17 citation statements)

References 4 publications

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“…Typical features include intra-alveolar calcifications (microliths), micronodules, ground-glass opacities along with superimposed smooth thickened of the interlobular septa, subpleural microhoneycombing cystic changes, subpleural reticular thickening and, in some instances, a crazy paving pattern. Calcifications of the pleura also have been reported [ 54 , 55 ] ( figure 3 ).…”

Section: Diagnosismentioning

confidence: 91%

“…thickening and, in some instances, a crazy paving pattern. Calcifications of the pleura also have been reported [54,55]…”

Section: Pulmonary Alveolar Microlithiasismentioning

confidence: 93%

“…A black pleural sign or black pleural line is a common finding and describes the vertical strip of peripheral hyperlucency between the ribs and the adjacent diffusely dense calcified lung parenchyma and is caused by subpleural cystic changes. Often, the mediastinal contour and diaphragm cannot be identified due to intensive microliths and interstitial involvement in advanced disease [54] (figure 2).…”

Section: Imagingmentioning

confidence: 99%

“…High-resolution CT is regarded as the gold standard for PAM diagnosis and demonstrates numerous sandstorm-like calcifications throughout the lungs with subpleural and peribronchial distribution particularly affecting the basal segments and paracardiac region [ 54 ]. Typical features include intra-alveolar calcifications (microliths), micronodules, ground-glass opacities along with superimposed smooth thickened of the interlobular septa, subpleural microhoneycombing cystic changes, subpleural reticular thickening and, in some instances, a crazy paving pattern.…”

Section: Diagnosismentioning

confidence: 99%

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Pulmonary alveolar microlithiasis: no longer in the stone age

Bendstrup

Jönsson

2020

ERJ Open Res

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Pulmonary alveolar microlithiasis (PAM) is a rare parenchymal lung disease caused by variants in the SCL34A2 gene and characterised by the accumulation of intra-alveolar microliths. PAM has been reported in fewer than 1100 cases throughout the world. It is an autosomal recessive hereditary disease and often associated with consanguinity. Progress with respect to the genetic background and pathophysiology has resulted in an increased understanding of the disease in recent years. Until now, 30 genetic different SLC34A2 variants have been reported, which all are considered significant for disease development. There is no sex difference and the majority of cases are diagnosed at the age of 30–40 years. Many patients are asymptomatic and the diagnosis is made at random. When symptomatic, dyspnoea, cough, chest pain and fatigue are common complaints. The diagnosis of PAM can confidently be based on typical radiographic findings and genetic testing proving rare biallelic SCL34A2 gene variants. Bronchoalveolar lavage and histopathology may show microliths. There is no disease-specific treatment and management is supportive. Lung transplantation should be considered in advanced cases.

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Section: Diagnosismentioning

confidence: 91%

“…thickening and, in some instances, a crazy paving pattern. Calcifications of the pleura also have been reported [54,55]…”

Section: Pulmonary Alveolar Microlithiasismentioning

confidence: 93%

Section: Imagingmentioning

confidence: 99%

Section: Diagnosismentioning

confidence: 99%

See 2 more Smart Citations

Pulmonary alveolar microlithiasis: no longer in the stone age

Bendstrup

Jönsson

2020

ERJ Open Res

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“…Since then, 27 cases have been reported, of which ten are from India, including the present case. [5910] With an increasing proportion of cases being reported from India, a national registry of this “orphan” disease will help us better study the trends and improve management by having timely referrals for lung transplant.…”

mentioning

confidence: 99%