Combined pulmonary fibrosis with emphysema in nonsmokers needs connective tissue disease workup: A case report

Combined pulmonary fibrosis and emphysema (CPFE) is a heterogenous lung disease documented in smokers, which includes emphysema in upper lobes and pulmonary fibrosis in lower lobes. CPFE in nonsmokers is rare, and very few literatures are available. In this case report, we have reported an 80-year-old male presented with progressive shortness of breath with fatigability and hypoxia treated as emphysema with inhaled bronchodilators. Response to medical treatment was not satisfactory with worsening of shortness of breath and fatigability. Clinical examination revealed bilateral basal velcro crepitations with resting oxygen saturation was 88% at room air. High-resolution computerized imaging documented emphysema in upper lobes with honeycombing and tractional bronchiectasis in lower lobes. Echocardiography documented pulmonary hypertension with dilated right atrium and ventricle. Connective tissue disease (CTD) panel documented workup positive for polymyositis. We have treated with oxygen supplementation during rest and ambulation, long-acting inhaled bronchodilator medicines, antifibrotic nintedanib, anti-inflammatory methylprednisolone, and pulmonary vasodilator tadalafil. Improvement in cardiopulmonary parameters including in 6-minute walk distance was significant with bronchodilators, mycophenolate mofetil, tadalafil, and antifibrotics.