NOD2 and Yao syndrome: We reaffirm the basic definition of YAOS (OMIM Entry #617321) as a syndrome of episodic fever, rash, arthralgia/arthritis, chronic gastrointestinal symptoms, and sicca-like symptoms and describe novel clinical features identified in a sizeable cohort along with a previously unreported NOD2 variant (13, 14, 16, 17, 21).