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Semin Respir Crit Care Med 2018; 39(04): 434-458
DOI: 10.1055/s-0038-1660874
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Granulomatosis with Polyangiitis (Wegener's Granulomatosis): Evolving Concepts in Treatment

Joseph P. Lynch III
1   Division of Pulmonary, Critical Care Medicine, Allergy and Clinical Immunology, Department of Medicine, The David Geffen School of Medicine at UCLA, Los Angeles, California
,
Ariis Derhovanessian
1   Division of Pulmonary, Critical Care Medicine, Allergy and Clinical Immunology, Department of Medicine, The David Geffen School of Medicine at UCLA, Los Angeles, California
,
Henry Tazelaar
2   Department of Laboratory Medicine and Pathology, Mayo Clinic, Scottsdale, Arizona
,
John A. Belperio
1   Division of Pulmonary, Critical Care Medicine, Allergy and Clinical Immunology, Department of Medicine, The David Geffen School of Medicine at UCLA, Los Angeles, California
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Publikationsdatum:
07. November 2018 (online)

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Abstract

Granulomatosis with polyangiitis (GPA), formerly termed Wegener's granulomatosis, is the most common of the pulmonary vasculitides. GPA typically involves the upper respiratory tract, lower respiratory tract (bronchi and lung), and kidney, with varying degrees of disseminated vasculitis. Cardinal histologic features include a necrotizing vasculitis involving small vessels, extensive “geographic” necrosis, and granulomatous inflammation. The spectrum and severity of the disease is heterogeneous, ranging from indolent disease involving only one site to fulminant, multiorgan vasculitis. Circulating antibodies against cytoplasmic components of neutrophils (ANCAs) play a role in the pathogenesis, and often correlate with activity of the disease. Treatment strategies are evolving. Cyclophosphamide (CYC) plus corticosteroids was the mainstay of therapy for generalized, multisystemic GPA since the 1970s. However, within the past decade, rituximab (RTX), a monoclonal antibody directed against B cells, has been shown to be at least as effective (and possibly more effective) as CYC. Furthermore, the use of RTX may reduce the need for maintenance immunosuppression. Optimal therapy for GPA remains controversial, and additional studies are required to determine the role and duration of maintenance therapy following successful induction therapy.

Keywords

granulomatosis with polyangiitis - Wegener's granulomatosis - granulomatous vasculitis - pulmonary vasculitis - capillaritis - antineutrophil cytoplasmic antibodies - rituximab