Combined pulmonary fibrosis and emphysema a unique case report

Smoking is associated with a spectrum of disease like chronic obstructive pulmonary disease, Langerhan cell histiocytosis, Respiratory bronchiolitis, Desquamative interstitial pneumonia and Idiopathic pulmonary fibrosis. Emphysema and pulmonary fibrosis have dissimilar physiologic effects. Clinically, either emphysema or fibrosis typically predominates and individual patients are rarely recognized as having both disorders simultaneously. Combined pulmonary fibrosis and emphysema is a recently recognised radiologically defined syndrome. In this syndrome two different disease states, emphysema and pulmonary fibrosis coexist and manifest into a unique and distinct disease state that differs from either of the two components. Thesepatients have a different natural history, complications, and mortality than those with pulmonary fibrosis or emphysema alone. We report a 70 years male, smoker, who presented with exertional dyspnoea for 5 months, had hypoxemia with Spo2 of 72 room air, Auscultation showed bilateral NVBS with bibasilar fine end inspiratory crepts. ABG showed Type 1 respiratory failure. ECHO showed mild pulmonary hypertension(RVSP- 47 mm Hg). Spirometry showed mild obstruction(FEV1FVC 64.2, FEV12.15L, FEV1123, FVC3.34 L). HRCT Thorax showed Centrilobular and paraseptal emphysematous changes in both lungs, Few emphysematous bullae in right lower lobe, Peripheral subeplural reticulations in both lungs, extensive honeycombing in both lower lobes and left lingular segment and was diagnosed as Combined pulmonary fibrosis and emphysema. He was managed with oxygen support, nebulised bronchodilators and improved symptomatically.This illustrates the limitations of a simplistic diagnostic dichotomy between lung emphysema and fibrosis. Tobacco smoking may cause emphysemadominant, fibrosis-dominant, or CPFE pathology, with differing implications for treatment, complications, and prognosis. We should take a broad view of the mixture of lung pathologies in patients and avoid single-minded application of classification schemes. Biomarkers and moresensitive imaging techniques are needed to better define the CPFE syndrome.