Table 4.
Crude incidence rates (/100,000 PYs) for each autoimmune disease per age groups and databases over the period 2003–2014
| Health outcome | Age groups, years | Italy | Denmark | Spain | UK | Europe | US | |||
|---|---|---|---|---|---|---|---|---|---|---|
| ARS | Val Padana | Pedianet | AUH/SSI | BIFAP | RCGP RSC | THIN | ADVANCE | PRISM | ||
| IR (95% CI) | IR (95% CI) | IR (95% CI) | IR (95% CI) | IR (95% CI) | IR (95% CI) | IR (95% CI) | IR (95% CI) | IR | ||
| Acute disseminated encephalomyelitis | 0–1 | 6.8 (5.14–9.06) | 1.4 (0.20–10.17) | 1.2 (0.17–8.76) | 4.5 (3.6–5.6) | 1.3 (0.67–2.49) | 0.8 (0.25–2.45) | 0.6 (0.28–1.11) | 2.86 (2.43–3.37) | 1 |
| 2–4 | 5.8 (4.54–7.53) | 4.8 (2.01–11.57) | 1.0 (0.14–6.86) | 3.8 (3.1–4.6) | 0.2 (0.06–0.90) | 1.2 (0.55–2.73) | 1.2 (0.79–1.79) | 2.71 (2.35–3.13) | 1 | |
| 5–14 | 4.2 (3.58–4.96) | 2.8 (1.50–5.18) | 1.9 (0.73–5.15) | 2.0 (1.8–2.4) | 0.7 (0.47–1.14) | 1.3 (0.83–1.95) | 0.7 (0.52–0.93) | 1.79 (1.63–1.97) | 1 | |
| 15–24 | 12.1 (10.99–13.31) | 10.1 (7.23–14.17) | – | 2.5 (2.3–2.8) | 0.8 (0.56–1.06) | 1.6 (1.06–2.28) | 1.0 (0.76–1.25) | 3.10 (2.90–3.32) | 1 | |
| 25–44 | 20.2 (19.37–21.01) | 22.9 (20.18–25.97) | – | 6.7 (6.3–7.0) | 1.2 (1.01–1.45) | 3.1 (2.57–3.66) | 1.1 (0.96–1.29) | 6.99 (6.79–7.21) | 2 | |
| 45-64 | 15.0 (14.26–15.68) | 14.4 (12.33–16.84) | – | 7.1 (6.7–7.5) | 2.2 (1.87–2.58) | 2.9 (2.42–3.50) | 1.2 (1.08–1.43) | 6.31 (6.11–6.52) | 3 | |
| 65+ | 8.6 (8.02–9.21) | 8.6 (6.86–10.73) | – | 6.4 (5.9–6.8) | 6.8 (6.02–7.59) | 2.5 (1.97–3.23) | 0.9 (0.77–1.16) | 5.34 (5.11–5.58) | 6 | |
| Overall | 13.5 (13.11–13.82) | 13.4 (12.35–14.65) | 2.1 (1.93–2.26) | 5.4 (5.2–5.5) | 2.1 (1.93–2.26) | 2.4 (2.19–2.70) | 1.0 (0.96–1.13) | 5.25 (5.15–5.34) | – | |
| Bell’s palsy | 0–1 | 4.7 (3.34–6.60) | 5.7 (2.15–15.27) | 3.7 (1.19–11.47) | 8.1 (6.82–9.60) | 14.8 (12.20–17.95) | 3.2 (1.79–5.57) | 3.5 (2.69–4.67) | 6.76 (6.08–7.52) | 22 |
| 2–4 | 5.5 (4.20–7.09) | 3.9 (1.45–10.27) | 3.9 (1.45–10.30) | 8.1 (7.09–9.34) | 8.9 (7.14–11.09) | 7.6 (5.47–10.43) | 5.8 (4.86–7.03) | 7.05 (6.45–7.69) | 17 | |
| 5–14 | 6.5 (5.66–7.37) | 6.4 (4.26–9.65) | 8.2 (5.11–13.22) | 10.4 (9.81–11.13) | 19.3 (17.68–20.98) | 12.3 (10.74–14.14) | 13.8 (12.94–14.75) | 11.83 (11.41–12.28) | 24 | |
