Abstract
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.
Highlights
Pulmonary alveolar microlithiasis (PAM), or Harbitz’ syndrome, is a rare lung disease characterized by the presence of innumerable small calculi in the alveoli called microliths or calcospherites [1]
The present report is an example of a “typical” case of PAM with striking radiological features but mild clinical symptoms
PAM is characterized by periodic acid–Schiff-positive microliths, typically calcareous concentric lamellae around a central nucleus with an amorphous or granular aspect
Summary
Pulmonary alveolar microlithiasis (PAM), or Harbitz’ syndrome, is a rare lung disease characterized by the presence of innumerable small calculi in the alveoli called microliths or calcospherites [1]. The patient had presented as a young 35-year-old female post a motor vehicle accident She denied past medical and surgical history. Her oxygen saturation was 96.3% on two liters of oxygen delivered via nasal cannula. On physical examination, she had coarse crackles diffusely in bilateral lung fields. Computed tomography was performed which demonstrated diffuse pulmonary interstitial and alveolar calcification with minimal sparing of the apices (Figure 2). C: Coronal view, intense calcification of the interstitium and pleural serosa sparing the lung apices. The patient did well throughout the hospital stay As her ambulating oxygen saturation was 88%, she was discharged with home oxygen therapy.
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