Table 1 Patients characteristics with MCTD and OAS
Clinical features | Results |
|---|---|
N = 16 patients | |
 Median Age (years) | 11.9 (2.4–17) |
 Median Follow-up (years) | 7.9 (2.1–19.6) |
 Girls/Boys % | 88/12 (7/1) |
 Family history of autoimmune disease % | 31 |
 Cutaneous manifestation % | 94 |
 Raynaud’s phenomenon % | 44 |
 Swollen fingers or hands % | 62.5 |
 Articular manifestation (arthalgia) % | 88 |
 Arthritis % | 75 |
 Mucosal manifestations ( oral ulcers) % | 69 |
 Fever % | 69 |
 Proximal muscle weakness and increased muscle enzymes % | 25 |
 Pulmonary manifestations | 31 |
 Serositis | 25 |
 Gastrointestinal manifestations | 25 |
 Pulmonary hypertension | 25 |
 Renal manifestations | 20 |
 Hepatic manifestations | 12.5 |
 Endocrine manifestations | 12.5 |
 Neurological manifestations | 0 |
Biological features | |
 Anemia (Hb < 12 g/dl) % | 56 |
 Thrombocytopenia (< 100,000/mm3) % | 50 |
 Hypergammaglobulinemia % | 75 |
 ESR (mean value, mm after 1 h) % | 70 |
 CRP (mean value, mg/L) % | 39 |
Immunological profile | |
 Anti nuclear antibody > 1/1280% | 100 |
 Anti-U1-RNP % | 62.5 |
 Anti-dsDNA % | 56 |
 Anti-SSA/ Anti-SSB % | 44 |
 Anti-Sm % | 30 |
 Biological APL % | 20 |
 Low C3/C4 | 44 |