Table 6

Main clinical and serological features for the case series (at the time of the first observation)

No (%) Number missing
1 Generalised complaints163/290  (56)0
2 Joints/tendons
 Tendon rubs39/290 (13)0
 Myositis17/290 (6)0
 Myalgia62/290 (21)0
 Muscle weakness69/290 (24)0
 Arthralgia151/290 (52)0
 Arthritis51/290 (18)0
 Flexion contractures79/290 (27)0
 Acro-osteolysis61/290 (21)0
3 Skin
 Scleredema157/290 (54)0
 Skin sclerosis274/290 (94)0
 Melanoderma100/290 (34)0
 Telangiectasia193/290 (67)0
 Calcinosis76/290 (26)0
 Atrophic skin ulcers117/290 (40)0
 Vasculitic lesions19/290 (7)0
 Sicca syndrome6-150 77/290 (27)0
 Sicca syndrome6-151 165/290 (57)0
4 Lung
 Dyspnoea179/290 (62)0
 Interstitial involvement127/171 (74)119
  x Ray84/132 (64)158
  CT6-154 scan89/105 (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 failure9/290 (3)0
6 Peripheral vascular system
 Hypertension53/290 (18)0
 Raynaud's phenomenon266/290 (92)0
 Digital pitting181/290 (62)0
 Digital necrosis50/290 (17)0
7 Gastrointestinal apparatus
 Oesophageal involvement229/290 (79)0
 Diarrhoea31/290 (11)0
8 Kidney involvement6-153 52/290 (18)0
9 Nervous system
 Headache/trigeminal neuropathy25/290 (9)0
 Depression44/290 (15)0
10 Laboratory parameters
 10.1 Immunological tests:
  ANA6-154 233/249 (94)41
  ACA6-154 57/253 (23)37
  Scl-70116/269 (43)21
  aCL6-154 22/215 (10)75
  RF6-154 test53/231 (23)59
 10.2 Routine laboratory tests:
  ESR6-154 >30 mm/1st h71/273 (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/C433/233 (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.