Thread by @vikkypaedia

Folks...
Have you all seen anything like this before..!?
What could this be?

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▶️A 57-year-old man of Javanese ethnicity, Indonesia, with a 12-year history of progressive shortness of breath on exertion
▶️there were inspiratory coarse crackles & occasional wheezing with⏬vesicular sound across all thoracic areas, more in the bilateral base of the lungs.

▶️Cardia was unremarkable, with observable clubbed drumstick appearance of the fingers, without cyanosis or peripheral edema
▶️heavy smoker, around 40 pack-years.
▶️previous h/o completed treatment of pulmonary Tb in 2018.

▶️HRCT scan, revealed diffuse calcification along the interlobar septa & subpleural regions in the lower pulmonary regions, with ground-glass attenuation & septal thickening in btw. Multiple cysts along the subpleural line were identified as causing the black pleural line signs

▶️A bronchoscopy with bronchoalveolar lavage & transbronchial lung biopsy was performed
▶️The branches of the bronchial tree were all patent.
▶️Unfortunately, the microliths were absent & the histology findings were inconclusive.

▶️2DEcho showed: dilated ventricles, resulting in mitral and tricuspid valve regurgitation.
▶️LV contractility ⏬with an EF of 22% & RV contractility ⏬with a tricuspid annular plane systolic excursion of 1.4 cm.

▶️PFTs : not performed due to clinical deterioration of progressive dyspnea during IP hospital observation.
▶️Hematologic tests were unremarkable.
▶️ABG showed metabolic alkalosis with respiratory compensation.

what are the DDs for a sandstorm appearance with black pleura sign (due to subpleural cystic changes) on chest radiograph and micro- calcification, subpleural cystic changes and calcified pleura on HRCT?

Final Diagnosis: Pulmonary alveolar microlithiasis (PAM); an uncommon pulmonary disease characterized by deposition of microliths in the alveoli.

(Hematoxylin-eosin stain sections): shows multiple laminated calcospherites (black arrow) within the alveoli of lung parenchyma suggestive of PAM of lung (100x).

▶️It dev chronically with an initially poorly defined etiopathogenesis.
▶️recently revealed that mut of the SLC34A2 gene causes the disease.
▶️The SLC34A2 gene encodes pH-sensitive Na-dep phosphate transporter, i.e., Na-dep phosphate transport protein 2b or NaPi-IIb.

▶️This protein plays a pivotal role in phosphate homeostasis in several organs and tissues in the body.
▶️However, its main location is in the alveoli of the lungs, specifically in alveolar type II cells

▶️These cells create surfactant from phospholipids & are also resp for recycling & decomposing old surfactant.
▶️The problem with SCLC34A2 is that it is likely to make it harder to get rid of the waste product phosphate, which leads to calcium-phosphate buildup in the alveoli

Sadly, the patient in the vignette, refused to be treated & decided to stop the treatment.
Source of the case vignette: 10.12659/AJCR.938456