Journal
CURRENT RHEUMATOLOGY REPORTS
Volume 20, Issue 6, Pages -Publisher
SPRINGER
DOI: 10.1007/s11926-018-0743-3
Keywords
Type I interferonopathies; Autoinflammation; Therapy
Categories
Funding
- Deutsche Forschungsgemeinschaft [249, LE 1074/4-2, TU 421/1-2]
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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.
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