In 2021, Magg et al. [10] reported that OPAID, characterized by recurrent fever, dermatitis, inflammatory bowel disease, alveolar proteinosis, and hypogammaglobulinemia, is caused by gain-of-function variants in OAS1. OAS1 is a type I interferon-induced, intracellular dsRNA sensor that generates 2’−5’-oligoadeylate to activate RNase L. The OAS1 variant protein exhibits dsRNA-independent activity, which alters both transcription and translation during interferon-induced expression and is involved in the complex pathogenesis of dysregulation in monocytes, macrophages, and B cells. Here, OAS1 is linked to Intraalveolar phospholipid accumulation.