A 25 years old married female, mother of two children came in the outpatient clinic with complaints of persistent cough for the last 10 years and breathlessness for six years with off and on fever during this period. According to the patient she was well 10 years ago when she started getting bouts of dry cough especially at night so much so that she used to wake up from sleep. Also she started having episodes of fever along with cough which sometimes resolved spontaneously (after variable periods of time) and at other times she took treatment from a doctor for which she had to travel far as she resided in a far flung area of Baluchistan desert in Pakistan with almost no medical facilities. After few years of intractable cough, she as well started noticing breathlessness which was initially on exertion and later on at rest also with exacerbations at the time of fever. She never had hemoptysis but had history of passing gritty particles in sputum. There was no history of orthopnea, paroxysmal nocturnal dyspnoea, body swelling, urinary or gastrointestinal complaint and she was never hospitalized. For ten years, she consulted many general practitioners and consultants and considering her chest Xrays has tuberculosis like radiological appearance, she was prescribed anituberculous therapy thrice, each upto 8-9 months duration. In addition, she has had multiple courses of antibiotics and both oral and inhaled corticosteroids. There was no improvement from any of these drugs.
She presented to us with severe exertional dyspnoea for the last 2 weeks. On examination, she was afebrile, tachypnoeac with respiratory rate of 32 breaths per minute with normal blood pressure and pulse. Her Chest examination revealed scattered crepitations all over the chest but otherwise no other abnormality. Rest of her systemic examination was normal. Her blood oxygen saturation while breathing room air was 82% while arterial blood gas analysis showed low pressures of Oxygen.
Her laboratory tests showed hemoglobin level of 11.5 gm/dl, TLC of 9500/cmm with 60% neutrophils and 25% lymphocytes and elevated ESR of 30 mm in 1st hour. Liver function tests, serum electrolytes, renal function tests, serum parathyroid hormone and vitamin D were all within normal range. Pulmonary function tests showed moderately restrictive lung disease which was not reversed with bronchodilators Sputum smear for AFB was negative three times and culture showed no growth. Her Chest Xray (Fig. 1A, B and C) showed bilateral diffuse fine nodules predominantly in the middle and lower Zones (Sand Storm Appearance). Her CT Scan Chest images (Fig. 2A, B and C) demonstrated widespread tiny micro calcifications throughout the lungs with septal thickening and ground-glass opacification. Later on, Bronchoscopic alveolar lavage was performed which revealed rehtypical microliths and cytologic smears showed extracellular and intracellular concentrically round microliths confirming the diagnosis of pulmonary alveolar microlithiasis. She was symptomatically treated by Intravenous Moxifloxacin to cover possible infections and oxygen therapy via nasal cannula at 3 lit/min and later on discharged with advice of long term oxygen therapy at home. Because of her travel logistic difficulties, she is on 4 monthly (May 2015, September 2015, January 2016 and May 2016) follow up and regular till now.