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Nailfold videocapillaroscopy patterns in systemic sclerosis: implications for cutaneous subsets, disease features and prognostic value for survival

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

 

  1. Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Parc Taulí, Hospital Universitari, Sabadell, Barcelona, and Universitat Autònoma de Barcelona, Spain.
  2. Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain.
  3. Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Universitari Vall d’Hebron, Barcelona, and Universitat Autònoma de Barcelona, Spain. alfredo.guillen@vallhebron.cat
  4. Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain.
  5. Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain.
  6. Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Parc Taulí, Hospital Universitari, Sabadell, Barcelona, and Universitat Autònoma de Barcelona, Spain.
  7. Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Parc Taulí, Hospital Universitari, Sabadell, Barcelona, and Universitat Autònoma de Barcelona, Spain.
  8. Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, spain.
  9. Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Universitari de Bellvitge-IDIBELL, L’Hospitalet de Llobregat, Barcelona, Spain.
  10. Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Universitario de Cabueñes, Gijón, Spain.
  11. Department of Systemic Autoimmune Diseases, Institut Clinic de Medicina i Dermatología, Hospital Universitario Clínic, Barcelona, Spain.
  12. Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Parc Taulí, Hospital Universitari, Sabadell, Barcelona, and Universitat Autònoma de Barcelona, Spain.
  13. Department of Internal Medicine, Hospital Universitario Virgen del Rocío, Sevilla, Spain.
  14. Department of Internal Medicine, Hospital Universitario Miguel Servet, Zaragoza, Spain.
  15. Autoimmune Diseases Research Unit, Department of Internal Medicine, Biocruces Bizkaia Health Research Institute, Hospital Universitario Cruces, University of the Basque Country, Barakaldo, Spain.
  16. Department of Internal Medicine, Unit of Systemic Autoimmune Diseases, Hospital Universitario San Cecilio, Instituto de Investigación Biosanitaria Ibs Granada, Facultad de Medicina Granada, Spain.
  17. Unit of Autoimmune Diseases, Department of Internal Medicine, Hospital Clínico Universitario de Santiago, Santiago de Compostela, A Coruña, Spain.
  18. Department of Internal Medicine, Hospital Universitario Virgen de las Nieves, Granada, Spain.
  19. Department of Internal Medicine, Hospital Universitario La Paz, Madrid, Spain.
  20. Department of Internal Medicine, Hospital Universitario y Politécnico La Fe, Valencia, Spain.
  21. Department of Internal Medicine, Hospital Clinic, Barcelona, Spain.
  22. Department of Internal Medicine, Hospital Clínico Universitario de Salamanca, Universidad de Salamanca-IBSAL, Salamanca, Spain.
  23. Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Consorci Hospitalari de Vic, Vic, Barcelona, Spain.
  24. Department of Internal Medicine, Hospital General San Jorge, Huesca, Spain.
  25. Department of Internal Medicine, Hospital Universitario Fundación Alcorcón, Madrid, Spain.
  26. Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Universitari Vall d’Hebron, Barcelona, and Universitat Autònoma de Barcelona, Spain.
  27. Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Universitari Vall d’Hebron, Barcelona, and Universitat Autònoma de Barcelona, Spain.

the RESCLE Investigators

CER16814
2023 Vol.41, N°8
PI 1695, PF 1703
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PMID: 37534953 [PubMed]

Received: 08/05/2023
Accepted : 25/07/2023
In Press: 01/08/2023
Published: 03/08/2023

Abstract

OBJECTIVES:
To assess the associations and prognostic value of scleroderma patterns by nailfold videocapillaroscopy (NVC) in patients with systemic sclerosis (SSc) and cutaneous subsets.
METHODS:
At baseline, 1356 SSc patients from the RESCLE registry were compared according to the scleroderma pattern as Late pattern and non-Late pattern, which included Early and Active patterns. Patient characteristics, disease features, survival time and causes of death were analysed.
RESULTS:
Late pattern was identified in 540 (39.8%), and non-Late pattern in 816 (60.2%) patients (88% women; 987 lcSSc/251 dcSSc). Late pattern was associated to dcSSc (OR=1.96; p<0.001), interstitial lung disease (ILD) (OR=1.29; p=0.031), and scleroderma renal crisis (OR=3.46; p<0.001). Once the cutaneous subset was disregarded in an alternative analysis, both digital ulcers (DU) (OR=1.29; p<0.037) and anti-topoisomerase I antibodies (OR=1.39; p< 0.036) emerged associated with the Late pattern. By cutaneous subsets, associations with Late pattern were: (1) in dcSSc, acro-osteolysis (OR=2.13; p=0.022), and systolic pulmonary artery pressure >40 mmHg by Doppler echocardiogram (OR=2.24; p<0.001); and (2) in lcSSc, ILD (OR=1.38; p=0.028). Survival was reduced in dcSSc with Late pattern compared to non-Late pattern (p=0.049). Risk factors for SSc mortality in multivariate regression Cox analysis were age at diagnosis (HR=1.03; p<0.001), dcSSc (HR=2.48; p<0.001), DU (HR=1.38; p=0.046), ILD (HR=2.81; p<0.001), and pulmonary arterial hypertension (HR=1.99; p<0.001).
CONCLUSIONS:
SSc patients with Late pattern more frequently present dcSSc and develop more fibrotic and vascular manifestations. Advanced microangiopathy by NVC identifies dcSSc patients at risk of reduced survival due to SSc-related causes.

DOI: https://doi.org/10.55563/clinexprheumatol/8lrofr

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