Prior studies report variable rates of renal involvement in TBS-GPA, ranging from 28% to 53%.1,3,16 Reported rates of ANCA-negative disease range from 6% to 25% across studies.1–4,16 We suspect our cohort includes a relatively high proportion of patients with non-severe and ANCA-negative disease due to our center’s close collaboration with ENT providers and, therefore, high proportion of referrals of isolated otolaryngologic disease. The gene discussed is GYPA; the disease is Townes-Brocks syndrome.