Clinical Manifestations

WG is often described in the literature as "limited" or "generalized." Generalized disease is defined by the presence of GN, but does not necessarily indicate more severe disease. For example, patients may develop central nervous system (CNS) involvement, pulmonary hemorrhage, or gut ischemia in the absence of GN and have severe and life-threatening disease. It is therefore best to avoid terms "limited" and "generalized," and instead, note the organ systems involved and severity of illness. WG can progress over time and change from being mild to severe. Renal involvement is present in approximately 20% of patients at onset of disease and in approximately 80% later during the course of disease (Table 1) (7).

The initial symptoms typically involve the ENT region. The nose and sinuses are the most frequently affected sites in the head and neck. Symptoms of nasal mucosa inflammation include crusting, pain, epistaxis, ulceration, altered sense of smell, serosanguineous or purulent discharge, and nasal obstruction. Affected nasal mucosa results in loss of local

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