The remainder of this review will focus on four different fungal pathogens that can have severe presentations in association with the presence of specific ACAAs: (1) Talaromyces marneffei infection and anti-IFN-γ, (2) histoplasmosis and anti-IFN-γ, (3) Cryptococcus gattii infection and anti-GM-CSF, and (4) chronic mucocutaneous candidiasis and anti-IL-17A/F (IL-22). The gene discussed is CSF2; the disease is histoplasmosis.