CD40LG and Decreased total B cell count: Despite B cell lymphopenia, the levels of serum IgM, IgA, and IgG (predominantly IgG1) were elevated while the IgE level was normal (Table 1 and Supplemental Table 1; supplemental material available online with this article; https://doi.org/10.1172/jci.insight.148887DS1), and ANA, pANCA, and PR3-proteinase were also positive (Table 1).