Session Information
Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
Background/Purpose: Colchicine is the main therapy for familial Mediterranean fever (FMF); however, around 5-10% of FMF patients are colchicine-resistant. Currently there is no standard and validated definition for colchicine resistance. We aimed to compare the existing definitions for colchicine resistance in both adult and pediatric FMF patients to find out the best definition to determine the colchicine-resistant patients.
Methods: 385 FMF patients were evaluated and patients receiving anti-interleukin-1 (IL-1) treatment were included. Nine different definitions (found out after PubMed search for colchicine resistance in FMF) (Table 1) were applied to all patients. Results were re-analyzed after excluding the patients with no clinical attacks but had persistently high acute phase reactants (APRs) and/or amyloidosis.
Results: Among 385 FMF patients 60 (40 (66.7%) adults, 20 (33.3%) children) were colchicine-resistant. The highest percentage of FMF patients fulfilled the definition 5 (93.3%), while definition 9 had the poorest performance (28%) (Table 2). Significantly higher percentage of adult patients were meeting definitions 4 and 6 than pediatric patients (87.5% vs 50%, p=0.002; 75% vs 40%, p=0.008; respectively) (Table 2). After excluding patients without clinical attacks, the highest percentage of patients fulfilled definition 2 (94.4%). We combined the attack frequency definition (> 1 typical episode per 3 months) of definition 2 and presence of amyloidosis /APR definition (increase in at least two out of three acute phase reactants (C-Reactive Protein, Erythrocyte sedimentation rate, and serum amyloid A) of definition 5 to create a new definition. The new criteria set were met by 59 (98.3%) colchicine-resistant FMF patients.
Conclusion: Definition of colchicine resistance is still controversial. Definitions of colchicine-resistant patients with both clinical and laboratory criteria were met by a higher percentage of patients than without laboratory criteria. However, the proper definitions for the attack-free period and persistence of APRs are still lacking.
Table 1. The definitions for colchicine resistance in familial Mediterranean fever (FMF) in the literature
|
Definition 1 |
i) Despite taking 2 mg/day of colchicine, at least 1 episode per month |
|
Definition 2 |
i)Despite taking 2 mg/day colchicine, more than 1 typical episode per 3 months |
|
Definition 3 |
i) Despite receiving 2 mg/day colchicine, 3 or more attacks have been reported in the last 6 months |
|
Definition 4 |
i) Despite the fact that adequate doses of colchicine are taken in children and increased doses of 2 mg/day in adults, at least one episode per month during the following 3 months and increased ESR or increased CRP or increased SAA ii) Presence of amyloidosis iii) Protracted febrile myalgia that needs steroid treatment iiii) Presence of persistent arthritis |
|
Definition 5 |
i) More than 3 episodes in 4-6 months, or more than 6 typical episodes per year, despite adequate doses of colchicine ii) An increase in at least two out of three acute phase reactants (CRP, ESR, and SAA) between attacks |
|
Definition 6 |
i) 1 or more episodes per month despite the use of colchicine at the maximum dose of at least 6 months ii) and/or presence of amyloidosis |
|
Definition 7 |
i) At least 1 episode per month despite taking 2 mg/day colchicine ii) Persistently elevated AFR iii) Organ involvement (especially renal) iiii) Losing job or not continuing to school |
|
Definition 8
|
i) Despite taking 2 mg/day of colchicine (or the maximum dose that can be tolerated), at least 1 episode per month ii) Symptoms continue despite 2 mg/day colchicine intake (or maximum tolerable dose) iii) SAA, ESR and CRP elevation 1.5 times higher than the normal limit between attacks |
|
Definition 9
|
i) Despite 2 mg of colchicine, at least 2 episodes per month and elevation of CRP and/or SAA between attacks |
AFR, acute phase reactants; CRP, C-reactive protein; ESR, erythrocyte sedimentation rate; FMF, familial Mediterranean fever; SAA, serum amyloid A
Table 2. Number of familial Mediterranean fever (FMF) patients defined as colchicine resistant according to different definitions
|
Definitions |
All patients (n=60) |
Adult patients (n=40) |
Pediatric patients (n=20) |
P value |
|
Definition 1, n (%) |
29 (48.3) |
21 (52.5) |
8 (40) |
0.36 |
|
Definition 2, n (%) |
51 (85) |
33 (82.5) |
18 (90) |
0.44 |
|
Definition 3, n (%) |
50 (83.3) |
32 (80) |
18 (90) |
0.32 |
|
Definition 4, n (%) |
45 (75) |
35 (87.5) |
10 (50) |
0.002 |
|
Definition 5, n (%) |
56 (93.3) |
36 (90) |
20 (100) |
0.29 |
|
Definition 6, n (%) |
38 (63.6) |
30 (75) |
8 (40) |
0.008 |
|
Definition 7, n (%) |
52 (86.7) |
37 (92.5) |
15 (75) |
0.10 |
|
Definition 8, n (%) |
54 (90) |
34 (85) |
20 (100) |
0.06 |
|
Definition 9, n (%) |
16 (28.3) |
14 (35) |
2 (10) |
0.06 |
To cite this abstract in AMA style:
Erden A, Batu ED, Sari A, Sonmez HE, Armagan B, Demir S, Fırat E, Bilginer Y, Apras Bilgen S, Karadag O, Kalyoncu U, Kiraz S, Ertenli I, Ozen S, Akdogan A. Which Definition Should be Used to Determine Colchicine Resistance in Patients with Familial Mediterranean Fever? [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/which-definition-should-be-used-to-determine-colchicine-resistance-in-patients-with-familial-mediterranean-fever/. Accessed .« Back to 2017 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/which-definition-should-be-used-to-determine-colchicine-resistance-in-patients-with-familial-mediterranean-fever/