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Background: Familial Mediterranean fever (FMF) is an auto-inflammatory disease commonly affects people from Mediterranean basin. It is characterized by acute self-limiting inflammatory attacks of serous membrane. Some recent studies showed diastolic dysfunction in patients with FMF however systolic function was rarely evaluated before.
Objectives: To assess cardiac functions by using speckle tracking echocardiography (STE) in addition to routine measurements and to evaluate whether there is any difference between colchicine responsive patients and those with colchicine-resistant or severe disease.
Methods: Seventy-four FMF patients (57 responsive [60% female and median age 35 [18-63] years], 17 resistant [53% female and median age 31 [20-49] years]) and 74 healthy controls ([53% female and median age 37 [22-55] years]) were included in the study. Patients with cardiac disease or risk factors affecting STE were excluded. Patients who had ≥1 attacks in three months despite ≥2 mg/day od colchicine or who were treated with IL-1 blocking agents were defined as colchicine resistant disease and who had amyloidosis (whom had proteinuria ≥500 mg/day in at least 2 occasion or biopsy-proven amyloidosis) defined as severe disease. Demographic and disease related characteristics were obtained by using a structured form. A detailed echocardiographic examination including the M-mode, Doppler, and STE was applied to whole study population.
Results: There was no significant difference between groups in terms of age and sex. Disease duration was not different between colchine responsive and resistant patients (median duration in resistant diasease was 11 years (1-27), in responsive patients 6 years (0-33) and, p: 0.133). Although ejection fraction was similar among groups, global longitudinal peak systolic strain; a marker of systolic function, was significantly lower in FMF patients in comparison with healthy subjects and this difference was due to colchicine resistant FMF patients (p<0.05). There was no significant difference in echocardiographic parameters between non-resistive and resistive FMF patients (p>0.05) (Table).
Comparison of standard and speckle tracking echocardiographic findings among groups
| Responsive FMF (n: 57) | Resistant FMF (n: 17) | Healthy Controls (n: 74) | |
|---|---|---|---|
| LA | 31 (23-41) | 30 (26-35) | 31 (22-44) |
| LVD | 43 (35-86) | 41 (38-48) | 43 (20-50) |
| LVS | 26 (20-32) | 24 (18-31) | 26 (19-34) |
| EF | 62 (40-77) | 64 (46-73) | 60 (52-70) |
| E | 95 (23-148) | 98 (9-130) | 93 (57-130) |
| A | 83 (26-153) | 79 (8-134) | 81 (57-120) |
| DT | 187 (100-303) * | 183 (100-296) | 161 (100-254) |
| ELateral | 14 (8-29) | 13 (10-23) | 14 (8-23) |
| ALateral | 11 (5-18) | 10 (7-18) | 10 (6-17) |
| ESeptal | 11 (6-20) | 9 (5-16) | 11 (5-16) |
| ASeptal | 10 (5-17) | 10 (5-17) | 9 (4-23) |
| GLS | 20 (8-55) * | 20 (15-32) | 22 (16-33) |
| GCS | 21 (10-29) | 20 (11-42) | 20 (13-30) |
*Significant difference among groups, p<0,05. LA: Left atrium, LVD: left ventricular diastolic diameter, LVS: Left ventricular systolic diamater, EF: Ejection fraction, E: Early diastolic filling A: Atrial filling, DT: Deceleration time, GLS: Global Longitudinal strain GCS: Global circumflex strain
Conclusion: This study shows that there is subclinical myocardial dysfunction in FMF patients. Those patients with severe or colchicine resistant disease has a numerical but statistically different trend toward systolic dysfunction.
Disclosure of Interests: None declared