Table 1 Demographic details, laboratory results, radiologic findings and clinical description of study patients
Characteristic | Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 |
|---|---|---|---|---|---|
Age at manifestation | 12 years | 8 years | 6 months | 9 years | 16 years |
Sex | Male | Female | Female | Female | Male |
Ethnicity – country of origin | Egypt, Turkey Bukharin | Morocco | Morocco, Persia, Egypt | Morocco | Morocco, Libya |
FMF genetic MEFV mutation | Compound heterozygous M694V, V726A | Heterozygote M694V | Homozygote M694V | Heterozygote M694V | Heterozygote M694V |
Total days of fever ≥ 38 DC | 10 | 4 | 1 | 3 | 1 |
Days of hospitalization | 9 | 12 | 28 | 23 | 6 |
Asymmetric myalgia | Yes | Yes | No | Yes | No |
Corticosteroids treatment | No | Yes | No | Yes | No |
CRP max value (mg/dL) | 27.6 | 15 | 9.4 | 24.6 | 8.14 |
WBC max value (K/microliter) | 19 | 13.3 | 30 | 29.3 | 11 |
ESR max value (mm/h) | 89 | 81 | 79 | ND | 85 |
MRI findings | High T2FS signal within the arm muscles (Triceps, Biceps and Brachialis) compatible with myositis. | High signal with enhancement of the calve muscles. Normal bone marrow. | Confluent muscle edema. Muscular atrophy with fat hyperplasia. | Bilateral calf edema and increased signal intensity of the gastrocnemius muscles | Increased diffuse signal intensity in T2FS of thigh muscles with enhancement after gadolinium injection. |