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We report three unrelated children with influenza A virus (IAV) infection manifesting as acute respiratory distress syndrome (IAV-ARDS), heterozygous for rare TLR3 variants (P554S in two patients and P680L in the third) causing autosomal dominant (AD) TLR3 deficiency. AD TLR3 deficiency can underlie herpes simplex virus-1 (HSV-1) encephalitis (HSE) by impairing cortical neuron-intrinsic type I IFN immunity to HSV-1. TLR3-mutated leukocytes produce normal levels of IFNs in response to IAV. In contrast, TLR3-mutated fibroblasts produce lower levels of IFN-\u03b2 and -\u03bb, and display enhanced viral susceptibility, upon IAV infection. Moreover, the patients\u2019 iPSC-derived pulmonary epithelial cells (PECs) are susceptible to IAV. Treatment with IFN-\u03b12b or IFN-\u03bb1 rescues this phenotype. AD TLR3 deficiency may thus underlie IAV-ARDS by impairing TLR3-dependent, type I and\/or III IFN\u2013mediated, PEC-intrinsic immunity. Its clinical penetrance is incomplete for both IAV-ARDS and HSE, consistent with their typically sporadic nature.<\/jats:p>","DOI":"10.1084\/jem.20181621","type":"journal-article","created":{"date-parts":[[2019,6,19]],"date-time":"2019-06-19T14:45:20Z","timestamp":1560955520000},"page":"2038-2056","update-policy":"https:\/\/doi.org\/10.1084\/jem.crossmarkpolicy","source":"Crossref","is-referenced-by-count":191,"title":["Severe influenza pneumonitis in children with inherited TLR3 deficiency"],"prefix":"10.1084","volume":"216","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2202-0312","authenticated-orcid":true,"given":"Hye Kyung","family":"Lim","sequence":"first","affiliation":[{"name":"St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY 1"},{"name":"Laboratory of Human Genetics of Infectious Diseases, Necker Branch, Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale UMR 1163, Paris, France 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