Cumulative clinical manifestations (%) | Clinical manifestations at last review | |
---|---|---|
Skin | ||
Calcinosis | 11 (44) | 8 (32) |
Skin ulcers | 11 (44) | 1 |
Oedema (Generalized, Periorbital or limb) | 11 (44) | 0 |
Alopecia | 3 (12) | 3 (12) |
Lipodystrophy | 1 (4) | 1 (4) |
Musculoskeletal | 7 (28) | |
Muscle tenderness | 13 (52) | 0 |
Arthritis | 7 (28) | 2 (8) |
Contractures | 5 (20) | 5 (20) |
Osteoporosis with fracture | 1 (4) | 1 (4) |
Endocrine | 9 (36) | 9 (36) |
Growth failure | 8 (32) | 8 (32) |
Diabetes mellitus | 1 (4) | 1 (4) |
Adrenal insufficiency | 1 (4) | 0 |
Respiratory | 3 (12) | 3 (12) |
Interstitial lung disease (ILD) | 2 (8) | 2 (8) |
Dysphonia | 2 (8) | 0 |
Pulmonary hemorrhage | 1 (4) | 1 (4) |
Gastrointestinal | ||
Dysphagia | 5 (20) | 0 |
Abdominal pain or bleeding | 3 (12) | 0 |
Ocular | 2 (8) | 2 (8) |
Cataracts | 2 (8) | 2 (8) |
Cardiovascular | ||
Abnormal capillaroscopy | 15 (60) | NA |
Raynaud’s phenomenon | 6 (24) | NA |
Cardiomyopathy | 1 (4) | 0 |
Nervous system | 3 (12) | 1 (4) |
Seizures; neuropathy | 2 (8) | 0 |
Sensorineural hearing loss | 1 (4) | 1 (4) |
Infections | ||
Fungal (skin) | 7 (28) | NA |
Bacterial (staph aureus 2, gram negative 1) | 3 (12) | NA |
TB | 2 (8) | NA |