Author, country, year (ref) | Data source | n/N (%) | F (%) | DPS-DU (%) | ILD (%) | Hearth (%) | PAH (%) | SRC (%) | OES (%) | MS (%) | AA (%) | Entry criteria |
Poormoghim et al, Massachusetts, 20008 | Monocentric registry | 48/556 (8.6)* | 85.4 | 60.4† | 38.6 | 8.8 | 22.9 | 0 | – | 47.9 | Scl-70: 6.25 ACA: 31.2 | No skin thickening on physical examination |
Hunzelmann et al, Germany, 20089 | Multicentric inter-disciplinary registry | 22/1483 (1.4) | 90.9 | 33.3‡ | 59.1 | 13.6 | 13.6 | 22.7 | 72.7 | 45.5 | Scl-70: 40 ACA: 35 | No skin alterations |
Simeón-Aznar et al, Spain, 201210 | Multicentric registry | 67/916 (7.3) | 92.5 | 14.5‡ | 39.1 | 49.3 | 24.6 | 1.4 | 44.9 | 52.2 | Scl-70: 9.5 ACA: 41.5 | 1980 ACR 2001 LeRoy-Medsger |
Marangoni et al, Brazil, 201311 | Retrospective bi-centric database | 79/947 (8.3) | 96.2 | 24.1‡ | 56.9 | – | 22.8 | 2.5 | 83.1 | 46.9 | Scl-70: 7.8 ACA: 41.7 | No skin thickening on physical examination |
Diab et al, Canada, 201412 | Multicentre registry | 27/1417 (1.9) | 88.9 | 29.6† | 25.9 | – | 11.5 | 3.7 | 51.8 | 14.8 | Scl-70: 16.6 ACA: 60 | Physician diagnosis |
Tolosa-Vilella et al, Spain, 201613 | Multicentric registry | 118/1326 (8.9) | 91.5 | 16.1‡ | 27.9 | 29.6 | 25.8 | 1.4 | 19.1 | 21.8 | Scl-70: 12.7 ACA: 46.6 | 1980 ACR 2001 LeRoy-Medsger 2013 ACR-EULAR |
De Almeida Chaves et al, France, 202114 | Retrospective bi-centric database | 33/375 (8.8) | 84.8 | 9.1‡ | 9.1 | 15.1 | 9.1 | 3 | – | 42.4 | Scl-70: 12.1 ACA: 78.7 | 2001 LeRoy-Medsger 2013 ACR-EULAR |
Present study, 2022 | Multicentric registry | 61/1808 (3.3) | 95.1 | 26.2† | 37.7 | 27.4 | 5.9 | 1.6 | 42.6 | 32 .7 | Scl-70: 18.3 ACA: 40 | 2013 ACR- EULAR |
Lowest and highest values for each item are highlighted in bold
*lcSSc+ssSSc.
†DPS+DU.
‡DU.
AA, autoantibodies; ACA, anticentromere; DPS, digital pitting scars; DU, digital ulcer; ILD, interstitial lung disease; lcSSc, limited cutaneous systemic sclerosis; MS, musculoskeletal; OES, oesophageal involvement; PAH, pulmonary hypertension; SRC, scleroderma renal crisis.