The IFN-mediated autoinflammatory diseases CANDLE (chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperatures) and SAVI (stimulator of IFN genes–associated [STING-associated] vasculopathy with onset in infancy) are Mendelian innate immune–dysregulatory disorders that present early in life with fevers, sterile organ inflammation, and a high type I IFN response gene signature (IRS) in peripheral blood cells (1, 2) and are part of the spectrum of conditions termed interferonopathies (3).
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