GPA is a relatively rare and potentially life-threatening disease, which should be considered in the differential diagnosis of unclear necrotizing inflammation affecting any organ, in patients who suffer from seemingly unrelated problems in a variety of organ systems. Testing for ANCA and clinical imaging may be sufficient in many patients but in case of doubts, performing a biopsy is strongly recommended since it may spare unnecessary surgical interventions or even oncological therapy; complete remission is possible if the correct treatment is administered, as in the three reported case.
On the other hand, continuous vigilance and screening for secondary malignancies is needed in patients with established vasculitis, particularly in patients with new pathological findings without a concurrent increase in ANCA or other signs of disease activity.