TABLE 3.
Summary of Autoantibody Positivity in Patients With APECED and With or Without APAH
| Autoantibodies | All Patients (n = 42) | Patients With APAH (n = 17) | Patients Without APAH (n = 25) | HR (95% CI) | P Value |
|---|---|---|---|---|---|
| AADC | 32 (76.2) | 16 (94.1) | 16 (64) | 8.39 (1.1-63.8) | 0.03 |
| CYP1A2 | 5 (11.9) | 5 (29.4) | 0 (0) | 7.89 (2.54-24.49) | 0.004 |
| HDC | 22 (52.3) | 13 (76.5) | 9 (36) | 4.38 (1.4-13.73) | 0.01 |
| TPH | 32 (76.2) | 16 (94.1) | 16 (64) | 6.3 (0.83-47.53) | 0.03 |
| BPIFB1 | 14 (33) | 10 (58.8) | 4 (16) | 3.85 (1.46-10.18) | 0.004 |
| 21-OH | 19 (45.2) | 11 (64.7) | 8 (32) | 2.94 (1.06-8.17) | 0.04 |
| GAD65 | 17 (40.4) | 8 (47.1) | 9 (36) | 1.27 (0.49-3.3) | 0.47 |
| IA-2 | 1 (2.3) | 0 (0) | 1 (4) | 0 (0-inf) | 0.41 |
| IFN-α4 | 41 (97.6) | 17 (100) | 24 (96) | (0-inf) | 0.40 |
| IFN-ω | 40 (95.2) | 16 (94.1) | 24 (96) | 0.84 (0.11-6.36) | 0.78 |
| IL-22 | 38 (90.4) | 16 (94.1) | 22 (88) | 2.18 (0.29-16.44) | 0.51 |
| KCNRG | 6 (14.3) | 3 (17.7) | 3 (12) | 1.05 (0.3-3.68) | 0.61 |
| NALP5 | 24 (57.1) | 12 (70.5) | 12 (48) | 1.93 (0.68-5.5) | 0.15 |
| SCC | 27 (64.2) | 11 (64.7) | 16 (64) | 1.16 (0.42-3.16) | 0.96 |
| SOX10 | 13 (30.1) | 6 (35.3) | 7 (28) | 1.21 (0.45-3.28) | 0.62 |
| TGM4 | 9 (21.4) | 2 (11.8) | 7 (28) | 0.35 (0.08-1.56) | 0.21 |
| 17-OH | 21 (50) | 10 (58.8) | 11 (44) | 1.56 (0.59-4.14) | 0.35 |
Data are presented as n (%) or median (IQR).
Abbreviations: CI, confidence interval; GAD65, glutamic acid decarboxylase 65; HR, hazard ratio; IA-2, tyrosine phosphatase-related islet antigen 2; IL-22, interleukin-22; inf, infinity; KCNRG, potassium channel regulator; NALP5, nucleotide-binding oligomerization domain, leucine rich repeat and pyrin domain containing 5; SCC, side-chain cleavage enzyme; SOX10, sex determining region Y-box 10; TGM4, transglutaminase 4.