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Semin Respir Crit Care Med 2004; 25(5): 491-521
DOI: 10.1055/s-2004-836143
Copyright © 2004 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001 USA.

Wegener's Granulomatosis: Evolving Concepts in Treatment

Joseph P. Lynch1  III , Eric White2 , Henry Tazelaar3 , Carol A. Langford4
  • 1Department of Medicine, Division of Pulmonary, Critical Care Medicine, and Hospitalists, The David Geffen School of Medicine at UCLA, Los Angeles, California
  • 2Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Michigan Medical Center, Ann Arbor, Michigan
  • 3Department of Laboratory Medicine and Pathology, Mayo Clinic Foundation, Rochester, Minnesota
  • 4Center for Vasculitis Care and Research, Department of Rheumatic and Immunologic Diseases, The Cleveland Clinic Foundation, Cleveland, Ohio
Further Information

Publication History

Publication Date:
09 November 2004 (online)

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Wegener's granulomatosis (WG), the most common of the pulmonary granulomatous vasculitides, typically involves the upper respiratory tract, lower respiratory tract (bronchi and lung), and kidney, with varying degrees of disseminated vasculitis. Major histological features include a necrotizing vasculitis involving small vessels, extensive “geographic” necrosis, and granulomatous inflammation. Clinical manifestations of WG are protean; virtually any organ can be involved. Further, the spectrum and severity of the disease is heterogeneous, ranging from indolent disease involving only one site to fulminant, multiorgan vasculitis leading to death. The pathogenesis of WG has not been elucidated, but both cellular and humoral components are involved. Circulating antineutrophil cytoplasmic antibodies (cANCA) likely play a role in the pathogenesis and often correlate with activity of the disease. Treatment strategies are evolving. Cyclophosphamide (CYC) plus corticosteroids (CS) is the mainstay of therapy for generalized, multisystemic WG. Historically, the combination of CYC plus CS was used for a minimum of 12 months, but concern about late toxicities associated with CYC has led to novel treatment approaches. Currently, short-course (3-6 months) induction treatment with CYC plus CS, followed by maintenance therapy with less toxic agents (e.g., methotrexate, azathioprine) is recommended. Further, recent studies suggest that methotrexate combined with CS may be adequate for limited, non-life threatening WG. The role of other immunomodulatory agents (including trimethoprim-sulfamethoxazole) is also explored.

KEYWORDS

Wegener's granulomatosis - granulomatous vasculitis - pulmonary vasculitis - capillaritis - antineutrophil cytoplasmic antibodies - geographic necrosis

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Joseph P Lynch IIIM.D 

Division of Pulmonary and Critical Care Medicine and Hospitalists

The David Geffen School of Medicine at UCLA

10833 Le Conte Ave., Rm. 37-131 CHS

Los Angeles, CA 90095-1690

Email: jplynch@mednet.ucla.edu

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