A diagnosis of granulomatosis with polyangiitis (GPA) (formerly known as Wegener's granulomatosis) should be considered in all patients suspected of having a multisystem inflammatory disease on the basis of the signs and symptoms described below. Although careful history and physical examination are essential to establish disease extent, there is no single symptom or sign that is pathognomonic for the disease. Further evaluation of organ systems that are frequently affected by GPA in the absence of overt symptoms or signs is also required. At a minimum, this includes chest imaging, urinalysis, and urine microscopy.

Positive anti-neutrophil cytoplasmic antibody (ANCA) testing can strongly support the diagnosis in patients with a moderate to high pretest probability of GPA. A positive ANCA test in the setting of the classic triad of typical otorhinolaryngeal, lung, and renal involvement is generally sufficient to make the diagnosis, even in the absence of histological confirmation. However, a negative ANCA test does not exclude the diagnosis, and false positives may occur.

Demonstration of necrotising vasculitis and granulomatous inflammation on tissue biopsy can confirm the diagnosis in the appropriate clinical setting. The differential diagnosis of GPA varies significantly with the pattern, extent, and severity of organ involvement.

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