Therapeutic Approaches to Type I Interferonopathies

• Published in: Current rheumatology reports Vol. 20; no. 6; p. 32
• Main Authors: Bienias, Marc, Brück, Normi, Griep, Constanze, Wolf, Christine, Kretschmer, Stefanie, Kind, Barbara, Tüngler, Victoria, Berner, Reinhard, Lee-Kirsch, Min Ae
• Format: Journal Article
• Language: English
• Published: New York Springer US 01.06.2018
• Subjects: Medicine; Medicine & Public Health; Pediatric Rheumatology (S Ozen; Rheumatology; Section Editor; Topical Collection on Pediatric Rheumatology; Autoinflammation; ᅟType I interferonopathies; Therapy
• ISSN: 1523-3774; 1534-6307; 1534-6307
• DOI: 10.1007/s11926-018-0743-3

Purpose of Review To review recent scientific advances and therapeutic approaches in the expanding field of type I interferonopathies. Summary Type I interferonopathies represent a genetically and phenotypically heterogenous group of disorders of the innate immune system caused by constitutive activation of antiviral type I interferon (IFN). Clinically, type I interferonopathies are characterized by autoinflammation and varying degrees of autoimmunity or immunodeficiency. The elucidation of the underlying genetic causes has revealed novel cell-intrinsic mechanisms that protect the organism against inappropriate immune recognition of self nucleic acids by cytosolic nucleic acid sensors. The type I IFN system is subject to a tight and complex regulation. Disturbances of its checks and balances can spark an unwanted immune response causing uncontrolled type I IFN signaling. Novel mechanistic insight into pathways that control the type I IFN system is providing opportunities for targeted therapeutic approaches by repurposing drugs such as Janus kinase inhibitors or reverse transcriptase inhibitors.