This has now been recognized as the genetic mode of inheritance for at least 17 known PIDs, including those associated with previously reported variants in NFKB1. 6, 40, 41, 42 In monogenic causes of PID, incomplete penetrance has been more frequently described in haploinsufficient relative to dominant negative PID disease, having been reported in more than half of the monogenic autosomal dominant haploinsufficient immunologic conditions described.40 The gene discussed is NFKB1; the disease is pelvic inflammatory disease.