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Case study semester 4
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Case study semester 4


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  • 1. Emphysema • Emphysema is a respiratory disease that result in millions alveoli stretching out of shape or rupturing. • As the alveoli become damaged or destroyed, the lungs lose their elasticity and become unable to empty easily.
  • 2. Combined Pulmonary Fibrosis and Emphysema • Combined pulmonary fibrosis and emphysema is characterized by the coexistence of both upper lobe emphysema and lower lobe pulmonary fibrosis. • Tobacco smoking is said to be the main factor in its etiology. • It is mostly seen in males older than 60 with a substantial smoking history(> 40 year pack history).
  • 3. • It is a unique form of idiopathic pulmonary fibrosis because of the preserved lung volumes due to the opposing effects of both emphysema and fibrosis on the total lung volume. • Patients classically present with progressive shortness of breath, with symptoms lasting for about 3 years before diagnosis. • Hypoxemia is a common finding and it is generally mild to moderate at rest and gets worse during exercise.
  • 4. • Physical examination usually reveals bilateral lower lobe inspiratory crackles and digital clubbing. • Pulmonary hypertension is highly prevalent in this disease and is the leading cause of death. • Patients CPFE may have a higher prevalence of lung cancer, with squamous cell carcinoma being the most common histologic type.
  • 5. • High-resolution computed tomogram is the imaging of choice, and it typically shows the characteristic upper lobe paraseptal emphysema with bullae, and lower lobe subpleural honeycombing with interstitial fibrosis and traction bronchiectasis. • Surgical lung biopsy is usually necessary to make a confident clinicopathologic diagnosis of CPFE
  • 6. • Corticosteroids, immunosuppressive agents, and antibiotic agents offer little to no benefit. • Home oxygen can be offered for symptomatic relief. • Lung transplant has been shown to offer some survival advantage in IPF patients.
  • 7. Case Study • 70-year-old Hispanic male who presented with progressive shortness of breath • Said he was in his usual state of health until the year before, when he started getting short of breath with exertion • Initially, dyspnea occurred only with severe exertion, but it progressively got worse to the point where the patient was getting short of breath even with mild exertion and sometimes at rest
  • 8. • Over the last year he also had a persistent dry cough • Exercise tolerance had dramatically decreased, to less than a block • He denied any paroxysmal nocturnal dyspnea, chest pain, fever, or hemoptysis
  • 9. Past History • 40 pack-year history, and he quit 3 years ago • Past medical history included benign prostatic hyperplasia • Denied any surgical history • Denied any alcohol or drug abuse. • He worked as a laborer all his life, including occasional painting and marble cleaning jobs. • Influenza and pneumococcal vaccines are up to date
  • 10. Physical Examination • • • • • Mild distress Vital signs and basic lab work were normal Low oxygen saturation of 92%. Inspiratory crackles and substantial clubbing On 6-min walk test he walked only 200 m (normal predicted was 600 m)and desaturated to 82% • Peak expiratory flow was 550 L/min
  • 11. • Chest x-ray showed bilateral lower lobe pulmonary fibrosis
  • 12. • HRCT showed lower lobe subpleural honeycombing, along with fibrosis and traction bronchiectasis
  • 13. • Bilateral upper lobe paraseptal emphysema with bullae
  • 14. • Pulmonary function tests showed no restrictive or obstructive pattern • Showed preserved lung volumes with severely decreased diffusing capacity • Echocardiogram showed moderate pulmonary hypertension with right ventricular systolic pressure of 50 mm Hg
  • 15. Subjective • I was healthy a year ago , then I started getting short of breath with exertion.
  • 16. Objective • Shortness of breath • Vital signs and lab work: Normal with Spo2 at 92% • Dry cough • Inspiratory crackles • Peak expiratory flow 500L/min • CXR:Bilateral lower lobe pulmonary fibrosis • HRTC: lower lobe subpleural honeycombing, fibrosis, traction bronchiectasis, and bilateral upper lobe paraseptal emphysema with bullae
  • 17. • PFT: No restrictive or obstructive pattern, preserved lung volumes with severely decreased diffusing capacity • ECG: Moderate pulmonary hypertension with RV systolic pressure of 50 mm Hg(normal <25 mm Hg)
  • 18. Assessement • Pt in mild respiratory distress(Shortness of breath, Spo2) • Mild hypoxemia(Spo2) • Idiopathic pulmonary fibrosis (History, dry cough, shortness of breath, clubbing, crackles, CXR, honeycombing, ECG) • Emphysema( history, SOB, cough, crackles, Spo2, HRCT,)
  • 19. Plan • • • • • • • Oxygen therapy via nasal cannula CPT Bronchodilator Acetylcysteine Prednisone Pulmonary rehab Monitor and reevaluate