Usual Interstitial Pneumonia

Joseph P. Lynch; Rajan Saggar; S. Sam Weigt; David A. Zisman; Eric S. White

doi:10.1055/s-2006-957335

Seminars in Respiratory and Critical Care Medicine, Table of Contents

Semin Respir Crit Care Med 2006; 27(6): 634-651
DOI: 10.1055/s-2006-957335

Usual Interstitial Pneumonia

Joseph P. Lynch¹ III , Rajan Saggar¹ , S. Sam Weigt¹ , David A. Zisman¹ , Eric S. White²

¹Division of Pulmonary, Critical Care Medicine, and Hospitalists, Department of Internal Medicine, The David Geffen School of Medicine at UCLA, Los Angeles, California
²Division of Pulmonary and Critical Care Medicine, University of Michigan Medical Center, Ann Arbor, Michigan

Abstract

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 (CT) 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 that 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. Lung transplantation is a viable option for patients failing medical therapy. This review discusses diagnostic criteria for UIP (both histopathological and radiographic), natural history and clinical course, and therapeutic approaches (both current and future).

KEYWORDS

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

Full Text

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Joseph P Lynch IIIM.D.

Division of Pulmonary, Critical Care Medicine, and Hospitalists, The David Geffen School of Medicine at UCLA

10833 Le Conte Ave., Rm. CHS 37-131, Los Angeles, CA 90095

Email: jplynch@mednet.ucla.edu