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Pulmonary Alveolar Microlithiasis “Stone Lungs”: A Case of Clinico-Radiological Dissociation
Pulmonary alveolar microlithiasis (PAM) is a rare infiltrative lung disease characterized by deposition of spherical calcium phosphate microliths called calcospherites within the alveoli. PAM was first described by Friedrich in 1856 and then by Harbitz in 1918. The disease pathogenesis is based on m...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Cureus
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5037061/ https://www.ncbi.nlm.nih.gov/pubmed/27688986 http://dx.doi.org/10.7759/cureus.749 |
Sumario: | Pulmonary alveolar microlithiasis (PAM) is a rare infiltrative lung disease characterized by deposition of spherical calcium phosphate microliths called calcospherites within the alveoli. PAM was first described by Friedrich in 1856 and then by Harbitz in 1918. The disease pathogenesis is based on mutations in the SLC34A2 gene that encodes for the Type IIb sodium-phosphate cotransporter. The majority of the patients are diagnosed at an early age, usually between the ages of 20 and 40 years. The hallmark of this disease is a striking dissociation between the radiological findings and the mild clinical symptoms. We report a case of 35-year-old woman who presented post-motor vehicle accident with back pain and with minimal dyspnea on exertion. The final diagnosis was made after computed tomography and lung biopsy. The present case exhibits the remarkable clinico-radiological dissociation with complete calcification of the lungs on radiographic images with a relatively mild clinical presentation. |
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