Significance and innovations
-
In pediatric care setting, the most important reason to consider resistance to colchicine treatment in patients with FMF was a high frequency of attacks.
-
In adult care setting, the most important reason to consider resistance to colchicine treatment was secondary amyloidosis.
-
In both group, digestive intolerance, persistent subclinical inflammation and joint symptoms contributed to colchicine resistance.
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Overall full compliance to colchicine treatment was low (40%), especially in the adult care setting group (22%)
Background
Patients and methods
Patients and setting
Statistical analysis
Results
Study patients
All patients
n = 51 | Group Ia
n = 28 | Group IIb
n = 23 |
p values
| |
---|---|---|---|---|
Age at diagnosis (years) | 7.8 ± 8.7 | 12.2 ± 9.6 | 2.7 ± 2.8 | 0.001 |
Sex | 0.166 | |||
Male | 23 (45%) | 10 (36%) | 13 (57%) | |
Female | 28 (55%) | 18 (64%) | 10 (43%) | |
MEFV mutation
| 0.235 | |||
M694V/M694V
| 27 (51%) | 13 (43%) | 14 (61%) | |
M694V/M694I
| 7 (13%) | 4 (13%) | 3 (13%) | |
M694I/M694I
| 1 (2%) | 0 (0%) | 1 (4%) | |
M680I/M680I
| 1 (2%) | 0 (0%) | 1 (4%) | |
M694V/V726A
| 1 (2%) | 1 (3%) | 0 (0%) | |
M694V/I591T
| 1 (2%) | 1 (3%) | 0 (0%) | |
M694V/E148Q
| 1 (2%) | 1 (3%) | 0 (0%) | |
M726V/M680V
| 1 (2%) | 1 (3%) | 0 (0%) | |
M694V/TRAPS polymorph.
| 1 (2%) | 0 (0%) | 1 (4%) | |
E148Q/I692del/V726A
| 1 (2%) | 0 (0%) | 1 (4%) | |
M694V/-
| 4 (8%) | 1 (3%) | 3 (13%) | |
M694I/-
| 1 (2%) | 1 (3%) | 0 (0%) | |
NA | 4 (7.5%) | 4 (13%) | 0 (0%) | |
Frequency of attacks | 0.019 | |||
> 1/2 weeks | 13 (26%) | 5 (18%) | 8 (35%) | |
> 1/month | 17 (33%) | 7 (25%) | 10 (44%) | |
> 1/3 months, <1/month | 3 (6%) | 1 (4%) | 2 (9%) | |
< 1/3 months, >3/year | 2 (4%) | 1 (4%) | 1 (4%) | |
< 3/year | 1 (2%) | 0 (0%) | 1 (4%) | |
NA | 15 (29%) | 14 (50%) | 1 (4%) | |
Duration of attacks (hr) | 57 ± 22.4 | 67 ± 17 | 50 ± 24 | 0.025 |
NA | 17 (33%) | 13 (46%) | 4 (17%) | |
Delay to treatment (years) | 8.7 ± 12.9 | 15 ± 15.9 | 2.4 ± 2.6 | <0.001 |
Total | 51 | 28 (55%) | 23 (45%) |
Frequency and duration of attacks before and after colchicine treatment
Severity of attacks before and during colchicine treatment
Clinical features before and during colchicine treatment
Population n (%) | Group Ia
n (%) | Group IIb
n (%) |
p values ¥
| |
---|---|---|---|---|
Data available | 45 (88%) | 22 (79%) | 23 (100%) | |
Fever | 44 (83%) | 21 (70%) | 23 (100%) |
0.3
|
Abdominal pain | 33 (73%) | 20 (91%) | 13 (57%) |
0.009
|
Arthralgia | 21 (41%) | 11 (39%) | 10 (44%) |
0.494
|
Asthenia | 2 (4%) | 1 (4%) | 1 (5%) |
0.974
|
Myalgia | 8 (18%) | 2 (9%) | 6 (26%) |
0.136
|
Thoracic pain | 10 (22%) | 6 (27%) | 4 (17%) |
0.4
|
Diarrhoea | 2 (4%) | 1 (5%) | 1 (4%) |
0.974
|
Amyloidosis | 1 (2%) | 1 (5%) | 0 (0%) |
0.301
|
Skin rash | 15 (33%) | 8 (36%) | 7 (30%) |
0.673
|
Arthritis | 6 (13%) | 4 (18%) | 2 (9%) |
0.349
|
Peritonitis | 8 (18%) | 2 (9%) | 6 (26%) |
0.14
|
Vomiting | 2 (4%) | 1 (5%) | 1 (4%) |
0.974
|
Headache | 3 (7%) | 0 (0%) | 3 (13%) |
0.08
|
Symptoms | Population n (%) | Group Ia
n (%) | Group IIb
n (%) |
p value
¥
|
---|---|---|---|---|
Data available | 46 (90%) | 24 (86%) | 22 (96%) | |
Fever | 40 (87%) | 20 (82%) | 20 (91%) | 0.446 |
Abdominal pain | 37 (80%) | 19 (79%) | 18 (82%) | 0.821 |
Arthralgia | 21 (46%) | 11 (46%) | 10 (46%) | 0.979 |
Asthenia | 13 (28%) | 5 (21%) | 8 (36%) | 0.243 |
Myalgia | 10 (22%) | 2 (8%) | 8 (36%) | 0.021 |
Thoracic pain | 9 (20%) | 4 (17%) | 5 (23%) | 0.605 |
Diarrhoea | 9 (20%) | 2 (8%) | 7 (32%) | 0.045 |
Amyloidosis | 9 (20%) | 9 (38%) | 0 (0%) | 0.001 |
Skin rash | 8 (17%) | 2 (8%) | 6 (27%) | 0.090 |
Arthritis | 7 (15%) | 5 (21%) | 2 (9%) | 0.268 |
Peritonitis | 6 (13%) | 3 (13%) | 3 (14%) | 0.909 |
Vomiting | 5 (11%) | 4 (17%) | 1 (5%) | 0.187 |
Headache | 5 (11%) | 1 (4%) | 4 (18%) | 0.127 |
Treatment responses and adherence
Inflammatory markers under colchicine treatment
Determination of inadequate response to colchicine (resistance)
Frequency of attacks
Amyloidosis and renal failure
Symptoms | Population
n (%) | Group Ia
n (%) | Group IIb
n (%) |
p value
¥
|
---|---|---|---|---|
Frequency of attacks | ||||
> 6/year | 42% | 23% | 65% | < 0.08 |
> 4/6 months | 26% | 30% | 22% |
ns
|
Biological inflammation | 45% | 47% | 44% |
ns
|
Intolerance to treatment | 19% | 16% | 22% |
ns
|
Amyloidosis | 22% | 0% |
0.02
| |
Renal failure | 20% | 0% |
0.02
| |
Other reasons | 23 %
| 18% | 26% |
ns
|