Table 4 Clinical characteristics of MEFV mutation-negative FMF patients
From: Familial Mediterranean fever without MEFV mutations: a case–control study
Parameter § | Mutation-negative (N = 47) | Heterogeneous controls (N = 60) | P value * | p.M694V homozygous (N = 57) | P value # |
|---|---|---|---|---|---|
Average length of attack (days) | 2.53 ± 1.36 | 2.86 ± 1.46 | 0.535 | 2.66 ± 1.5 | 0.857 |
Abdominal attacks | 44 (93.6%) | 53 (88.3%) | 0.507 | 50 (87.7%) | 0.509 |
Joint attacks | 18 (38.2%) | 37 (61.6%) | 0.020 | 52 (91.3%) | 0.0001 |
Chest attacks | 18 (38.2%) | 25 (41.6%) | 0.842 | 36 (46.2%) | 0.017 |
ELE | 0 (0%) | 5 (8.3%) | 0.065 | 10 (17.5%) | 0.001 |
Fever alone attacks | 15 (31.9%) | 12 (20%) | 0.183 | 20 (35.1%) | 0.835 |
Trigger for attacks | 27 (57.4%) | 34 (56.6%) | 1.00 | 26/41 (63.4%) | 0.663 |
Exertional leg-pain | 25 (53.1%) | 40 (66.6%) | 0.168 | 47 (82.5%) | 0.002 |
Average colchicine dose (mg/day) | 1.54 ± 0.63 | 1.58 ± 0.53 | 0.727 | 1.9 ± 0.48 | 0.003 |
Intravenous colchicine treatment | 2 (4.2%) | 0 | 0.190 | 5 (8.8%) | 0.452 |
Proteinuria above 300 mg/day | 2 (4.2%) | 1 (1.6%) | 0.580 | 6 (10.5%) | 0.288 |
Amyloidosis | 0 (0%) | 1 (1.6%) | 1.00 | 5 (8.8%) | 0.062 |
Chronic renal failure | 0 (0%) | 1 (1.6%) | 1.00 | 6 (10.5%) | 0.031 |
Kidney disease of any type£ | 2 (4.2%) | 1 (1.6%) | 1.00 | 6 (10.5%) | 0.288 |
Chronic arthritis | 2 (4.2%) | 2 (3.3%) | 1.00 | 11 (19.3%) | 0.034 |
Splenomegaly | 2 (4.2%) | 3 (5%) | 1.00 | 3 (5.3%) | 1.000 |
Anemia of chronic disease | 4 (8.5%) | 8/43 (18.6%) | 0.217 | 3 (5.3%) | 0.698 |
Chronic inflammation (SAA > 10 mg/l) | 3 (6.3%) | 11 (18.3%) | 0.086 | 14/53 (26.4%) | 0.008 |
Combined chronic disease features& | 26 (55.3%) | 44 (73.3%) | 0.06 | 48 (84.2%) | 0.0021 |