| 15–24 | 3.2 (2.67–3.87) | 1.5 (0.62–3.58) | – | 12.2 (11.56–12.91) | 35.2 (33.53–36.84) | 22.9 (20.69–25.28) | 26.5 (25.25–27.80) | 19.06 (18.55–19.59) | 40 | |
| 25–44 | 4.9 (4.48–5.29) | 2.1 (1.37–3.16) | – | 21.6 (20.96–22.27) | 42.3 (40.97–43.61) | 29.7 (28.02–31.40) | 33.7 (32.84–34.65) | 24.90 (24.51–25.30) | 90 | |
| 45–64 | 6.9 (6.41–7.37) | 2.8 (1.99–4.01) | – | 24.3 (23.60–25.04) | 51.3 (49.66–53.07) | 36.7 (34.88–38.72) | 40.1 (39.16–41.16) | 28.83 (28.39–29.28) | 121 | |
| 65+ | 10.5 (9.82–11.13) | 4.6 (3.36–6.20) | – | 27.5 (26.49–28.46) | 62.9 (60.52–65.30) | 39.0 (36.58–41.49) | 41.8 (40.59–43.15) | 31.13 (30.57–31.70) | 174 | |
| Overall | 6.7 (6.47–6.97) | 3.3 (2.79–3.94) | 6.1 (4.11–9.15) | 19.6 (19.28–19.93) | 42.4 (41.62–43.10) | 28.9 (28.08–29.83) | 32.1 (31.65–32.58) | 23.84 (23.64–24.05) | – | |
| Guillain-Barré Syndrome | 0–1 | 1.0 (0.47–2.09) | 0.0 | 0.0 | 0.4 (0.17–0.82) | 0.4 (0.14–1.34) | 0.8 (0.25–2.45) | 0.4 (0.19–0.93) | 0.50 (0.34–0.74) | 2 |
| 2–4 | 1.7 (1.03–2.67) | 0.0 | 1.9 (0.48–7.73) | 1.0 (0.68–1.48) | 0.5 (0.17–1.20) | 1.2 (0.55–2.73) | 1.0 (0.67–1.60) | 1.05 (0.84–1.32) | 2 | |
| 5–14 | 0.9 (0.64–1.29) | 1.1 (0.42–2.97) | 0.0 | 0.7 (0.51–0.85) | 0.5 (0.28–0.82) | 0.5 (0.24–0.97) | 0.6 (0.48–0.87) | 0.66 (0.57–0.77) | 2 | |
| 15–24 | 1.5 (1.14–1.96) | 1.2 (0.45–3.17) | – | 1.2 (0.99–1.41) | 0.5 (0.33–0.73) | 1.0 (0.59–1.56) | 1.0 (0.76–1.25) | 1.03 (0.92–1.16) | 3 | |
| 25–44 | 1.7 (1.47–1.94) | 1.8 (1.14–2.81) | – | 2.0 (1.83–2.23) | 1.0 (0.77–1.17) | 1.4 (1.08–1.83) | 1.3 (1.15–1.50) | 1.57 (1.47–1.67) | 6 | |
| 45–64 | 3.1 (2.79–3.43) | 2.8 (1.99–4.01) | – | 3.4 (3.12–3.66) | 1.6 (1.36–1.97) | 2.2 (1.74–2.67) | 2.3 (2.12–2.60) | 2.73 (2.60–2.87) | 12 | |
| 65+ | 4.5 (4.14–5.00) | 5.7 (4.32–7.47) | – | 4.6 (4.19–5.00) | 1.8 (1.43–2.24) | 3.4 (2.75–4.21) | 3.2 (2.86–3.57) | 3.84 (3.65–4.05) | 23 | |
| Overall | 2.6 (2.49–2.80) | 2.8 (2.30–3.35) | 0.5 (0.13–2.04) | 2.4 (2.27–2.49) | 1.1 (0.97–1.21) | 1.8 (1.57–2.00) | 1.8 (1.67–1.89) | 2.06 (2.00–2.12) | – | |
