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Rheumatology (Oxford). 2021 Jun 19; doi: 10.1093/rheumatology/keab510. Epub 2021 Jun 19.

Digital pitting scars are associated with a severe disease course and death in systemic sclerosis: a study from the EUSTAR cohort.

Rheumatology (Oxford, England)

Michael Hughes, Calvin Heal, Jörg Henes, Alexandra Balbir-Gurman, Jörg H W Distler, Paolo Airò, Ulf Müller-Ladner, Nicolas Hunzelmann, Eduardo Kerzberg, Lidia Rudnicka, Marie-Elise Truchetet, Simon Stebbings, Yoshiya Tanaka, Anna Maria Hoffman-Vold, Armando Gabrielli, Oliver Distler, Marco Matucci-Cerinic,

Affiliations

  1. Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK.
  2. Centre for Biostatistics, University of Manchester, Manchester, UK.
  3. Centre for Interdisciplinary Clinical Immunology, Rheumatology and Autoinflammatory Diseases and Department of Internal Medicine II (Hematology, Oncology, Immunology and Rheumatology), University Hospital Tuebingen, Tuebingen, Germany.
  4. B. Shine Rheumatology Institute, Rambam Heath Care Campus and Rappaport Faculty of Medicine - Technion, Haifa, Israel.
  5. Department of Internal Medicine 3 and Institute for Clinical Immunology, University Hospital Erlangen-Nuremberg, Erlangen, Germany.
  6. Rheumatology and Clinical Immunology, Spedali Civili, Brescia, Italy.
  7. Department of Rheumatology and Clinical Immunology, Justus-Liebig University Giessen, Campus Kerckhoff, Bad Nauheim, Germany.
  8. Department of Dermatology, University of Cologne, Cologne, Germany.
  9. Servicio de Reumatología, Hospital J. M. Ramos Mejía, Urquiza 609, 1221 Ciudad Autónoma de Buenos Aires, Argentina.
  10. Department of Dermatology, Medical University of Warsaw, Warsaw, Poland.
  11. Department of Rheumatology, Bordeaux University Hospital, Bordeaux, France.
  12. Department of Medicine, Dunedin School of Medicine, University of Otago, Dunedin, New Zealand.
  13. Department of Internal Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan.
  14. Department of Rheumatology, Oslo University Hospital, Oslo, Norway.
  15. Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Ancona, Italy.
  16. Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
  17. Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Florence, Italy.

PMID: 34146098 DOI: 10.1093/rheumatology/keab510

Abstract

OBJECTIVE: Digital pitting scars (DPS) are frequent, but little studied in SSc to date.

METHODS: An analysis of SSc patients enrolled in the EUSTAR database. Primary objectives were to 1) examine DPS prevalence, 2) whether DPS are associated with digital ulcers (DUs) and active digital ischaemia (DUs or gangrene), and 3) describe other associations with DPS including internal organ complications. Secondary objectives were whether DPS are associated with 1) functional impairment, 2) structural microvascular disease, 3) and mortality. Descriptive statistics and parametric/non-parametric tests were used. Binary logistic regression was used to examine the association between DPS and DUs, active digital ischaemia, and mortality.

RESULTS: 9671 patients were included with reported DPS at any time point (n = 4924) or 'never' DPS (n = 4747). The majority (86.9%) were female and mean age was 55.7 years. DPS were associated with longer disease and Raynaud's duration (both P = <0.001). DPS were associated with interstitial lung disease, pulmonary hypertension, conduction blocks, telangiectases, calcinosis (all P = <0.001) and joint synovitis (P = <0.021). Patients were more likely to have more severe capillaroscopic abnormality and greater hand functional impairment. Multivariable logistic regression analyses showed that DPS were associated (OR) with DUs: 22.03 (19.51 to 24.87), active digital ischaemia: 6.30 (5.34 to 7.42), and death: 1.86 (1.48 to 2.36).

CONCLUSION: DPS are associated with a severe disease course including death. The impact of DPS on hand function and ischaemia is significant. The presence of DPS should alert the clinician to a poor prognosis and need to optimise the therapeutic strategy.

© The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: [email protected].

Keywords: Death; Digital ischaemia; Digital pitting scars; Scleroderma; Systemic sclerosis; Vasculopathy

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