Skip to main content

Table 1 Baseline characteristics of patients initiating canakinumab in FMF, MKD and TRAPS

From: Canakinumab treatment real world evidence in 3 monogenic periodic fever syndromes in 2009–2022: an interim analysis using the French JIR cohort database

 

Overall

< 18 years

≥ 18 years

Previously treated with anakinra

Not previously treated with anakinra

Patient treated after HTA approval

n (%)

n (%)

n (%)

n (%)

n (%)

n (%)

FMF

N = 31

N = 16

N = 15

N = 15

N = 16

N = 20

Demographic characteristics

      

Age (y), median (Q1-Q3)

14.4 (7.7–34.8)

7.7 (5.7–9.7)

37.1 (28.2–44.6)

14.4 (8.4–30.2)

15.6 (7.1–37.6)

16.7 (8.2–32.0)

Female

25 (80.6)

11 (68.7)

14 (93.3)

13 (86.7)

12 (75.0)

16 (80.0)

Disease onset

      

Age (y) at first symptoms, median (Q1-Q3)

3.0 (2.3–4.2)

2.3 (1.0-3.3)

3.4 (3.0–9.0)

2.9 (1.1–4.5)

3.1 (2.6-4.0)

2.8 (2.4–4.1)

Time from first symptoms to diagnosis (y), median (Q1-Q3)

1.3 (0.4–2.8)

1.5 (0.7–2.1)

1.0 (0.3–6.1)

1.6 (1.1–3.7)

0.9 (0.2–2.1)

1.9 (0.7–3.6)

Time from diagnosis to canakinumab initiation (y), median (Q1-Q3)

7.3 (4.3–19.3)

4.7 (2.8–5.5)

21.0 (15.5–33.6)

8.9 (5.1–15.5)

6.9 (3.1–28.2)

8.1 (3.8–16.1)

Genetic status

      

Confirmatory genotype: Homozygous mutations on MEFV Exon 10

29 (93.6)

15 (93.8)

14 (93.3)

15 (100.0)

14 (87.5)

20 (100.0)

Confirmatory genotype: Composite heterozygotes mutations on MEFV gene

2 (6.4)

1 (6.2)

1 (6.7)

0 (0.0)

2 (12.5)

0 (0.0)

Complications/comorbidities

      

AA amyloidosis

3 (9.7)

0 (0.00)

3 (20.0)

3 (20.0)

0 (0.0)

1 (5.0)

Renal impairment

2 (6.4)

0 (0.00)

2 (13.3)

2 (13.3)

0 (0.00)

1 (5.0)

Renal transplantation

1 (3.2)

0 (0.00)

1 (6.7)

1 (6.7)

0 (0.00)

1 (5.0)

Ankylosing spondylitis

2 (6.4)

1 (6.2)

1 (6.7)

1 (6.7)

1 (6.2)

1 (5.0)

Previous treatments

      

Colchicine*

28 (90.3)

15 (93.7)

13 (86.7)

12 (80.0)

16 (100.0)

18 (90.0)

Anakinra

15 (48.4)

8 (50.0)

7 (46.7)

15 (100.0)

0 (0.0)

9 (45.0)

Etanercept

1 (3.2)

1 (6.2)

0 (0.0)

0 (0.0)

1 (3.2)

0 (0.0)

MKD

N = 26

N = 18

N = 8

N = 10

N = 16

N = 7

Demographic characteristics

      

Age (y), median (Q1-Q3)

9.7 (3.8–21.7)

6.2 (3.4–9.8)

24.1 (22.9–42.7)

7.5 (3.3–17.0)

12.4 (4.6–22.8)

9.5 (5.2–26.1)

Female

17 (65.4)

13 (72.2)

4 (50.0)

6 (60.0)

11 (68.7)

5 (71.4)

Disease onset

      

Age (y) at first symptoms, median (Q1-Q3)

0.5 (0.25–1.23)

0.4 (0.2–0.9)

1.3 (0.4–3.8)

0.3 (0.3–1.3)

0.8 (0.2–1.2)

1.0 (0.3–1.2)

Time from first symptoms to diagnosis (y), median (Q1-Q3)

2.8 (1.8–7.8)

2.2 (1.4–3.8)

16.6 (11.1–24.8)

2.5 (1.3–7.5)

