CPFE Syndrome

Combined Pulmonary Fibrosis and Emphysema Syndrome (CPFE). Chief Complaints A 81 yr old male,Farmer by profession, Non Smoker attended Mopd with Insidious progressive onset of SOB and Non productive over 4 year. No Associated Chest pain ,Fever,Hemoptysis,Sputum production,Palpitation, Diaphoresis,Referral of pain etc. No H/O Pulm Koch,HTN,T2DM,Thyroid disorder or Substance Abuse. General Examination is normal. Vitals stable. No Clubbing,cyanosis,pedal edema etc. Systemic Examination normal except B/L lower Zone Velcro crepts. Routine Ix was advised. All Ix are normal except CXR showing B/L Hyperinflated lungs with few bronchiectatic changes. Next PFT with CT Thorax was advised. PFT is normal with Normal FEV1,normal FVC,Normal FEV1/FVC ratio and Normal PEF. CT thorax shows diffuse Emphysematous changes with Bronchiectasis and Mild Fibrosis. Pt Started on Conservative treatment. 2D ECHO is being planned to rule out Pulmonary HTN and Associated any Cardiac involvement.

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Bad combination. Needs meticulous monitoring Low flow oxygen support Mucolytics ICS LABA LAMA combination Antifibrotics Sos Rx of PAH after echo CD Influenza and pneumococcal vaccination Chest physiotherapy, incentive spirometry

Hrct and xray chest are consistent with emphysematous with bronchiactasis and lung fibrosis Rest of investigations are insignificant Bronchodilators Antifibrotic drugs like pirfenidone 200mg 2tds Tab pulmoclear 1bd Tab nintena 150mg 1bd Lama and laba inhalers Diuretics Spirometry Sos 02 support Broadspectrum antibiotics like lenazolinid Or inj Ceftriaxozone Inj piperacillin+inj tazobactum Consider oral steroids in case DOE is not improved

Thanx dr Sandeep Ghodekar
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? BRONCHIECTASIS .. ? ILD ..

Tnx Dr Shivraj Agarwal sir
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Bronchiectasis I L D As Spirometery Postural drainage

Spondylosis. Disc spaces intact

Heading towards cor pulmonale

BRONCHIECTASIS? REFER TO PULMONOLOGIST OPINION FOR BETTER DIAGNOSIS.

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