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A 51-year-old male non-smoker presented with a complaint of progressive dyspnoea over 25 years, worsening during the past year. There was occasional cough with mucoid expectoration. There was no history of fever, cutaneous rashes, joint pains, Raynaud’s phenomenon or chronic drug intake. There was no history of exposure to occupational dusts. The patient had been treated for pulmonary tuberculosis in the past with no improvement in symptoms. On physical examination, he was of thin build and dyspnoeic at rest with a respiratory rate of 30/min. He had grade 3 digital clubbing. He did not have pallor, cyanosis or pedal oedema. On auscultation of the chest, he had vesicular breathing with bilateral basal fine end-inspiratory crepitations. He was on inhaled bronchodilators and home oxygen therapy.
Baseline investigations revealed haemoglobin of 17 g/dl, haematocrit 50.7% and a total leucocyte count of 9600/mm3. Erythrocyte sedimentation rate at the end of the first hour (Westergren method) was 35 mm. Chest radiograph and high resolution chest tomography (HRCT) images are shown in figs 1 and 2. Arterial blood gas analysis showed type 1 respiratory failure. Spirometry showed a severe restrictive abnormality with impaired diffusion capacity of the …
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