PURPOSE: Pulmonary Alveolar Microlithiasis (PAM) is a rare idiopathic disorder, first described by Friedric in 1856. Till now only few hundred cases have been reported world wide. We report a series of five cases from Himachal Pradesh, a hilly state situated in North Western Himalyan region of India.
METHODS: We confirmed five cases (3 males and 2 females) of PAM with age range of 18 –55 years during the last five years (2002-2006), belonging to different parts of the state. A detailed history including family and occupational history was obtained in each case and complete physical examination was carried out. All the cases were subjected to chest radiograph, spirometry including diffusion studies, CT thorax, transbronchial lung biopsy(TBLB) and other relevant heametologic and bio-chemical tests.
RESULTS: All the five cases of PAM came from rural background with no specific occupation and no family history of similar illness. Three patients did not have significant complaints and were referred to us with abnormal chest radiograph with typical‘Sand Storm Appearance’. One patient had exertional dyspnoea and another female aged 45 years progressed to develop cardio respiratory failure during the followup period. Family screening carried out in three patients was negative. In three patients (2 females and 1 male) had near normal lung function tests while in two cases restrictive ventilatory defect with reduced DLCO was recorded. One patient had evidence of pleural and pericardial calcification on CT thorax.Histology of TBLB specimens revealed intra alveolar calcification with little fibrosis of alveolar walls. All the patients were counselled about the nature of the disease and in one patient treatment for cardio-respiratory failure was initiated.
CONCLUSION: Five cases of PAM from Himachal Pradesh,North India have been reported with evidence of pericardial calcification in one case.To the best of our knowledge pericardial calcification has not been reported with PAM.
CLINICAL IMPLICATIONS: Awareness of this rare disorder among the physicians is essential to suspect the cases of PAM to avoid unnecessary investigations.
DISCLOSURE: Surender Kashyap, No Financial Disclosure Information; No Product/Research Disclosure Information