3.4 (2.1–8.5)

5.8 (2.0-17.8)

Time from diagnosis to canakinumab start (y), median (Q1-Q3)

2.2 (1.1–9.2)

1.8 (1.1–5.1)

9.1 (2.2–10.3)

2.2 (1.2–4.1)

3.6 (1.0-9.3)

1.6 (0.6–4.4)

Genetic status

      

Confirmatory genotype: homozygous or composite heterozygotes with variant 4 and 5 on MVK gene

21 (80.8)

14 (77.8)

7 (87.5)

8 (80.0)

13 (81.3)

7 (100.0)

Non-confirmatory genotype: others mutations on MVK gene**

5 (19.2)

4 (22.2)

1 (12.5)

2 (20.0)

3 (18.7)

0 (0.0)

Complications/comorbidities

      

AA amyloidosis

0 (0.0)

0 (0.0)

0 (0.0)

0 (0.0)

0 (0.0)

0 (0.0)

Renal impairment

0 (0.0)

0 (0.0)

0 (0.0)

0 (0.0)

0 (0.0)

0 (0.0)

Renal transplantation

0 (0.0)

0 (0.0)

0 (0.0)

0 (0.0)

0 (0.0)

0 (0.0)

Previous treatments

      

Corticosteroid*

9 (34.6)

8 (44.4)

1 (12.5)

3 (30.0)

6 (37.5)

3 (42.9)

NSAIDs*

2 (7.7)

2 (11.1)

0 (0.0)

0 (0.0)

2 (12.5)

0 (0.0)

Anakinra

10 (38.5)

7 (38.9)

3 (37.5)

10 (100.0)

0 (0.0)

3 (42.9)

Etanercept

2 (7.7)

2 (11.1)

0 (0.0)

0 (0.0)

2 (12.5)

0 (0.0)

TRAPS

N = 7

N = 3

N = 4

N = 5

N = 2

N = 4

Demographic characteristics

      

Age (y), median (Q1-Q3)

18.9 (15.1–38.2)

-

-

-

-

-

Female

3 (42.9)

-

-

-

-

-

Disease characteristics

      

Age (y) at first symptoms, median (Q1-Q3)

3.0 (2.8-5.0)

-

-

-

-

-

Time from first symptoms to diagnosis (y), median (Q1-Q3)

11.4 (1.6–29.6)

-

-

-

-

-

Time from diagnosis to canakinumab start (y), median (Q1-Q3)

3.6 (2.2–9.1)

-

-

-

-

-

Genetic status

      

Confirmatory genotype: heterozygotes with variant 4 and 5 on TNFRSF1A gene

5 (71.4)

-

-

-

-

-

Non-confirmatory genotype: others mutations on TNFRSF1A gene***

2 (28.6)

-

-

-

-

-

Complications/comorbidities at canakinumab initiation

      

AA amyloidosis

1 (14.3)

-

-

-

-

-

Renal impairment

1 (14.3)

-

-

-

-

-

Renal transplantation

1 (14.3)

-

-

-

-

-

Previous treatments

      

Corticosteroid*

1 (14.3)

-

-

-

-

-

NSAIDs*

0 (0.0)

-

-

-

-

-

DMARDs*

1 (14.3)

-

-

-

-

-

Anakinra

5 (71.4)

-

-

-

-

-

Etanercept

0 (0.0)

-

-

-

-

-

  1. DMARDs: Disease-modifying antirheumatic drugs; FMF: Familial Mediterranean Fever, HTA: Heath technology assessment; MKD: Mevalonate Kinase Deficiency; NSAID: Non-steroidal anti-inflammatory drugs; TRAPS: Tumor necrosis factor Receptor Associated Periodic Syndrome
  2. *Within 3 months prior to canakinumab initiation
  3. **The other mutations identified in the 5 MKD with non-confirmatory genotype were the following: Val377Ile/Asp204Glu, Val377Ile/ Gly335Ala, Val377Ile/del 631-633GAG, Val377Ile/Gly335Ala and Val377Ile/wt
  4. ***The other mutations identified in the 2 TRAPS with non-confirmatory genotype were the following: Asp41Glu/wt and Arg121Gln/wt
Back to article page

Arthritis Research & Therapy

ISSN: 1478-6362

Contact us