Therefore, aberrant SERPINB3 expression as a result of SERPINB3 mutations may result in the accumulation of polymers of misfolded SERPINB3 proteins, overactivation of cathepsin L, inactivation of SERPINA1, dysregulated B cell reactivity, impaired immune homeostasis, disruptive apoptotic mechanism, increased autoantigen burden, dysregulated autoreactive cell proliferation, and subsequently an autoimmune disorder such as AOID with pustular reaction [35,55] (Figure 7). Here, CTSL is linked to Autoimmunity.