Interstitial And Fibrotic Lung Disease

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Interstitial And Fibrotic Lung Disease

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Questions and Answers
  • 1. 
    A 62-year-old man presents to your clinic for follow-up evaluation of shortness of breath and dry cough. His breathlessness on exertion started insidiously 6 months ago. Review of his evaluation reveals pulmonary function tests consistent with restriction and severely decreased diffusing capacity of the lung for carbon monoxide (DLCO). Highresolution computed tomography (HRCT) demonstrates bibasilar reticular abnormalities without ground-glass opacities or consolidation. A transbronchial lung biopsy was nondiagnostic. All serologies for connective tissue disease have been negative, and on repeat history today, the patient denies any past exposure to heavy metals, chemical toxins, molds, dusts, or asbestosis. On examination today, you note a resting O2 saturation of 88% on room air and bibasilar inspiratory crackles. There is no jugular venous distention, s3, or peripheral edema.   Which of the following is the most likely diagnosis for this patient?
    • A. 

      Acute interstitial pneumonitis

    • B. 

      Cryptogenic organizing pneumonia (COP)

    • C. 

      Idiopathic pulmonary fibrosis (IPF)

    • D. 

      Mycoplasma pneumonia

    • E. 

      Congestive heart failure

  • 2. 
    A 68-year-old woman with known IPF presents to your offi ce for follow-up. She states that her lung breathing trouble seems to be about the same, but she has felt a little weaker lately. Today on examination, her O2 saturation is 93% on 2 L O2 by nasal cannula. Her lung examination again reveals coarse, Velcro-like inspiratory crackles at both bases. She does not have elevated jugular venous pressure or peripheral edema, but you do note clubbing. Her pulmonologist has recently been treating her lung disease with prednisone and azathioprine, but the patient is not sure if this is helping. You order an arterial blood gas, chest x-ray, pulmonary function testing, and a 6-minute walk test.   Which of the following findings would be most predictive of further clinical deterioration of the patient's disease?
    • A. 

      Decrease in forced vital capacity (FVC) by 12% from her previous study

    • B. 

      Moderate increase in reticular opacities on chest x-ray

    • C. 

      Carbon dioxide tension (PCO2) > 45 mm Hg on arterial blood gas

    • D. 

      Decrease in distance walked in 6 minutes by 10 meters

  • 3. 
    A 58-year-old male former smoker returns to your clinic for a follow-up appointment for IPF. On your initial evaluation, he reported several months of insidiously worsening shortness of breath and dry cough. He denied any significant previous exposures, and all serologies for connective tissue diseases were negative. HRCT demonstrated peripheral interstitial opacities with honeycombing and traction bronchiectasis. A surgical lung biopsy was consistent with usual interstitial pneumonia (UIP).   For this patient, which of the following therapies has proven survival benefit?
    • A. 

      Prednisone

    • B. 

      Prednisone + cyclophosphamide

    • C. 

      Methotrexate

    • D. 

      Lung transplantation

    • E. 

      Colchicine

  • 4. 
    A 49-year-old previously healthy man presented to the emergency room (ER) with 3 weeks of progressive shortness of breath. He has noticed headaches, myalgias, malaise, sore throat, and a productive cough. His dyspnea has progressed to the point that he is now markedly short of breath at rest. He denied any recent exposures or inhalation. On arrival to the ER, his O2 saturation was 82% on room air, and he was in marked respiratory distress. He was intubated and transferred to the intensive care unit. A chest x-ray on admission demonstrated bilateral pulmonary opacities. His arterial oxygen tension (PaO2) was 115 mm Hg on 100% fraction of inspired oxygen (FiO2). Right heart catheterization demonstrated a pulmonary capillary wedge pressure of 13 cm H2O. All cultures, including bronchoalveolar lavage fl uid, were negative for bacterial, fungal, or viral pathogens. Transbronchial biopsy revealed diffuse alveolar damage. A rapid fl u test was negative.   Which of the following is the most likely diagnosis in this patient?
    • A. 

      Acute interstitial pneumonia

    • B. 

      Nonspecific interstitial pneumonia

    • C. 

      Lupus pneumonitis

    • D. 

      Miliary tuberculosis

    • E. 

      Desquamative interstitial pneumonia

  • 5. 
    A 61-year-old man with hypertension and hyperlipidemia returns to your clinic for follow-up on cough and shortness of breath. Over the past several months, he has had several bouts of "pneumonia," which have not responded to moxifloxacin or amoxicillin outpatient therapy. His chest x-rays have demonstrated persistent alveolar opacities throughout the illness. After several courses of antibiotics and months of shortness of breath and productive cough, you referred the patient to a cardiothoracic surgeon for video-assisted thoracoscopic lung biopsy. Today you received a pathology report stating that the biopsy was consistent with cryptogenic organizing pneumonia (COP).   What is the most appropriate next step for this patient?
    • A. 

      Prescribe a 3-month course of doxycycline

    • B. 

      Prescribe prednisone, 60 mg daily

    • C. 

      Initiate mycophenolate mofetil

    • D. 

      Observe the patient; follow up in 3 months

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