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Clinical distinctions of anti-topoisomerase positive limited cutaneous systemic sclerosis in early disease: results from the early Systemic sclerOsis Longitudinal Assessment Registry from Turkey

1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49, 50, 51, 52

 

  1. Ankara University Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Ankara, Turkey. enesyayla@hotmail.com
  2. University of Health Sciences, Adana City Training and Research Hospital, Department of Internal Medicine, Division of Rheumatology, Adana, Turkey.
  3. University of Health Sciences, Adana City Training and Research Hospital, Department of Internal Medicine, Division of Rheumatology, Adana, Turkey.
  4. University of Health Sciences Basaksehir Cam and Sakura City Hospital, Department of Rheumatology, Istanbul, Turkey.
  5. University of Health Sciences Basaksehir Cam and Sakura City Hospital, Department of Rheumatology, Istanbul, Turkey.
  6. Kocaeli University, Department of Internal Medicine, Division of Rheumatology, Kocaeli, Turkey.
  7. Hacettepe University School of Medicine, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey.
  8. Hatay Mustafa Kemal University School of Medicine, Department of Internal Medicine, Division of Rheumatology, Hatay, Turkey.
  9. Ankara University Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Ankara, Turkey.
  10. Etlik City Hospital, Clinic of Rheumatology, Etlik, Ankara, Turkey.
  11. Ufuk University Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Ankara, Turkey.
  12. Gazi University Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Ankara, Turkey.
  13. Gazi University Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Ankara, Turkey.
  14. Department of Rheumatology, Faculty of Medicine, Necmettin Erbakan University, Konya, Turkey.
  15. Istanbul University Cerrahpaşa Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Istanbul, Turkey.
  16. Çukurova University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Adana, Turkey.
  17. Çukurova University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Adana, Turkey.
  18. Süleyman Demiral University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Isparta, Turkey.
  19. Süleyman Demiral University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Isparta, Turkey.
  20. Marmara University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Istanbul, Turkey.
  21. Marmara University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Istanbul, Turkey.
  22. Tekirdağ İsmail Fehmi Cumalıoğlu City Hospital, Clinic of Rheumatology, Tekirdağ, Turkey.
  23. Ankara Bilkent City Hospital, Clinic of Rheumatology, Ankara, Turkey.
  24. Ankara Bilkent City Hospital, Clinic of Rheumatology, Ankara; and Ankara Yıldırım Beyazıt University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Ankara, Turkey.
  25. Ankara Yıldırım Beyazıt University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Ankara, Turkey.
  26. Erciyes University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Kayseri, Turkey.
  27. Erciyes University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Kayseri, Turkey.
  28. Fırat University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Elazığ, Turkey.
  29. Fırat University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Elazığ, Turkey.
  30. Kartal Dr. Lütfi Kırdar City Hospital, Clinic of Rheumatology, Istanbul, Turkey.
  31. Kartal Dr. Lütfi Kırdar City Hospital, Clinic of Rheumatology, Istanbul, Turkey.
  32. Van Training and Research Hospital, Clinic of Rheumatology, Van, Turkey.
  33. Istanbul University, Istanbul Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Istanbul, Turkey.
  34. Sivas Cumhuriyet University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Sivas, Turkey.
  35. Sivas Cumhuriyet University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Sivas, Turkey.
  36. Eskişehir Osmangazi University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Eskişehir, Turkey; and Royal Wolverhampton NHS Trust, Department of Rheumatology, Wolverhampton, UK.
  37. Eskişehir Osmangazi University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Eskişehir, Turkey.
  38. Antalya Training and Research Hospital, Clinic of Rheumatology, Antalya, Turkey.
  39. Antalya City Hospital, Clinic of Rheumatology, Antalya, Turkey.
  40. Gaziantep University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Gaziantep, Turkey.
  41. Gaziantep University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Gaziantep, Turkey.
  42. Koç University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Istanbul, Turkey.
  43. Akdeniz University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Antalya, Turkey.
  44. Dokuz Eylül University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, İzmir, Turkey.
  45. Istanbul University Cerrahpaşa Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Istanbul, Turkey.
  46. Istanbul University, Istanbul Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Istanbul, Turkey.
  47. Istanbul University, Istanbul Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Istanbul, Turkey.
  48. Fırat University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Elazığ, Turkey.
  49. Kocaeli University, Department of Internal Medicine, Division of Rheumatology, Kocaeli, Turkey.
  50. Marmara University, Faculty of Medicine, Division of Rheumatology, Department of Internal Medicine, Istanbul, Turkey.
  51. Etlik City Hospital, Clinic of Rheumatology, Etlik, Ankara, Turkey.
  52. Hacettepe University School of Medicine, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey.

CER19600
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PMID: 42544615 [PubMed]

Received: 06/12/2025
Accepted : 27/02/2026
In Press: 21/07/2026

Abstract

OBJECTIVES:
Anti-topoisomerase I antibody (ATA) is typically associated with diffuse cutaneous systemic sclerosis (dcSSc). However, subset of limited cutaneous SSc (lcSSc) patients also present with ATA positivity. Emerging data suggest that ATA-positive lcSSc may represent a distinct or intermediate clinical phenotype. This study aimed to compare the demographic, clinical, and treatment features of early ATA-positive lcSSc patients with those of ACA-positive lcSSc and ATA-positive dcSSc patients.
METHODS:
Patients were recruited from the multicentre Turkish SOLAR cohort (Systemic sclerOsis Longitudinal Assessment Registry). Among 295 SSc patients screened, 172 were included: 74 ACA-positive lcSSc, 55 ATA-positive lcSSc, and 43 ATA-positive dcSSc. Demographic, clinical, and treatment-related variables were analysed and compared across groups.
RESULTS:
ATA-positive lcSSc patients were younger at the onset of Raynaud’s phenomenon (RP) (p=0.042), the first non-RP symptom (p=0.016), and at diagnosis (p=0.018) compared with ACA-positive lcSSc patients. Interstitial lung disease (ILD) was significantly more frequent in ATA-positive lcSSc (74.5%) than ACA-positive lcSSc (8.1%, p<0.001) and was comparable to ATA-positive dcSSc. Modified Rodnan skin scores were highest in ATA-positive dcSSc but were also significantly elevated in ATA-positive lcSSc (p<0.001). Pitting scars were more frequent in dcSSc. Among patients with ILD, ATA-positive lcSSc and ATA-positive dcSSc showed similar HRCT patterns. ATA-positive lcSSc patients were more frequently treated with glucocorticoids and mycophenolate mofetil than ACA-positive lcSSc, whereas cyclophosphamide was highest in dcSSc.
CONCLUSIONS:
ATA-positive lcSSc patients exhibit a clinically distinct phenotype characterized by a substantial risk of internal organ involvement, despite having less extensive skin disease. Their overlap with dcSSc and divergence from ACA-positive lcSSc highlight the importance of incorporating both skin involvement and serologic subtyping into the early management and risk stratification of SSc.

DOI: https://doi.org/10.55563/clinexprheumatol/lh87cq

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