The presentation of AAV is MPA-like in most patients, with articular, cutaneous and renal involvement, as well as IAH in some patients, concordant with the exclusive positivity of anti-MPO antibodies.24 Although ear–nose–throat involvement was present in some patients, no granuloma has been documented on renal pathology, and no patient with SLE/AAV overlap from our study or the literature had anti-PR3 antibodies. Here, MPO is linked to systemic lupus erythematosus.