When the frequencies of the IPF risk alleles TERT rs2736100A and DSP rs2076295G were compared between AAV subsets and healthy controls, both alleles were significantly increased in microscopic polyangiitis (MPA) (TERT P = 2.3 × 10−4, Pc = 0.0023, odds ratio [OR] 1.38; DSP P = 6.9 × 10−4, Pc = 0.0069, OR 1.32) and MPO-AAV (TERT P = 1.5 × 10−4, Pc = 0.0015, OR 1.33; DSP P = 0.0011, Pc = 0.011, OR 1.26). This evidence concerns the gene DSP and microscopic polyangiitis.