Role of Colchicine Treatment in Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS): Real-Life Data from the AIDA Network
Table 2
Demographic and clinical manifestations of patients distinguished on the basis of response to colchicine (complete and partial response versus unresponsive patients).
Colchicine-responsive patients ()
Colchicine-unresponsive patients ()
value
Demographic and clinical information
Age at disease onset in years, mean (IQR)
16.7 (20)
8.2 (9.8)
0.31
Age at diagnosis in years, mean (SD)
0.21
Male/female patients
3/4
7/10
1.00
Patients with pediatric onset-TRAPS, (%)
8 (47.1)
5 (71.4)
0.39
Patients with adult onset-TRAPS, (%)
9 (52.9)
2 (28.6)
High-/low-penetrance mutations (%/%)
5/12 (83.3/66.7)
1/6 (16.7/33.3)
0.62
Family members with symptoms, (%)
7 (41.2)
3 (42.9)
1.00
Relapsing-remitting disease course, (%)
13 (76.5)
5 (71.4)
1.00
Chronic disease course, (%)
4 (23.5)
2 (28.6)
Duration of flares, (IQR)
10.1 (8)
12.33 (18)
0.90
Flares/year, (IQR)
2.29 (1)
3.00 (2)
0.22
Amyloidosis at diagnosis
1 (5.9)
0 (0)
0.54
Clinical manifestations during flares, (%)
Thoracic pain
7 (41.2)
3 (42.9)
1.00
Pericarditis
7 (41.2)
2 (28.6)
0.67
Pleuritis
2 (11.8)
0 (0)
1.00
Abdominal pain
8 (47.1)
5 (71.4)
0.39
Pharyngitis
7 (41.2)
2 (28.6)
0.67
Oral aphthosis
3 (17.6)
3 (42.9)
0.31
Skin rash
7 (41.2)
2 (28.6)
0.78
Lymphadenopathy
5 (29.4)
3 (42.9)
0.65
Myalgia
10 (58.8)
5 (71.4)
0.67
Arthralgia
12 (70.6)
5 (71.4)
1.00
Arthritis
3 (17.6)
2 (28.6)
0.61
Periorbital pain
2 (11.8)
2 (28.6)
0.55
Laboratory findings
Median erythrocyte sedimentation rate, mm/1 h (IQR)
63.3 (51)
31 (37)
0.08
Median C-reactive protein, mg/L (IQR)
8.0 (7.9)
7.1 (10.7)
0.89
Median serum amyloid A, mg/L (IQR)
60.2 (69.4)
59.4 (89.2)
0.37
Proteinuria, (%)
1 (5.9)
0 (0)
0.48
Abbreviations: IQR: interquartile range; : number of patients; SD: standard deviation; TRAPS: tumor necrosis factor associated periodic syndrome.
