Table 1 Main demographic and clinical characteristics of the cohort. Results are presented into absolute values, and as a percentage of the overall cohort in parentheses. The information was gathered for each of the patients, unless otherwise specified. Familial history of autoimmune disease is known for 16 patients. AI, autoimmune disease; DM, dermatomyositis, F/M, female to male, MCP, metacarpophalangeal joint; RP, Raynaud phenomenon
From: An updated overview of Juvenile systemic sclerosis in a French cohort
Demographic characteristics | Clinical presentation | ||
|---|---|---|---|
Sex | Cutaneous involvement | 18/18 (100) | |
Female | 16 (89) | RP | 13 (72) |
Male | 2 (11) | Skin induration | 15 (83) |
F/M ratio | 8:1 | Scleroderma proximal to MCP | 10 (56) |
Familial history of AI disease (n = 16) | 5 (31) | Digital ulcerations | 13 (72) |
1st degree | 3 (19) | Nailfold changes | 13 (72) |
2nd degree | 2 (11) | Telangiectasia | 9 (50) |
Age at diagnosis (years) | Livedo | 5 (28) | |
Median | 10 | Calcinosis | 4 (22) |
Range | 4–15 | Chilblain | 3 (17) |
Duration of symptoms before diag. (months) | Digital infarction | 2 (11) | |
Median | 9 | Articular involvement | 8/18 (44) |
Range | 1–24 | Synovitis | 5 (28) |
Duration of follow-up (years) | Arthritis | 4 (22) | |
Median | 4,5 | Tendinous retraction | 4 (22) |
Clinical presentation | Muscular involvement | 3/18 (17) | |
Limited | 10 (56) | Gastro-intestinal involvement | 14/18 (78) |
Diffuse | 8 (44) | Abdominal pain | 9 (50) |
Overlap syndromes | 5 (28) | Pyrosis / reflux | 8 (44) |
DM | 4 (22) | Diarrhea | 3 (17) |
Lupus | 2 (11) | Vomiting | 2 (11) |
Sjögren | 1 (6) | Digestive occlusion | 1 (6) |
Esophagal dysmotility | 6 (33) | ||
Pulmonary involvement | 14/18 (78) | ||
Dyspnea | 12 (67) | ||
Intersitial lung disease | 7 (39) | ||
Restrictive lung disease | 10 (56) | ||
Low CO-transfer | 9 (50) | ||
Pulmonary arterial hypertension | 1 (6) | ||
Cardiac involvement | 6/18 (33) | ||
Pericarditis | 3 (17) | ||
Myocarditis | 2 (11) | ||
Renal crisis | 0/18 |