Combined pulmonary fibrosis and emphysema a unique case report.
Abstract
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 synd
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