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A 27-year-old man was seen in the outpatient pulmonary division of this hospital because of progressive dyspnea and decreased exercise tolerance.
Approximately 2 years earlier, the patient noted a slight decrease in his exercise tolerance. He was able to exercise regularly and so did not seek medical attention. Six months before this evaluation, dyspnea on exertion progressively increased, and the next month, an episode of hemoptysis occurred. Wheezing developed, associated with a chronic cough productive of clear-to-green sputum. He saw a physician at another facility. The temperature was normal, the blood pressure 90/60 mm Hg, the pulse 116 beats per
Differential Diagnosis
Important Features of the Case
Idiopathic Interstitial Pneumonias and Other Idiopathic Diseases
Pulmonary Manifestations of Connective-Tissue Diseases
Exposures Associated with Pulmonary Fibrosis
Familial and Genetic Disorders Associated with Pulmonary Fibrosis
The Hermansky–Pudlak Syndrome
Clinical Manifestation of HPS
Summary
Clinical Diagnosis
Dr. Andrew M. Tager's Diagnosis
Pathological Discussion
Anatomical Diagnosis
Source Information
From the Pulmonary and Critical Care Unit and the Center for Immunology and Inflammatory Diseases, Department of Medicine (A.M.T.), and the Departments of Radiology (A.S.) and Pathology (E.J.M.), Massachusetts General Hospital; and the Departments of Medicine (A.M.T.), Radiology (A.S.), and Pathology (E.J.M.), Harvard Medical School.
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