Table 1 Patient characteristics

Patients, n

34

25

9

Female, n (%)

17 (50.0)

12 (48.0)

5 (55.6)

1.0000

Median age at onset, years

49 [40-67]

51 [39-66]

65 [38-74]

0.3587

Disease duration, years

5.0 [2.5-6.8]

5.0 [2.0-6.9]

4.5 [2.7-6.7]

0.6819

Time until diagnosis, days

150 [86-265]

151 [83-236]

149 [77-306]

0.9223

Time until treatment, days

153 [84-270]

151 [82-245]

175 [81-378]

0.4944

Autoimmune disease, n (%)

3 (8.8)

3 (12.0)

0 (0.0)

0.5488

 Behçet’s syndrome

2 (5.9)

2 (100.0)

0 (0.0)

Malignancy, n (%)

2 (5.9)

2 (8.0)

0 (0.0)

1.0000

 Myelodysplastic syndromes

1 (2.9)

1 (100.0)

0 (0.0)

 Median number of initial symptoms, n

1 [1-2]

1 [1-2.5]

1 [1-2]

0.385

 Auricular involvement

23 (67.6)

15 (60.0)

8 (88.89)

0.2137

 Tracheobronchial involvement

11 (32.4)

11 (44.0)

0 (0.0)

0.0172

 Articular involvement

10 (29.4)

9 (36.0)

1 (11.1)

0.2250

 Nasal involvement

5 (14.7)

3 (12.0)

2 (22.2)

0.5908

 Eye involvement

4 (11.8)

4 (16.0)

0 (0.0)

0.5536

 Vestibulocochlear involvement

3 (8.8)

2 (8.0)

1 (11.1)

1.0000

Preceding infection

10 (29.4)

8 (32.0)

2 (22.2)

0.6921

Baseline RPDAI

25 [19-35]

30 [19.5-41.5]

23 [13.5-29.5]

0.0786

 WBC n=24^a (/μl)

7200 [5870-8188]

7700 [6080-8600], n=15

7000 [5375-8025], n=9

0.6798

  Neutrophil n=24^a (/μl)

4774 [3691-5971]

5605 [3806-5986], n=15

4613 [3156-5745], n=9

0.4561

  Lymphocyte n=24^a (/μl)

1595 [1248-2029]

1519 [1232-2070], n=15

1798 [1301-2102], n=9

0.4929

  Monocyte n=24^a (/μl)

435 [346-542]

468 [297-589], n=15

378 [272-451], n=9

0.0786

 Hb n=25^a (g/dl)

12.3 [10.8-13.8]

11.6 [10.4-13.9], n=16

13.0 [11.8-13.9], n=9

0.1407

 Plt n=25^a (×10⁴/μl)

30.3 [24.5-38.1]

32.5 [24.5-46.5], n=16

30.3 [24.4-30.7], n=9

0.1407

 CRP n=25^a (mg/dl)

3.3 [0.72-6.85]

4.7 [2.60-9.81], n=16

1.15 [0.10-3.10], n=9

0.0024

 ESR n=22^a (mm/h)

70 [27-101.5]

78 [40-107], n=15

28 [16-83], n=7

0.0722

 IgG n=22^a (mg/dl)

1455 [1140-1836]

1704 [1249-1982], n=16

1159 [984-1431], n=6

0.0325

 Anti-type II collagen antibody (+) n=26^a, n (%)

13 (50.0)

8 (47.1), n=17

5 (55.6), n=9

1.0000

 Glucocorticoid, n (%)

34 (100)

25 (100)

9 (100)

  Initial prednisolone dose, n=33^a (mg)

30 [15-47.5]

30 [15-48.75]

15 [10-40]

0.2717

 Immunosuppressant, n (%)

24 (70.6)

23 (92.0)

2 (22.2)

<0.001

  Methotrexate

20 (83.3)

18 (72.0)

2 (22.2)

0.0168

  Cyclophosphamide

8 (33.3)

8 (32.0)

0 (0.0)

0.0770

  Azathioprine

7 (29.2)

7 (28.0)

0 (0.0)

0.1506

  Tacrolimus

6 (25.0)

5 (20.0)

1 (11.1)

1.0000

  Cyclosporine A

2 (8.3)

2 (8.0)

0 (0.0)

1.0000

 Biologics, n (%)

15 (44.1)

12 (48.0)

3 (33.3)

0.6974

  Tocilizumab

12 (80.0)

10 (40.0)

2 (22.2)

0.4385

  Infliximab

7 (46.7)

6 (24.0)

1 (11.1)

0.6445

  Adalimumab

1 (6.7)

1 (100.0)

0 (0.0)

 Airway intervention, n (%)

6 (17.6)

6 (24.0)

0 (0.0)

0.1622

 Death, n (%)

2 (5.9)

1 (4.0)

1 (11.1)

0.4652