| ITP (broad) | 0–1 | 26.5 (22.92–30.55) | 22.9 (14.05–37.44) | 2.5 (0.62–9.86) | 22.3 (20.08–24.67) | 29.3 (25.56–33.63) | 15.5 (12.04–20.06) | 14.5 (12.63–16.57) | 20.77 (19.54–22.07) | 9 |
| 2–4 | 26.1 (23.19–29.47) | 28.0 (19.43–40.23) | 1.9 (0.48–7.73) | 14.9 (13.47–16.51) | 20.6 (17.84–23.83) | 14.9 (11.86–18.76) | 11.1 (9.69–12.66) | 16.22 (15.30–17.19) | 9 | |
| 5–14 | 10.6 (9.56–11.74) | 6.7 (4.49–9.99) | 3.4 (1.61–7.10) | 5.3 (4.85–5.80) | 15.8 (14.38–17.38) | 4.6 (3.68–5.77) | 5.1 (4.59–5.70) | 7.15 (6.82–7.49) | 5 | |
| 15–24 | 8.7 (7.73–9.69) | 3.3 (1.81–5.92) | – | 4.9 (4.58–5.44) | 22.9 (21.63–24.30) | 9.8 (8.42–11.43) | 6.3 (5.72–6.96) | 9.31 (8.95–9.68) | 6 | |
| 25–44 | 9.5 (8.95–10.07) | 6.5 (5.15–8.26) | – | 7.9 (7.49–8.28) | 30.2 (29.11–31.34) | 13.9 (12.81–15.13) | 9.5 (9.07–10.03) | 12.39 (12.11–12.67) | 9 | |
| 45–64 | 19.9 (19.10–20.74) | 11.7 (9.81–13.87) | – | 15.9 (15.37–16.54) | 66.4 (64.50–68.37) | 24.2 (22.67–25.78) | 17.7 (17.01–18.33) | 23.76 (23.36–24.17) | 12 | |
| 65+ | 47.5 (46.13–48.91) | 22.0 (19.09–25.24) | – | 35.7 (34.63–36.87) | 130.3 (126.90–133.78) | 64.0 (60.91–67.20) | 45.5 (44.17–46.83) | 53.30 (52.57–54.04) | 31 | |
| Overall | 21.9 (21.41–22.31) | 12.1 (11.05–13.23) | 2.8 (1.56–5.07) | 13.9 (13.66–14.20) | 50.0 (49.17–50.78) | 23.8 (22.99–24.58) | 17.3 (16.92–17.60) | 21.76 (21.57–21.96) | – | |
| ITP (narrow) | 0–1 | 5.8 (4.29–7.92) | 4.3 (1.39–13.33) | 1.2 (0.17–8.76) | 13.6 (11.93–15.53) | 4.6 (3.25–6.50) | 7.9 (5.52–11.30) | 8.4 (7.04–10.05) | 9.00 (8.21–9.87) | 9 |
| 2–4 | 8.1 (6.52–10.03) | 7.7 (3.85–15.41) | 1.0 (0.14–6.86) | 11.6 (10.32–13.00) | 3.8 (2.74–5.36) | 10.4 (7.92–13.70) | 9.6 (8.34–11.11) | 9.26 (8.58–10.00) | 9 | |
| 5–14 | 3.2 (2.64–3.84) | 1.7 (0.75–3.72) | 2.4 (1.01–5.81) | 3.6 (3.24–4.02) | 1.4 (1.05–1.96) | 3.0 (2.25–3.93) | 3.7 (3.29–4.23) | 3.25 (3.03–3.49) | 5 | |
| 15–24 | 1.3 (0.99–1.76) | 0.6 (0.15–2.38) | – | 2.6 (2.28–2.90) | 1.0 (0.77–1.34) | 2.8 (2.06–3.67) | 2.3 (1.96–2.72) | 2.05 (1.89–2.23) | 6 | |
| 25–44 | 1.0 (0.82–1.19) | 0.5 (0.20–1.13) | – | 3.3 (3.05–3.56) | 1.1 (0.95–1.38) | 3.0 (2.54–3.63) | 2.6 (2.33–2.83) | 2.28 (2.16–2.40) | 9 | |
| 45–64 | 1.6 (1.35–1.81) | 1.1 (0.62–1.92) | – | 4.7 (4.43–5.07) | 1.4 (1.11–1.67) | 3.7 (3.11–4.32) | 3.7 (3.39–3.99) | 3.26 (3.12–3.41) | 12 | |
