Anti-Interferon Autoantibodies in Autoimmune Polyendocrinopathy Syndrome Type 1
Figure 1
Anti-IFN Neutralisation Titres in APS1 Patients at Different Sampling Times
(A) Anti-IFN neutralisation titres in the first and last available serum samples from 51 AIRE-genotyped Finnish patients. We use arrowheads to mark the unusual APS1 patients A–D (detailed in Table 3), and numerals for patients whose samples neutralised both IFN-β and IFN-λ1. Bars indicate geometric mean titres. From first to last available samples (average interval = 16 y), titres were substantially increased, unchanged, or decreased in 19%, 43%, and 38% patients, respectively.
(B) Anti-IFN neutralisation titres in Finnish APS1 patients against IFN-α2, IFN-ω, and IFN-β in the first available sera in relation to the time of diagnosis of CMC. Results are grouped for patients sampled 1 yr prior to diagnosis of CMC (−1), at diagnosis (0), or within the indicated number of years thereafter (0–4, 4–7, 7–10, 10–19, or >19 y). This figure excludes four otherwise typical APS1 patients who never developed overt CMC but who also had high titres against IFN-ω (see text); it includes 63 AIRE-genotyped and 11 untyped patients. For IFN-β, only the positive patients are shown (for clarity). Arrowheads mark unusual APS1 patients: patients A–E (detailed in Table 3); patient x, who currently has only one detectable AIRE mutation; patients y and z, who are two of the three Finnish patients with no detectable AIRE mutations; and patient E, who meets the criteria for APS1 (Table 3), but for whom no neutralising autoantibodies or AIRE mutations have yet been found.