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Table 1 Patient demographics, disease characteristics and clinical manifestations of PFS patients at canakinumab initiation

From: Reasons for canakinumab initiation among patients with periodic fever syndromes: a retrospective medical chart review from the United States

Characteristics

Overall (N=147)

Children (N=68)

Adults (N=79)

PFS subtypes

CAPS (N=59)

TRAPS (N=40)

HIDS/MKD (N=11)

FMF (N=42)

Age (years), mean (SD)

21.7 (16.1)

9.9 (4.5)

31.9 (15.6)

24.0 (18)

21.4 (14.6)

20.6 (9.8)

18.2 (12.4)

Female, n (%)

63 (42.9)

28 (41.2)

35 (44.3)

23 (39.0)

18 (45.0)

6 (54.5)

19 (45.2)

Body mass-index (for adults only; kg/m2), mean (SD)

24.9 (5.6) [N=62]

-

24.9 (5.6) [N=62]

27.3 (5.9) [N=25]

24.0 (6) [N=19]

25.0 ± 4.8 [N=3]

22.1 ± 2.9 [N=16]

Race/ethnicity, n (%)

 White/Non-Hispanic

103 (70.1)

40 (58.8)

63 (79.7)

46 (78.0)

29 (72.5)

7 (63.6)

25 (59.5)

 Hispanic

23 (15.6)

11 (16.2)

12 (15.2)

10 (16.9)

6 (15.0)

2 (18.2)

6 (14.3)

 Asian/Pacific Islander

8 (5.4)

7 (10.3)

1 (1.3)

1 (1.7)

1 (2.5)

2 (18.2)

4 (9.5)

 Black/Non-Hispanic

7 (4.8)

6 (8.8)

1 (1.3)

2 (3.4)

4 (10.0)

0 (0.0)

1 (2.4)

 Other

6 (4.1)

4 (5.9)

2 (2.5)

0 (0.0)

0 (0.0)

0 (0.0)

6 (14.3)

Insurance type, n (%)

 Commercial/private

127 (86.4)

59 (86.8)

68 (86.1)

51 (86.4)

38 (95.0)

9 (81.8)

32 (76.2)

 Medicare

15 (10.2)

8 (11.8)

7 (8.9)

4 (6.8)

2 (5.0)

2 (18.2)

8 (19.0)

 Medicaid

7 (4.8)

3 (4.4)

4 (5.1)

4 (6.8)

2 (5.0)

1 (9.1)

1 (2.4)

 Military

2 (1.4)

2 (2.9)

0 (0.0)

1 (1.7)

0 (0.0)

0 (0.0)

2 (4.8)

 Other (i.e., Tricare)

1 (0.7)

0 (0.0)

1 (1.3)

1 (1.7)

0 (0.0)

0 (0.0)

0 (0.0)

Primary medical specialty of the physician who prescribed canakinumab, n (%)

 Rheumatology

48 (32.7)

21 (30.9)

27 (34.2)

21 (35.6)

10 (25.0)

3 (27.3)

14 (33.3)

 Dermatology

46 (31.3)

23 (33.8)

23 (29.1)

18 (30.5)

14 (35.0)

5 (45.5)

11 (26.2)

 Immunology

31 (21.1)

18 (26.5)

13 (16.5)

14 (23.7)

3 (7.5)

3 (27.3)

13 (31.0)

 Allergy

22 (15.0)

6 (8.8)

16 (20.3)

6 (10.2)

13 (32.5)

0 (0.0)

4 (9.5)

Primary subspecialty of the physician who prescribed canakinumab, n (%)

 Adult

79 (53.7)

23 (33.8)

56 (70.9)

33 (55.9)

22 (55.0)

3 (27.3)

25 (59.5)

 Pediatrics

68 (46.3)

45 (66.2)

23 (29.1)

26 (44.1)

18 (45.0)

8 (72.7)

17 (40.5)

Disease duration (years) from first diagnosis to canakinumab initiation, mean (SD)

5.2 (6.2)

3.0 (2.5)

7.1 (7.6)

6.3 (6.7)

3.9 (4.2)

6.7 (8.5)

4.7 (5.9)

PFS severity, n (%)

 Mild

33 (22.4)

20 (29.4)

13 (16.5)

10 (16.9)

12 (30)

5 (45.5)

6 (14.3)

 Moderate

102 (69.4)

45 (66.2)

57 (72.2)

44 (74.6)

25 (62.5)

6 (54.5)

31 (73.8)

 Severe

12 (8.2)

3 (4.4)

9 (11.4)

5 (8.5)