| 65+ | 3.2 (2.84–3.55) | 3.0 (2.06–4.38) | – | 10.8 (10.18–11.41) | 3.0 (2.56–3.61) | 8.1 (7.02–9.25) | 8.6 (8.08–9.24) | 7.09 (6.83–7.36) | 31 | |
| Overall | 2.1 (1.99–2.27) | 1.6 (1.25–2.06) | 1.8 (0.85–3.75) | 5.3 (5.08–5.42) | 1.6 (1.49–1.78) | 4.4 (4.07–4.76) | 4.4 (4.24–4.58) | 3.82 (3.74–3.91) | – | |
| Kawasaki | 0–1 | 28.3 (24.63–32.52) | 8.6 (3.86–19.14) | 22.2 (13.99–35.25) | 7.3 (6.10–8.73) | 2.2 (1.30–3.57) | 10.8 (7.95–14.67) | 8.1 (6.72–9.67) | 10.28 (9.43–11.21) | 32 |
| 2–4 | 13.8 (11.66–16.22) | 2.9 (0.93–8.96) | 9.7 (5.20–17.96) | 5.9 (5.05–6.97) | 2.0 (1.28–3.22) | 7.6 (5.47–10.43) | 9.6 (8.34–11.11) | 7.72 (7.09–8.39) | 35 | |
| 5–14 | 2.0 (1.59–2.55) | 0.8 (0.27–2.59) | 1.0 (0.24–3.87) | 1.2 (0.78–1.43) | 0.4 (0.22–0.73) | 2.2 (1.58–3.03) | 1.3 (1.06–1.62) | 1.31 (1.17–1.46) | 15 | |
| 15–24 | 0.1 (0.06–0.34) | 0.0 | – | 0.1 (0.04–0.15) | 0.0 (0.00–0.14) | 0.5 (0.24–0.96) | 0.1 (0.06–0.25) | 0.11 (0.08–0.16) | – | |
| 25–44 | 0.0 (0.01–0.08) | 0.0 | – | 0.1 (0.02–0.09) | 0.0 (0.00–0.08) | 0.0 (0.00–0.18) | 0.0 (0.01–0.08) | 0.03 (0.02–0.05) | – | |
| 45–64 | 0.0 (0.00–0.06) | 0.0 | – | 0.1 (0.05–0.13) | 0.0 (0.00–0.10) | 0.0 | 0.0 (0.01–0.06) | 0.03 (0.02–0.05) | – | |
| 65+ | 0.0 (0.01–0.08) | 0.0 | – | 0.1 (0.02–0.15) | 0.0 | 0.0 | 0.0 (0.02–0.11) | 0.03 (0.02–0.05) | – | |
| Overall | 1.0 (0.93–1.12) | 0.3 (0.17–0.54) | 7.7 (5.36–10.96) | 0.6 (0.52–0.63) | 0.2 (0.12–0.21) | 0.8 (0.71–1.01) | 0.7 (0.64–0.78) | 0.66 (0.63–0.70) | – | |
| Narcolepsy | 0–1 | 0.0 | 0.0 | 0.0 | 0.0 | 0.0 | 0.0 | 0.0 | 0.0 | 1 |
| 2–4 | 0.2 (0.05–0.78) | 0.0 | 1.0 (0.14–6.86) | 0.5 (0.27–0.75) | 0.0 | 0.0 | 0.1 (0.01–0.37) | 0.23 (0.14–0.37) | 1 | |
| 5–14 | 0.4 (0.22–0.65) | 0.8 (0.27–2.59) | 0.5 (0.07–3.43) | 0.8 (0.63–1.00) | 0.2 (0.10–0.49) | 0.4 (0.16–0.81) | 0.4 (0.27–0.59) | 0.53 (0.45–0.63) | 4 | |
| 15–24 | 0.4 (0.26–0.71) | 0.3 (0.04–2.11) | – | 3.1 (2.78–3.46) | 0.7 (0.49–0.96) | 1.4 (0.96–2.14) | 1.3 (1.05–1.62) | 1.77 (1.61–1.93) | 24 | |
| 25–44 | 0.3 (0.19–0.39) | 0.0 | – | 2.5 (2.29–2.73) | 1.0 (0.82–1.23) | 1.3 (1.02–1.74) | 1.2 (1.05–1.40) | 1.40 (1.31–1.49) | 38 | |
