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(Chest. 1972;61:213-220.)
© 1972 American College of Chest Physicians

The Concept of Classic Interstitial Pneumonitis-Fibrosis (CIP-F) as a Clinicopathologic Syndrome

Richard A. DeRemee M.D., F.C.C.P.1; Edgar G. Harrison Jr. M.D.2; and Howard A. Andersen M.D., F.C.C.P.1

1 Mayo Clinic and Mayo Foundation: Division of Thoracic Diseases and Internal Medicine
2 Mayo Clinic and Mayo Foundation: Department of Surgical Pathology

The records of 81 patients with a roentgenologic pattern of diffuse reticulonodular pulmonary disease and a pathologic diagnosis of chronic interstitial pneumonitis and fibrosis were reviewed. All had restrictive patterns on pulmonary function testing with impairment of CO diffusing capacity. The most common features were characteristic dry rales, digital clubbing, and evidence of altered immune activity. Twenty patients manifested evidence of other disease thought to be of an autoimmune or hypersensitivity nature, with the pulmonary lesion dominating the clinical picture. The concept of "classic interstitial pneumonitis-fibrosis (CIP-F)" as a clinicopathologic syndrome has been proposed with the following categories: (1) definite—complete syndrome with rales, clubbing, and evidence of altered immune activity; (2) probable—rales and clubbing without evidence of altered immune activity; and (3) possible—histopathologic finding with or without evidence of altered immune activity but no rates or clubbing. Some patients progress from possible CIP-F to definite CIP-F; the latter category implies chronicity and commensurately worse prognosis.




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