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- W3040249622 abstract "Type I interferonopathies are a clinically heterogeneous group of inherited disorders of the innate immune system characterized by constitutive activation of the type I interferon signaling pathway. Cutaneous vasculopathy, lipodystrophy, interstitial lung disease and brain calcifications are the typical manifestations characterizing affected patients. The pathogenic mechanism commonly underlying these disorders is the abnormal activation of immune pathways involved in recognition of non-self-oligonucleotides. These natural defenses against virus consent humans to survive the infections. Target therapies capable of inhibiting type I interferon signaling pathway seem effective in these patients, albeit with possible incomplete responses and severe side effects." @default.
- W3040249622 created "2020-07-10" @default.
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- W3040249622 date "2020-12-01" @default.
- W3040249622 modified "2023-10-18" @default.
- W3040249622 title "Monogenetic causes of chilblains, panniculitis and vasculopathy: the Type I interferonopathies" @default.
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- W3040249622 doi "https://doi.org/10.23736/s0392-0488.20.06709-7" @default.
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