Table 1.
Autoimmune diseases in SAPHO patients and their first-degree relatives.
| Characteristics | Patient |
Relatives |
Prevalence, % | |||||
|---|---|---|---|---|---|---|---|---|
| Number | RR (95 % CI) | Parent | Sibling | Child | Total | RR (95 % CI) | ||
| Total N | 233 | / | 466 | 512 | 249 | 1227 | / | |
| Female sex | 68.2 % | / | 50 % | 48.8 % | 49.3 % | 49.2 % | / | |
| Disease, n (%) | ||||||||
| SAPHO | 233 (100) | NA | 1 (0.21) | 2 (0.39) | 3 (1.20) | 6 (0.49) | NA | NA |
| PPP | 185 (79.40) | / | 7 (1.50) | 5 (0.98) | 2 (0.80) | 14 (1.14) | 9.81 (5.83–16.51) | 0.12 [24] |
| SA | 33 (14.16) | / | 0 (0.00) | 0 (0.00) | 1 (0.40) | 1 (0.08) | 0.18 (0.03–1.30) | 0.45 [25] |
| Psoriasis | 29 (12.44) | / | 10 (2.15) | 11 (2.15) | 0 (0.00) | 21 (1.71) | 6.46 (3.68–11.34) | 0.27 [18] |
| RA | 12 (5.15) | 18.14 (9.41–34.98) | 11 (2.36) | 2 (0.39) | 1 (0.40) | 14 (1.14) | 4.02 (2.14–7.56) | 0.28 [18] |
| SS | 2 (0.86) | 19.96 (4.86–81.92) | 1 (0.21) | 2 (0.39) | 0 (0.00) | 3 (0.24) | 5.69 (1.77–18.30) | 0.043 [20] |
| Vitiligo | 2 (0.86) | 1.54 (0.38–6.19) | 5 (1.07) | 0 (0.00) | 0 (0.00) | 5 (0.41) | 0.73 (0.30–1.79) | 0.56 [21] |
| SLE | 1 (0.43) | 15.10 (1.58–144.64) | 0 (0.00) | 1 (0.20) | 0 (0.00) | 1 (0.08) | 2.87 (0.30–27.55) | 0.03 [18] |
| AIT | 4 (1.72) | 0.36 (0.14–0.95) | 0 (0.00) | 1 (0.20) | 0 (0.00) | 1 (0.08) | 0.02 (0.00–0.12) | 4.80 [22] |
| Scleroderma | 1 (0.43) | 45.29 (2.84–721.91) | 0 (0.00) | 0 (0.00) | 0 (0.00) | 0 (0.00) | NA | 0.01 [18] |
| UC | 4 (1.72) | 343.35 (92.78–1270.58) | 2 (0.43) | 0 (0.00) | 0 (0.00) | 2 (0.16) | 32.60 (6.33–167.87) | 0.005 [19] |
| MCTD | 0 (0.00) | NA | 2 (0.43) | 0 (0.00) | 0 (0.00) | 2 (0.16) | NA | NA |
| DM | 0 (0.00) | NA | 2 (0.43) | 0 (0.00) | 1 (0.40) | 3 (0.24) | 11.11 (3.33–37.08) | 0.022 [18] |
| AS | 0 (0.00) | NA | 1 (0.21) | 1 (0.20) | 0 (0.00) | 2 (0.16) | 0.98 (0.24–3.94) | 0.167 [27] |
| ReA | 0 (0.00) | NA | 1 (0.21) | 2 (0.39) | 0 (0.00) | 3 (0.24) | NA | NA |
| NMO | 0 (0.00) | NA | 1 (0.21) | 0 (0.00) | 0 (0.00) | 1 (0.08) | 9.70 (1.21–77.48) | 0.008 [26] |
| AIHA | 0 (0.00) | NA | 1 (0.21) | 0 (0.00) | 0 (0.00) | 1 (0.08) | NA | NA |
| Any, except SAPHOa | 21 (9.01)b | NA | 43 (9.23) | 20 (3.91) | 5 (2.01) | 68 (5.52) | NA | NA |
PPP: Palmoplantar pustulosis; SA: Severe acne; RA: Rheumatoid arthritis; SS: Sjögren's syndrome; SLE: Systemic lupus erythematosus; AIT: Autoimmune thyroiditis; UC: ulcerative colitis; MTCD: Mixed connective tissue disease; DM: Dermatomyositis; AS: Ankylosing spondylitis; ReA: Reactive arthritis; NMO: Neuromyelitis optical; AIHA: Autoimmune hemolytic anemia; NA: Not acquired.
Patients with two or more autoimmune diseases (except SAPHO) are not counted repeatedly, each person is only counted once.
PPP, SA, and PV are considered cutaneous manifestations of SAPHO rather than concomitant diseases for patients with SAPHO syndrome, so patients who only suffer from one or more diseases in PPP, SA, PV are not included.