Main clinical and serological features for the case series (at the time of the first observation)
No (%) | Number missing | |
1 Generalised complaints | 163/290 (56) | 0 |
2 Joints/tendons | ||
Tendon rubs | 39/290 (13) | 0 |
Myositis | 17/290 (6) | 0 |
Myalgia | 62/290 (21) | 0 |
Muscle weakness | 69/290 (24) | 0 |
Arthralgia | 151/290 (52) | 0 |
Arthritis | 51/290 (18) | 0 |
Flexion contractures | 79/290 (27) | 0 |
Acro-osteolysis | 61/290 (21) | 0 |
3 Skin | ||
Scleredema | 157/290 (54) | 0 |
Skin sclerosis | 274/290 (94) | 0 |
Melanoderma | 100/290 (34) | 0 |
Telangiectasia | 193/290 (67) | 0 |
Calcinosis | 76/290 (26) | 0 |
Atrophic skin ulcers | 117/290 (40) | 0 |
Vasculitic lesions | 19/290 (7) | 0 |
Sicca syndrome6-150 | 77/290 (27) | 0 |
Sicca syndrome6-151 | 165/290 (57) | 0 |
4 Lung | ||
Dyspnoea | 179/290 (62) | 0 |
Interstitial involvement | 127/171 (74) | 119 |
x Ray | 84/132 (64) | 158 |
CT6-154 scan | 89/105 (85) | 185 |
Reduced Tlco 6-154 | 144/285 (51) | 5 |
Reduced FVC6-154 | 69/252 (27) | 38 |
5 Heart | ||
Heart involvement6-152 | 61/290 (21) | 0 |
Heart failure | 9/290 (3) | 0 |
6 Peripheral vascular system | ||
Hypertension | 53/290 (18) | 0 |
Raynaud's phenomenon | 266/290 (92) | 0 |
Digital pitting | 181/290 (62) | 0 |
Digital necrosis | 50/290 (17) | 0 |
7 Gastrointestinal apparatus | ||
Oesophageal involvement | 229/290 (79) | 0 |
Diarrhoea | 31/290 (11) | 0 |
8 Kidney involvement6-153 | 52/290 (18) | 0 |
9 Nervous system | ||
Headache/trigeminal neuropathy | 25/290 (9) | 0 |
Depression | 44/290 (15) | 0 |
10 Laboratory parameters | ||
10.1 Immunological tests: | ||
ANA6-154 | 233/249 (94) | 41 |
ACA6-154 | 57/253 (23) | 37 |
Scl-70 | 116/269 (43) | 21 |
aCL6-154 | 22/215 (10) | 75 |
RF6-154 test | 53/231 (23) | 59 |
10.2 Routine laboratory tests: | ||
ESR6-154 >30 mm/1st h | 71/273 (26) | 17 |
High CRP6-154 | 34/239 (14) | 51 |
High LDH6-154 | 23/210 (11) | 80 |
High CK6-154 | 18/234 (8) | 56 |
Low C3/C4 | 33/233 (14) | 57 |
↵6-150 Both xerostomia and xerophthalmia.
↵6-151 Either xerostomia or xerophthalmia.
↵6-152 Either pericardial involvement or arrhythmias or conduction defects.
↵6-153 Either proteinuria or haematuria or increased creatininaemia.
↵6-154 CT = computed tomography; Tlco = carbon monoxide transfer factor; FVC = forced vital capacity; ANA = antinuclear antibodies; ACA = anticentromere antibodies; aCL = anticardiolipin antibodies; RF = rheumatoid factor; ESR = erythrocyte sedimentation rate; CRP = C reactive protein; LDH = lactic dehydrogenase; CK = creatine kinase.