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1998 Presentations for the Poster and Vignette Sessions
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PROGRESSIVE DYSPNEA - A CASE REPORT OF IDIOPATHIC PULMONARY FIBROSIS. Kurt C. Kamper, M.D. (Associate), Marshfield Clinic/St. Joseph's Hospital, Marshfield, WI 54449

A 55-year-old man with no significant past medical history presented with a several month history of progressive shortness of breath with exertion. Pulmonary function tests two weeks prior to admission demonstrated a severe restrictive defect. His shortness of breath worsened and on the day of admission he was found by his wife to be dyspneic and cyanotic. Physical exam showed the patient to be in moderate respiratory distress, with a respiratory rate of 42 breaths per minute and oxygen saturation 82% on 15-liter nonrebreather facemask. Pulmonary exam was significant for fine crackles posteriorly to the mid lung fields bilaterally. The remainder of his physical exam was unremarkable. Arterial blood gas revealed pH 7.45, pCO2 35, pO2 49, HCO3 24 on 15 liters 02. Chest x-ray demonstrated diffuse bilateral interstitial infiltrates. The patient was placed on mechanical ventilation. He underwent bronchoscopy which demonstrated mild diffuse mucosal erythema. Thoroscopic lung biopsy was subsequently performed, which demonstrated severe interstitial fibrosis with acute and chronic inflammation. The biopsy tissue was felt to be compatible with late stage appearance of usual interstitial pneumonitis. The patient proved to be very difficult to wean from mechanical ventilation. He was briefly extubated for a few days, but required reintubation for respiratory failure. Despite aggressive therapy, his pulmonary status failed to improve, and at the request of the family, ventilator support was withdrawn and the patient subsequently expired. Idiopathic Pulmonary Fibrosis (IPF) is a progressive interstitial lung disease of unknown etiology. The clinical features can be widely variable, ranging from an insidious onset over years to a rapidly fulminant and fatal presentation. I will discuss the current state of knowledge regarding IPF, focusing on the pathologic disease classification of the disease and its implications regarding treatment.

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