Table 2 FMF features in a cohort of FMF patients evaluated for FD manifestations
From: Screening for Fabry’s disease in a high-risk subpopulation of FMF
Set | Feature | Finding |
|---|---|---|
Demographic traits | Age at study entry—years, mean ± SD | 47 ± 14 |
Age at FMF onset—years, mean ± SD | 13 ± 12 | |
Female sex—n, (%) | 104, (60) | |
Family history of FMF—n, (%) | 128, (74) | |
Manifestations | Abdominal attacks—n, (%) | 163, (95) |
Chest attacks—n, (%) | 109, (63) | |
Arthritis attacks—n, (%) | 107, (62) | |
Erysipeloid rash—n, (%) | 17, (10) | |
Acute scrotum—n, (%) | 5, (3) | |
Short-term muscle attacks—n, (%) | 1, (1) | |
Long-term muscle attacks—n, (%) | 3, (2) | |
Fever only attacks—n, (%) | 12, (7) | |
Leg pain—n, (%) | 115, (67) | |
Associated comorbidities | Inflammatory bowel disease—n, (%) | 3, (2) |
Ankylosing spondylitis—n, (%) | 29, (17) | |
Behçet’s disease—n, (%) | 48, (28) | |
Fibromyalgia—n, (%) | 26, (15) | |
Patients with any comorbidity—n, (%) | 82, (48) | |
Treatment | Colchicine—n, (%) | 164, (95) |
Mean colchicine dose—mg/day, mean ± SD | 1.9 ± 0.84 | |
Colchicine dose ≥ 2 mg/day—n, (%) | 103, (60) | |
Response to colchicine- n, (%) | 52, (30) | |
Biologic drugs—n, (%) | 15, (9) | |
Genetics | Two or more mutations—n, (%) | 98, (57) |
One mutation—n, (%) | 35, (20) | |
No mutations in genetic testing—n, (%) | 28, (16) | |
M694V/M694V—n, (%) | 65, (38) | |
M694V/0—n, (%) | 28, (16) |