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Semin Respir Crit Care Med 2001; 22(4): 357-386
DOI: 10.1055/s-2001-17381
Copyright © 2001 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Usual Interstitial Pneumonia

Joseph P. Lynch, III1 , Mark Wurfel3 , Kevin Flaherty1 , Eric White1 , Fernando Martinez1 , William Travis2 , Ganesh Raghu3
  • 1Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Michigan Medical Center, Ann Arbor, Michigan
  • 2Armed Forces Institute of Pathology, Washington, D.C
  • 3Division of Pulmonary and Critical Care Medicine, University of Washington, Seattle, Washington
Further Information

Publication History

Publication Date:
26 September 2001 (online)

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ABSTRACT

Usual interstitial pneumonia (UIP) is a distinct histological lesion observed in idiopathic pulmonary fibrosis (IPF), but can be found in other etiologies. The diagnosis of UIP can be established by surgical lung biopsy or by high resolution thin section computed tomographic scans (provided the radiographic features are classical). Historically, patients labeled as ``IPF'' encompassed a group of disorders including UIP as well as other idiopathic interstitial pneumonias, which differ from UIP in prognosis and responsiveness to therapy. Current recommendations from international consensus statements restrict the term IPF to patients with idiopathic UIP. The inciting cause(s) and pathogenesis of UIP have not been elucidated, but alveolar epithelial cell injury and dysregulation or altered phenotypic expression of fibroblasts are key elements. Inflammatory cells may play minor roles in initiating or propagating the fibrotic process. The prognosis of UIP is poor. Mean survival following diagnosis approximates 3 years. Current therapies are of unproven value. Corticosteroids or immunosuppressive agents have been most often used, but data affirming benefit are lacking. Single-lung transplantation is a viable option for patients failing medical therapy. Novel therapeutic strategies based upon inhibiting fibroproliferation or enhancing alveolar reepithelialization are desperately needed. In this article, we discuss diagnostic criteria for UIP (both histopathological and radiographic), natural history and clinical course, and therapeutic approaches (both current and future).

KEYWORD

Usual interstitial pneumonia (UIP) - idiopathic pulmonary fibrosis (IPF) - cryptogenic fibrosing alveolitis (CFA) - fibroblast - fibroblastic foci - honeycombing

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