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Granulomatosis with Polyangiitis (Wegener’s Granulomatosis)

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Orphan Lung Diseases

Abstract

Granulomatosis with polyangiitis (formerly, Wegener’s granulomatosis) is a systemic necrotizing and granulomatous vasculitis predominantly affecting small-sized vessels. It is also one of the three vasculitides associated with anti-neutrophil cytoplasm antibodies (ANCA). The incidence rate is 2–12 cases per year and the prevalence 23–160 cases per million population. The cause remains unknown, but the pathophysiological mechanisms are being progressively defined. Disease onset can occur at any age, most commonly about 40–50 years. The main clinical characteristics include manifestations in the upper respiratory tract (erosive rhinitis and sinusitis, saddle-nose deformity), lungs (nodules, alveolar hemorrhage) and/or kidney. Biopsy of an affected organ can support the diagnosis, as can detection in serum of ANCA with a cytoplasmic labelling pattern (cANCA) by immunofluorescence and proteinase 3 (PR3) specificity by ELISA. Current therapy, based on a staged induction–maintenance strategy, can achieve remission in most patients. However, the toxicity of conventional treatment and relapse rate remain high, which calls for diligent efforts to further optimize therapy. Rituximab is now an alternative to cyclophosphamide, which increases the therapeutic armamentarium for inducing remission, but the optimal subsequent maintenance strategy remains to be defined. Refractory and/or relapsing diseases, as well as several morbidities, such as subglottic stenoses, continue to be challenges.

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Authors and Affiliations

  1. Division of Rheumatology, Vasculitis Clinic, Rebecca McDonald Centre for Arthritis and Autoimmune Diseases, Mount Sinai Hospital, University Health Network, 60 Murray Street, Toronto, ON, M5T 3L9, Canada

    Christian Pagnoux MD, MPH, MSc

  2. Department of Rheumatic and Immunologic Diseases, Center for Vasculitis Care and Research, Cleveland Clinic, 9500 Euclid ave, Cleveland, OH, 44195, USA

    Alexandra Villa-Forte MD, MPH

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  1. Christian Pagnoux MD, MPH, MSc
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  2. Alexandra Villa-Forte MD, MPH
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Correspondence to Christian Pagnoux MD, MPH, MSc .

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  1. Rare Pulmonary Diseases, Hôpital Louis Pradel, Lyon, France

    Vincent Cottin

  2. Rare Pulmonary Diseases, Hôpital Louis Pradel, Lyon, France

    Jean-Francois Cordier

  3. Respiratory Medicine, University of Southampton, Southampton, United Kingdom

    Luca Richeldi

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Pagnoux, C., Villa-Forte, A. (2015). Granulomatosis with Polyangiitis (Wegener’s Granulomatosis). In: Cottin, V., Cordier, JF., Richeldi, L. (eds) Orphan Lung Diseases. Springer, London. https://doi.org/10.1007/978-1-4471-2401-6_9

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