Progression of Idiopathic Pulmonary Fibrosis

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     A 75-year-old man with idiopathic pulmonary fibrosis was admitted because of progressive shortness of breath. He stated that he had not had new cough, fever, chest pain, palpitations, orthopnea, or paroxysmal nocturnal dyspnea. His home oxygen requirement had increased from 3 liters per minute to 6 liters per minute, administered by nasal cannula. The patient had received the diagnosis of idiopathic pulmonary fibrosis in 1998 on the basis of clinical and radiographic findings (Panel A). His disease had progressed despite corticosteroid and interferon gamma therapy. Repeated computed tomography showed marked progression of fibrotic changes (Panel B). Broad areas of honeycomb changes and patchy areas of ground-glass infiltrate were present. Pulmonary-function testing showed that the forced vital capacity had decreased from 3.5 liters (81 percent of the predicted value) to 1.9 liters (47 percent of the predicted value). These findings were consistent with progression of the idiopathic pulmonary fibrosis. The patient was discharged to his home, where he was to receive 6 liters of oxygen per minute by nasal cannula through hospice care.

    Rony M. Abou Jawde, M.D.

    Feyrouz Al-Ashkar, M.D.

    Cleveland Clinic Foundation

    Cleveland, OH 44195

    http://www.100md.com/html/DirDu/2006/11/29/30/78/70.htm