3 (7.5)

0 (0.0)

5 (11.9)

Number of attacks/flares per year, n (%)

 <1

7 (4.8)

5 (7.4)

2 (2.5)

5 (8.5)

0 (0.0)

0 (0.0)

2 (4.8)

 1-3

50 (34.0)

19 (27.9)

31 (39.2)

20 (33.9)

18 (45.0)

4 (36.4)

8 (19.0)

 4-6

64 (43.5)

29 (42.6)

35 (44.3)

22 (37.3)

15 (37.5)

6 (54.5)

24 (57.1)

 7-12

20 (13.6)

11 (16.2)

9 (11.4)

8 (13.6)

7 (17.5)

1 (9.1)

5 (11.9)

 >12

4 (2.7)

2 (2.9)

2 (2.5)

2 (3.4)

0 (0.0)

0 (0.0)

3 (7.1)

 Unknown

2 (1.4)

2 (2.9)

0 (0.0)

2 (3.4)

0 (0.0)

0 (0.0)

0 (0.0)

Average duration of attacks/flares, n (%)

 <1 day

14 (9.5)

7 (10.3)

7 (8.9)

4 (6.8)

4 (10.0)

3 (27.3)

3 (7.1)

 1-3 days

62 (42.2)

29 (42.6)

33 (41.8)

29 (49.2)

18 (45.0)

2 (18.2)

16 (38.1)

 4-7 days

54 (36.7)

23 (33.8)

31 (39.2)

18 (30.5)

12 (30.0)

6 (54.5)

20 (47.6)

 >7 days

10 (6.8)

3 (4.4)

7 (8.9)

3 (5.1)

6 (15.0)

0 (0.0)

1 (2.4)

 Unknown

7 (4.8)

6 (8.8)

1 (1.3)

5 (8.5)

0 (0.0)

0 (0.0)

2 (4.8)

PFS clinical manifestations at canakinumab initiation, n (%)

 Fever

116 (78.9)

57 (83.8)

59 (74.7)

41 (69.5)

33 (82.5)

8 (72.7)

38 (90.5)

 Fatigue/malaise

84 (57.1)

36 (52.9)

48 (60.8)

30 (50.8)

20 (50.0)

8 (72.7)

28 (66.7)

 Skin/cutaneous

84 (57.1)

36 (52.9)

48 (60.8)

30 (50.8)

20 (50.0)

8 (72.7)

28 (66.7)

 Musculoskeletal

74 (50.3)

31 (45.6)

43 (54.4)

27 (45.8)

19 (47.5)

6 (54.5)

26 (61.9)

 Gastrointestinal

42 (28.6)

22 (32.4)

20 (25.3)

12 (20.3)

9 (22.5)

6 (54.5)

16 (38.1)

 Mood/behavior

25 (17.0)

12 (17.6)

13 (16.5)

13 (22.0)

7 (17.5)

0 (0.0)

6 (14.3)

 Neurological

9 (6.1)

6 (8.8)

3 (3.8)

4 (6.8)

2 (5.0)

0 (0.0)

3 (7.1)

 Ocular

7 (4.8)

2 (2.9)

5 (6.3)

4 (6.8)

1 (2.5)

1 (9.1)

1 (2.4)

 Lymphoid organs

5 (3.4)

1 (1.5)

4 (5.1)

2 (3.4)

0 (0.0)

2 (18.2)

1 (2.4)

 Cardiorespiratory/circulatory organ

4 (2.7)

2 (2.9)

2 (2.5)

2 (3.4)

1 (2.5)

0 (0.0)

1 (2.4)

 Genitourinary

3 (2.0)

1 (1.5)

2 (2.5)

2 (3.4)

0 (0.0)

0 (0.0)

1 (2.4)

 Complications of PFS

1 (0.7)

1 (1.5)

0 (0.0)

1 (1.7)

0 (0.0)

0 (0.0)

0 (0.0)

 Othera

1 (0.7)

1 (1.5)

0 (0.0)

1 (1.7)

0 (0.0)

0 (0.0)

0 (0.0)

 None of the above

12 (8.2)

7 (10.3)

5 (6.3)

6 (10.2)

4 (10.0)

0 (0.0)

2 (4.8)

  1. CAPS cryopyrin-associated periodic syndromes, FMF familial Mediterranean fever, HIDS hyperimmunoglobulin D syndrome, MKD mevalonate kinase deficiency, PFS periodic fever syndrome, SD standard deviation, TRAPS tumor necrosis factor receptor-associated periodic syndrome
  2. aOther types of clinical manifestations included ‘hearing loss’