| 45–64 | 0.3 (0.24–0.45) | 0.2 (0.05–0.73) | – | 1.6 (1.38–1.75) | 0.7 (0.53–0.93) | 0.8 (0.59–1.18) | 1.0 (0.83–1.14) | 0.97 (0.89–1.06) | 31 | |
| 65+ | 0.5 (0.35–0.64) | 0.1 (0.02–0.79) | – | 1.5 (1.27–1.73) | 0.4 (0.29–0.70) | 0.7 (0.42–1.10) | 1.0 (0.78–1.17) | 0.89 (0.80–0.99) | 27 | |
| Overall | 0.4 (0.30–0.41) | 0.2 (0.09–0.37) | 0.5 (0.13–2.04) | 1.9 (1.76–1.95) | 0.7 (0.59–0.78) | 0.9 (0.77–1.08) | 1.0 (0.88–1.04) | 1.08 (1.04–1.13) | – | |
| Optic neuritis | 0–1 | 0.01 (0.02–1.01) | 0.0 | 0.0 | 0.0 | 0.1 (0.02–1.02) | 0.0 | 0.0 | 0.04 (0.01–0.16) | 2 |
| 2–4 | 0.2 (0.05–0.78) | 0.0 | 0.0 | 0.2 (0.08–0.48) | 0.0 | 0.2 (0.03–1.45) | 0.2 (0.08–0.55) | 0.17 (0.10–0.30) | 3 | |
| 5–14 | 1.6 (1.26–2.13) | 0.6 (0.14–2.23) | 0.0 | 0.8 (0.63–1.00) | 0.6 (0.39–1.00) | 0.9 (0.55–1.51) | 0.8 (0.57–1.00) | 0.88 (0.77–1.01) | 8 | |
| 15–24 | 3.2 (2.62–3.81) | 1.5 (0.62–3.58) | – | 3.8 (3.47–4.23) | 2.1 (1.74–2.55) | 2.4 (1.75–3.26) | 3.4 (2.95–3.86) | 3.21 (3.00–3.43) | 16 | |
| 25–44 | 3.6 (3.32–4.01) | 3.6 (2.61–4.93) | – | 7.6 (7.19–7.97) | 2.3 (2.04–2.66) | 6.3 (5.52–7.08) | 7.2 (6.77–7.60) | 5.77 (5.58–5.96) | 37 | |
| 45–64 | 2.5 (2.25–2.84) | 1.1 (0.62–1.92) | – | 4.6 (4.30–4.93) | 3.0 (2.62–3.44) | 4.6 (3.95–5.30) | 3.7 (3.44–4.05) | 3.67 (3.53–3.85) | 43 | |
| 65+ | 1.8 (1.52–2.06) | 1.7 (1.01–2.77) | – | 1.9 (1.71–2.24) | 3.5 (2.99–4.12) | 2.1 (1.59–2.73) | 1.8 (1.54–2.06) | 2.04 (1.90–2.19) | 52 | |
| Overall | 2.6 (2.40–2.71) | 1.8 (1.46–2.31) | 0.0 | 4.1 (4.00–4.30) | 2.3 (2.17–2.52) | 3.7 (3.37–4.00) | 3.8 (3.60–3.92) | 3.42 (3.34–3.50) | – | |
| Systemic lupus erythematosus | 0–1 | 1.0 (0.47–2.09) | 0.0 | 1.2 (0.17–8.76) | 0.9 (0.55–1.53) | 0.4 (0.14–1.34) | 0.8 (0.25–2.45) | 0.6 (0.33–1.20) | 0.76 (0.55–1.05) | 1 |
| 2–4 | 0.4 (0.15–1.04) | 1.0 (0.14–6.84) | 0.0 | 0.1 (0.02–0.32) | 0.2 (0.06–0.90) | 0.0 | 0.2 (0.08–0.55) | 0.19 (0.11–0.32) | 0.3 | |
| 5–14 | 0.9 (0.61–1.25) | 1.1 (0.42–2.97) | 0.5 (0.07–3.43) | 0.8 (0.64–1.01) | 1.1 (0.77–1.58) | 0.2 (0.06–0.56) | 0.6 (0.44–0.82) | 0.75 (0.65–0.87) | 2 | |
| 15–24 | 2.5 (2.02–3.08) | 1.2 (0.45–3.17) | – | 2.5 (2.24–2.85) | 4.3 (3.77–4.93) | 2.8 (2.11–3.74) | 3.1 (2.71–3.59) | 2.99 (2.79–3.20) | 16 | |
