Clinical features | Present study | Ferrada et al., 2018 [8] | Dion et al., 2016 [27] | Lin DF et al., 2016a [7] | Oka H et al., 2014 [30] | Mathew et al., 2012 [11] | Trentham et al.,1998 [13] | Zeuner et al., 1997 [9] | Michet et al., 1986[16] | McAdam et al., 1976b [14] |
---|---|---|---|---|---|---|---|---|---|---|
No. of patients | 126 | 304 | 142 | 158 | 239 | 43 | 66 | 62 | 112 | 159 |
Country | North China | International | France | South China | Japan | USA | USA | German | USA | USA |
Study design | Retro | Survey | Retro | Retro | Survey | Retro | Retro | Survey | Retro | Retro + Liter |
Demographic characteristics | ||||||||||
Sex, F/M | 60/66 | 263/41 | 86/56 | 65/93 | 112/127 | 23/20 | 49/17 | 26/36 | 55/57 | 76/83 |
Mean age at onset, years | 47.1 | NA | 43.5 | NA | 52.7 | NA | NA | NA | NA | NA |
Mean age at diagnosis, years | 47.8 | 43.2 | NA | 45.3 | NA | 43 | 46 | 46.6* | 51* | 43.8 |
Follow-up, months | 18 (1–153) | NA | 156! | NA | NA | NA | 96! | 11! | 72 (1–240) | 4.3 ! |
Median delay, months | 5 (0–132) | NA | 12 (0–300) | 14 (0.3–168) | NA | 38.4** | 34.8** | NA | NA | NA |
Clinical features, n (%) | ||||||||||
Auricular chondritis | 75 (59.6) | 262 (86.2) | 127 (89.4) | 107 (67.7) | 187 (78.2) | 38 (88.4) | 62 (93.9) | 58 (93.5) | 95 (84.8) | 141 (88.7) |
Laryngotracheal | 61 (48.4)¶ | NA | 71 (50.0)§ | 109 (68.9) | 119 (49.8)‡ | 16 (37.2) | 44 (66.7) | 19 (30.6) | 53 (47.3) | 89 (55.9) |
Laryngeal | 20 (15.9) | 180 (59.2) | 61 (43.0) | 45/128 (35.2)† | 47 (19.7) | NA | NA | NA | 19 (16.9) | NA |
Tracheobronchial | 52 (41.3) | 225 (74.0) | 32 (22.5) | NA | 97 (40.6) | NA | NA | NA | NA | NA |
Ocular inflammation | 34 (26.9) | 151 (49.7) | 80 (56.3) | 70 (44.3) | 109 (45.6) | 23 (53.5) | 37 (56.1) | 31 (50.0) | 57 (50.9) | 104 (65.4) |
Fever | 26 (20.7) | NA | 43 (30.3) | NA | NA | NA | NA | 15 (24.2) | 44 (39.3) | NA |
Nasal chondritis | 22 (17.5) | 193 (63.5) | 89 (62.7) | 85 (53.8) | 94 (39.3) | 15 (34.8) | 32 (48.5) | 35 (56.5) | 60 (53.6) | 115 (72.3) |
Arthritis | 23 (18.3) | 250 (82.2) | 47 (33.1) | 88 (55.7) | 92 (38.5) | 26 (60.5) | 56 (84.8) | 33 (53.2) | 58 (51.8) | 121 (76.1) |
Hearing loss | 15 (11.9) | 104 (34.2) | 39 (27.5) | 39 (24.7) | 52 (21.8) | 16 (37.2) | 28 (42.4) | 12 (19.4) | 29 (25.9) | 65 (40.9) |
Costochondritis | 7 (5.6) | 143 (47.0) | 57 (40.1) | 23 (14.6) | NA | NA | NA | NA | NA | 58 (36.5) |
Renal involvement | 0 (0) | NA | 0 (0) | 4/134 (2.5)† | 16 (6.7) | 1 (2.3) | NA | 4 (6.5) | 16 (14.3) | NA |
Neurological involvement | 6 (4.8) | NA | 17 (11.9) | 8/69 (11.6) | 23 (9.6) | NA | NA | 6 (9.7) | NA | 5 (3.1) |
Skin involvement | 2 (1.6) | NA | 40 (28.2) | 17/37 (45.9)† | 32 (13.4) | NA | 25 (37.9) | 15 (24.2) | 31 (27.7) | 26 (16.4) |
Valvulopathy | 1 (0.8) | NA | 31 (21.8) | 3/98 (3.1)† | 5 (2.1)^ | NA | 5 (7.6) | 0 (0) | 7 (6.3) | 14 (8.8) |
Arrhythmia | 13 (10.3) | NA | 13 (9.2) | 1/98 (1.0)† | 0 (0)^ | NA | NA | 2 (3.2) | NA | NA |
MDS | 1 (0.8) | NA | 12 (8.2) | 0 (0) | 4 (1.7) | NA | NA | 4 (6.5) | NA | NA |
Autoimmune diseases | 4 (3.2%) | NA | 31 (21.8) | 17 (10.8) | 11 (4.6) | 11 (25.6) | 13 (19.7) | 22 (35.5) | 38 (33.9) | NA |