Table 1.
The comparison of symptoms and treatment between East Asia versus other countries.
| Countries | p-value | ||
|---|---|---|---|
| East Asia | without East Asia | ||
| Counts of family | 39 | 23 | |
| Counts of Patients | 74 | 51 | |
| Number of male patients: n/total patients (%) | 35/73 (47.9%) | 15/51 (29.4%) | |
| Range of onset age | neonatal period to 32 y | 2 mo to 29 y | |
| Symptoms: n/total patients (%) | |||
| Recurrent stomatitis | 57/71 (80.2%) | 42/51 (82.6%) | 0.819 |
| Cutaneous lesions | 23/71 (32.4%) | 27/51 (52.9%) | 0.026 |
| Ocular symptoms | 3/71 (4.2%) | 5/51 (9.8%) | 0.277 |
| Genital ulcers | 27/71 (38.0%) | 34/51 (66.7%) | 0.003 |
| Arthritis | 23/71 (32.4%) | 22/51 (43.1%) | 0.257 |
| Abdominal symptoms | 40/71 (56.3%) | 28/51 (54.9%) | >1.000 |
| Cardiovascular lesion | 2/71 (2.8%) | 5/51 (9.8%) | 0.128 |
| Central nervous system symptoms | 3/71 (4.2%) | 2/51 (3.9%) | >1.000 |
| Pathergy | 2/71 (2.8%) | 4/51 (7.8%) | 0.235 |
| Recurrent fever | 50/71 (70.4%) | 19/51 (37.3%) | <0.001 |
| Criteria of ISGFBD 1990 | |||
| fulfil/total patients (%) | 18/71 (25.4%) | 19/51 (37.3%) | 0.169 |
| Number of autoimmune diseases and/or autoantibodies | 21/72 (29.2%) | 30/51 (58.8%) | 0.002 |
| Development of autoimmune diseases | Autoimmune thyroid disease, AIH, SLE, ALPS-U, PsA, Detection of autoantibodies | Autoimmune thyroid disease, SLE, ITP, type 1 DM, PsA, Detection of autoantibodies | |
| Symptoms that may be associated with HA20 without autoimmune diseases | IgA vasculitis, CH, IP, Lymphadenitis, Nephrotic syndrome, Aseptic meningitis, DD, MAS, HL, Craniopharyngioma, BCG dermatitis, Chronic active EBV infection | IgA vasculitis, CH, IP, Lymphadenitis, Cerebral infraction, Pancytopenia, IgG2 and IgG4 deficiency, Recurrent infection, Chronic active EBV infection, KD like Coronary vasculitis | |
| Treatment: Number of used patients (%) | |||
| Colchicine | 18 (24.3%) | 18 (35.3%) | |
| Steroid | 34 (45.9%) | 19 (37.3%) | |
| Anti-TNF-α agents | 21 (28.4%) | 14 (27.5%) | |
| (infliximab, adalimumab, etanercept) | |||
| Anti-IL-1 agents | 1 (1.4%) | 10 (19.6%) | |
| (anakinra, canakinumab, rilonacept) | |||
| Tocilizumab | 3 (4.1%) | 3 (5.9%) | |
| Rituximab | 1 (1.4%) | 2 (3.9%) | |
| JAK inhibitor agents | 1 (1.4%) | 6 (11.8%) | |
| (tofacitinib, baricitinib) | |||
| Hematopoietic cell transplantation | 1 (1.4%) | 2 (3.9%) | |
| Sirolimus | 0 (0.0%) | 1 (2.0%) | |
| NSAIDs | 4 (5.4%) | 1 (2.0%) | |
| Methotrexate | 12 (16.2%) | 7 (13.7%) | |
| Cyclophosphamide | 3 (4.1%) | 1 (2.0%) | |
| Cyclosporine A | 6 (8.1%) | 3 (5.9%) | |
| Tacrolimus | 2 (2.7%) | 1 (2.0%) | |
| Mycophenolate mofetil | 4 (5.4%) | 4 (7.8%) | |
| Azathioprine | 3 (4.1%) | 9 (17.6%) | |
| Mesalazine | 12 (16.2%) | 2 (3.9%) | |
| Thalidomide | 8 (10.8%) | 3 (5.9%) | |
| Hydroxychloroquine | 3 (4.1%) | 3 (5.9%) | |
| Dapsone | 0 (0.0%) | 2 (3.9%) | |
| Cimetidine | 2 (2.7%) | 0 (0.0%) | |
| Iguratimod | 1 (1.4%) | 0 (0.0%) | |
| Mizoribine | 1 (1.4%) | 0 (0.0%) | |
| IVIG | 1 (1.4%) | 2 (3.9%) | |
AIH, Autoimmune hepatitis; ALPS-U, autoimmune lymphoproliferative syndrome undefined; BCG, Bacille de Calmette et Guérin; CH, chronic hepatis; DD, developmental disability; DM, diabetes mellitus; EBV, Epstein-Barr Virus; HL, Hodgkin lymphoma; Ig, immunoglobulin; IL, interleukin; IP, interstitial pneumonia; ISGFBD, International Study Group for Behçet’s disease; ITP, idiopathic thrombocytopenic purpura; IVIG, intravenous immunoglobulin; JAK, Janus kinase; KD, Kawasaki disease; MAS, Macrophage activation syndrome; NSAID, nonsteroidal anti-inflammatory drug; PsA, psoriatic arthritis; SLE, systemic lupus erythematosus; TNF, tumor necrosis factor.