| 25–44 | 4.2 (3.87–4.61) | 4.9 (3.74–6.45) | – | 5.8 (5.43–6.11) | 9.2 (8.62–9.85) | 8.7 (7.87–9.71) | 7.1 (6.70–7.53) | 6.53 (6.33–6.73) | 45 | |
| 45–64 | 4.2 (3.84–4.60) | 4.6 (3.45–6.01) | – | 7.3 (6.91–7.70) | 9.1 (8.43–9.87) | 11.5 (10.52–12.67) | 8.8 (8.36–9.29) | 7.55 (7.33–7.78) | 53 | |
| 65+ | 3.6 (3.25–4.01) | 4.5 (3.27–6.07) | – | 6.9 (6.39–7.38) | 6.0 (5.27–6.75) | 9.3 (8.22–10.62) | 7.3 (6.82–7.89) | 6.18 (5.94–6.44) | 40 | |
| Overall | 3.5 (3.32–3.69) | 3.9 (3.28–4.52) | 0.5 (0.13–2.04) | 4.9 (4.74–5.07) | 6.7 (6.40–6.99) | 7.4 (7.00–7.89) | 6.0 (5.85–6.25) | 5.32 (5.23–5.42) | – | |
| Transverse myelitis | 0–1 | 0.0 | 0.0 | 0.0 | 0.1 (0.01–0.44) | – | 0.3 (0.04–1.87) | 0.6 (0.28–1.11) | 0.23 (0.13–0.43) | 0.2 |
| 2–4 | 0.0 | 1.9 (0.48–7.70) | 0.0 | 0.2 (0.11–0.54) | – | 0.8 (0.31–2.18) | 0.9 (0.55–1.41) | 0.47 (0.33–0.68) | 0.2 | |
| 5–14 | 0.1 (0.01–0.23) | 0.0 | 0.0 | 0.2 (0.15–0.35) | – | 0.7 (0.37–1.20) | 0.6 (0.44–0.82) | 0.34 (0.27–0.43) | 0.2 | |
| 15–24 | 0.3 (0.13–0.50) | 0.3 (0.04–2.11) | – | 0.6 (0.43–0.72) | – | 1.1 (0.68–1.71) | 0.9 (0.70–1.18) | 0.64 (0.55–0.76) | 0.3 | |
| 25–44 | 0.4 (0.27–0.49) | 0.0 | – | 1.3 (1.14–1.46) | – | 2.2 (1.82–2.75) | 2.0 (1.83–2.27) | 1.36 (1.26–1.46) | 1 | |
| 45–64 | 0.5 (0.36–0.61) | 0.5 (0.19–1.09) | – | 1.4 (1.20–1.54) | – | 1.6 (1.21–2.01) | 1.6 (1.44–1.84) | 1.23 (1.14–1.34) | 1 | |
| 65 + | 0.4 (0.29–0.55) | 0.4 (0.17–1.19) | – | 0.9 (0.74–1.10) | – | 1.2 (0.84–1.72) | 0.9 (0.69–1.06) | 0.76 (0.67–0.85) | 1 | |
| Overall | 0.4 (0.30–0.41) | 0.3 (0.17–0.54) | 0.0 | 0.9 (0.74–1.10) | – | 1.5 (1.28–1.68) | 1.4 (1.27–1.46) | 0.97 (0.92–1.01) | – | |
AUH/SSI Aarhus University Hospital/Staten Serum Institute, BIFAP Base de Datos para la Investigación Farmacoepidemiológica en Atención Primaria, ARS Agenzia regionale di sanità, THIN The Health Improvement Network, RCGP RSC Royal College of General Practitioners Research and Surveillance Centre, ADVANCE Accelerated Development of VAccine beNefit-risk Collaboration in Europe, PRISM Post-licensure Rapid Immunization Safety Monitoring programme, IR incidence rate, CI confidence interval, ITP immune thrombocytopenia purpura