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