Efgartigimod is a human IgG1 antibody Fc fragment that reduces IgG levels through neonatal Fc receptor blockade. This study evaluated whether efgartigimod affects the generation of T-cell-dependent antibodies and cellular immune responses to keyhole limpet hemocyanin (KLH) immunization in non-human primates. Cynomolgus monkeys received efgartigimod or vehicle control intravenously for 11 wk, followed by a recovery phase. KLH challenges occurred during both the dosing phase and the recovery phase. No statistically significant differences emerged in anti-KLH IgM levels between the efgartigimod and control groups. Likewise, comparable KLH-specific T cell responses were observed between groups. Anti-KLH IgG titers were lower in efgartigimod-treated animals compared with controls only after the first boost of KLH, coinciding with decreases in total IgG titers in efgartigimod-treated animals, and returned to baseline levels by the end of the recovery phase. Taken together, these results indicate that efgartigimod does not suppress T-cell-dependent antibody responses or antibody class-switching. The findings of this study are consistent with efgartigimod's pharmacological mechanism of action and suggest that efgartigimod does not impair the generation of effective immune responses.

PI4KA-related disorder is a highly clinically variable condition characterized by neurological (limb spasticity, developmental delay, intellectual disability, seizures, ataxia, nystagmus) and gastrointestinal (inflammatory bowel disease and multiple intestinal atresia) manifestations. Although features consistent with immunodeficiency (autoimmunity/autoinflammation and recurrent infections) have been reported in a subset of patients, the burden of B-cell deficiency and hypogammaglobulinemia has not been extensively investigated. We sought to describe the clinical presentation and manifestations of patients with PI4KA-related disorder and to investigate the metabolic consequences of biallelic PI4KA variants in B cells.

The diversity of the fauna of the late Permian K6 Formation of the Metangula graben of northern Mozambique is coming to light thanks to recent discoveries made by the PaleoMoz Project. Here we describe the first diagnosable gorgonopsian remains from the K6a2 Member of the Metangula graben, specimen PPM2018-7Z. This specimen is a large-bodied gorgonopsian, and shows several features similar to the latest late Permian species Inostrancevia africana. PPM2018-7Z shares various autapomorphies with Inostrancevia africana including the extremely narrowed jugal ventral to the orbit, the pineal foramen being positioned far posteriorly, the anteroposteriorly expanded ventral portion of postorbital bar, the orbit being larger than the temporal fenestra and by having prefrontal rugosities and foramina. The tooth morphology of Inostrancevia africana is here accessed based on the left canine of PPM2018-7Z, which is characterised by low labial ridges, a short mesial carina and thick basal denticles of the mesial carina. PPM2018-7Z is phylogenetically recovered close to Inostrancevia africana within the “Russian clade gorgonopsians”. PPM2018-7Z and Inostrancevia africana do not possess an interorbital ridge that is present in some rubidgeines such as Clelandina and Dinogorgon. Furthermore, PPM2018-7Z and inostranceviines retain a postfrontal with a posterior process that extends posteriorly invading the parietal border, unlike in rubidgeines in which the posterior process of the postfrontal is absent. The presence of Inostrancevia africana in the K6a2 Member of the Metangula graben helps correlating this member to the Daptocephalus Assemblage Zone in the Karoo Basin of South Africa.