Wegener's Granulomatosis: a Comprehensive Review

Authors

  • Maria Koukoulaki

DOI:

https://doi.org/10.2015/hc.v1i3.17

Keywords:

Wegener's Granulomatosis, vasculitis, pulmonary, renal, Anti-Neutrophil Cytoplasmic Antibody, Birmingham Vasculitis Activity Score, cyclophosphamide

Abstract

Wegener's Granulomatosis (WG) is a systemic multi-organ disease that is specifically characterised by inflammation of small and medium - sized vessels that could lead to tissue damage. Most commonly affected systems are the upper respiratory tract, the pulmonary, renal and ocular systems. Even though no diagnostic criteria have been established, the Chapel Hill Consensus definitions and the American College of Rheumatology classification criteria are widely used in clinical practice to identify WG. Definite diagnosis is confirmed by biopsy of the affected organ. This article reviews the epidemiology, pathophysiology, clinical manifestations, laboratory markers, diagnosis and disease assessment and, finally, the conventional and therapeutic options in WG.

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