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Rare forms of interstitial lung disease
Published in Muhunthan Thillai, David R Moller, Keith C Meyer, Clinical Handbook of Interstitial Lung Disease, 2017
Pulmonary alveolar microlithiasis is a rare lung disease characterized by extensive intra-alveolar deposition of concentrically lamellated calcium phosphate spheres that results in a distinctive calcific micronodular infiltrate seen on chest radiography and CT (109–111). This disease was recently discovered to be a genetic disease caused by mutations of the SLC34A2 gene, which encodes a type IIb sodium phosphate cotransporter (112,113). Mutations in the SLC34A2 gene that impair normal co-transporter function (transporting the phosphorus ion from the alveolar space into the alveolar type II cells) cause formation of intra-alveolar calcium phosphate microliths (i.e. pulmonary alveolar microlithiasis). It is inherited in an autosomal recessive pattern.
The Incidence, and Polarization Optical Characteristics of Pulmonary Alveolar Calcospherities (Corpora Amylacea) in Rheumatoid Arthritis - A Retrospective Clinicopathologic Study of 210 Autopsy Patients
Published in Gilles Grateau, Robert A. Kyle, Martha Skinner, Amyloid and Amyloidosis, 2004
Pulmonary alveolar microlithiasis is a progressive process, characterized by crystalloid formation of beta-2-Microglobulin. During this process the alveolar calcospherities progressively increase in number and size. The „maturation’ of alveolar corpora amylacea is accompanied by deposition of minerals, namely calcium phosphates and carbonates. The clinical significance of pulmonary alveolar microlithiasis is not known, but it does not seem to contribute to interstitial pneumonitis, fibrosis, or heart failure.
Heterogeneous manifestation of pulmonary ossification in idiopathic pulmonary fibrosis
Published in Canadian Journal of Respiratory, Critical Care, and Sleep Medicine, 2022
S.G. Chong, R. Alsilmi, C. Scallan, P.G. Cox, M.R.J. Kolb, N. Hambly, A. Naqvi
It is essential to note the critical differences between pulmonary ossification and pulmonary calcification. Pulmonary calcification refers to the deposition of calcium salts in tissue, unlike pulmonary ossification, which is characterized by bone tissue formation (calcification in a collagen matrix).2 Several pathological conditions may predispose to soft tissue calcification, including chronic renal insufficiency, hypervitaminosis D, multiple myeloma, histoplasmosis, tuberculosis and pulmonary alveolar microlithiasis.2 The lungs are susceptible to calcification along with other internal organs such as the stomach, kidneys, heart and blood vessels.2 In either condition, the role of laboratory findings in diagnosis is limited.14
Severe pulmonary alveolar microlithiasis
Published in Canadian Journal of Respiratory, Critical Care, and Sleep Medicine, 2023
Fanny Gabrysz-Forget, Charles Poirier
Pulmonary alveolar microlithiasis is a rare genetic condition affecting the pneumocytes’ type II sodium-dependent co-transporter called NPT2b.1 The mutation of its encoding gene, the SLC34A2 gene, in the alveolar type II cells has been found to cause the disease.2 As the disease progress, calcium-phosphate microliths accumulate within the alveoli as a result of deficient phosphate clearance during surfactant degradation. Over a thousand cases has been reported over the years.3 Different treatments have been sought without demonstrating effectiveness, thus lung transplantation remains the only cure.3