Table 1 Demographic features, clinical features, laboratory parameters, and treatment of 23 patients with GSS
Total (n = 23) | Bone onset group (n = 13) | Serositis onset group (n = 10) | P value | |
|---|---|---|---|---|
Age of disease onset (mean ± SD) (year) | 24 ± 11.7 | 24 ± 14.3 | 24 ± 7.7 | 0.855 |
Male/female ratio | 1:1.56 | 1:2.25 | 1:1 | 0.417 |
Disease duration, [median (range)] (year) | 4.5 (1.29–22) | 1.335 (1–8) | 0.131 | |
History of trauma (N, %) | 5 (22%) | 5 (38.5%) | 1 (10.0%) | 0.179 |
Clinical features (N, %) | ||||
Bone pain | 14 (61%) | 12 (92.3%) | 2 (20.0%) | 0.001* |
Skeletal deformity | 3 (13%) | 3 (23%) | 0 | 0.229 |
Pathological fracture | 3 (13%) | 3 (23%) | 0 | 0.229 |
Soft tissue swelling | 4 (17%) | 1 (7.8%) | 3 (30%) | 0.281 |
Decreased exercise tolerance | 9 (39%) | 2 (15.4%) | 7 (70%) | 0.013* |
Dyspnea | 3 (13%) | 1 (7.8%) | 2 (20%) | 0.56 |
Involvement of skeletal system [median (range)] | ||||
No. of bone involvement | 2 (1–3) | 2 (1–3.5) | 2 (1–3) | 0.771 |
Involvement of vascular system (N, %) | ||||
No. of vascular malformations | 9 (39%) | 1 (7.7%) | 8 (0.8) | 0.01* |
Serous effusion | 13 (56%) | 3(23%) | 10 (100%) | 0.00* |
Pleural effusion | 13 (56%) | 3 (23%) | 10 (100%) | 0.00* |
Ascites | 3 (13%) | 0 | 3 (30%) | 0.068 |
Pelvic effusion | 4 (17%) | 0 | 4 (40%) | 0.024* |
Pericardial effusion | 2 (8.7%) | 0 | 2 (20%) | 0.178 |
Laboratory parameters (N, %) | ||||
Leukopenia | 1/23 (4.3%) | 0/13 | 1/10 (10%) | 0.435 |
Anemia | 2/23 (8.7%) | 0/13 | 2/10 (20%) | 0.178 |
Thrombocytopenia | 2/23 (8.7%) | 0/13 | 2/10 (20%) | 0.178 |
Coagulative dysfunction$ | 6/18 (33%) | 1/9 (11%) | 5/9 (56%) | 0.131 |
Hypocalcemia | 7/23 (30.4%) | 5/13 (38%) | 2/10 (20%) | 0.405 |
Hyperphosphatemia$ | 6/19 (31.5%) | 5/11 (45%) | 1/8 (12.5%) | 0.17 |
Elevated β-CTX$ | 7/13 (54%) | 1/7 (14%) | 6/6 (100%) | 0.005* |
Reduced T25-OHD$ | 4/13 (31%) | 0/8 | 4/5 (80%) | 0.007* |
Treatment (N, %) | ||||
Bisphosphonates | 17 (74%) | 10 (78%) | 7 (70%) | 1.00 |
Sirolimus | 3 (13%) | 0 | 3 (30%) | 0.068 |
Surgery | 9 (39%) | 3 (23%) | 6 (60%) | 0.086 |
Radiotherapy | 2(15%) | 2 (15%) | 0 | 0.308 |