Nailfold capillary scleroderma pattern may be associated with disease damage in childhood-onset systemic lupus erythematosus: important lessons from longitudinal follow-up

Lupus Science & Medicine, vol. 9, pp. e000572

Abstract

Objectives To observe if capillary patterns in childhood-onset SLE (cSLE) change over time and find associations between a capillary scleroderma pattern with disease activity, damage or scleroderma-like features. Methods Clinical and (yearly) capillaroscopy data from a longitudinal cohort of patients with cSLE (minimum of four Systemic Lupus International Collaborating Clinics (SLICC) criteria, onset <18 years) were analysed. Disease activity was measured by Systemic Lupus Erythematosus Activity Index (SLEDAI) and disease damage by SLICC Damage Index. A scleroderma pattern was defined according to the ‘fast track algorithm’ from the European League Against Rheumatism Study Group on Microcirculation in Rheumatic Diseases. An abnormal capillary pattern, not matching a scleroderma pattern, was defined as ‘microangiopathy’. Results Our cohort consisted of 53 patients with cSLE with a median disease onset of 14 years (IQR 12.5–15.5 years), median SLEDAI score at diagnosis was 11 (IQR 8–16), median SLEDAI at follow-up was 2 (IQR 1–6). A scleroderma pattern (ever) was seen in 18.9%, while only 13.2% of patients had a normal capillary pattern. Thirty-three patients had follow-up capillaroscopy of which 21.2% showed changes in type of capillary pattern over time. Type of capillary pattern was not associated with disease activity. Raynaud’s phenomenon (ever) was equally distributed among patients with different capillaroscopy patterns (p=0.26). Anti-ribonucleoprotein antibodies (ever) were significantly more detected (Χ2, p=0.016) in the scleroderma pattern subgroup (n=7 of 10, 70%). Already 5 years after disease onset, more than 50% of patients with a scleroderma pattern had SLE-related disease damage (HR 4.5, 95% CI 1.1 to 18.8, p=0.034), but they did not develop clinical features of systemic sclerosis at follow-up. Number of detected fingers with a scleroderma pattern was similar between cSLE, juvenile systemic sclerosis and juvenile undifferentiated connective tissue disease. Conclusion This longitudinal study shows that the majority of capillary patterns in cSLE are abnormal and they can change over time. Irrespective of disease activity, a capillary scleroderma pattern in cSLE may be associated with higher risk of SLE-related disease damage.

Authors 15

1. Dieneke Schonenberg‐Meinema corresponding

   Emma Kinderziekenhuis · Amsterdam University Medical Centers · University of Amsterdam

   Affiliation as printed

   Department of Pediatric Immunology, Rheumatology and Infectious diseases, Emma Children's Hospital, Amsterdam University Medical Centres, University of Amsterdam, Amsterdam, The Netherlands

2. Sandy C. Bergkamp

   Emma Kinderziekenhuis · Amsterdam University Medical Centers · University of Amsterdam

   Affiliation as printed

   Department of Pediatric Immunology, Rheumatology and Infectious diseases, Emma Children's Hospital, Amsterdam University Medical Centres, University of Amsterdam, Amsterdam, The Netherlands

3. Amara Nassar‐Sheikh Rashid

   Zaans Medisch Centrum

   Affiliation as printed

   Department of Pediatrics, Zaans Medisch Centrum, Zaandam, The Netherlands

4. Mariken P. Gruppen

   Emma Kinderziekenhuis · Amsterdam University Medical Centers · University of Amsterdam

   Affiliation as printed

   Department of Pediatric Immunology, Rheumatology and Infectious diseases, Emma Children's Hospital, Amsterdam University Medical Centres, University of Amsterdam, Amsterdam, The Netherlands

5. Maritza Albertina Middelkamp-Hup

   Amsterdam University Medical Centers · University of Amsterdam

   Affiliation as printed

   Department of Dermatology, University of Amsterdam, Amsterdam Universitair Medische Centra, Amsterdam, The Netherlands

6. Wineke Armbrust

   University Medical Center Groningen · University of Groningen · Beatrix Kinderziekenhuis

   Affiliation as printed

   University Medical Centre Groningen, University of Groningen, Department of Pediatric Rheumatology and Immunology, Beatrix Children's Hospital, Groningen, The Netherlands

7. Koert M. Dolman

   Reade · OLVG

   Affiliation as printed

   Department of Pediatric Rheumatology, Reade, Amsterdam, The Netherlands

   Department of Pediatrics, Onze Lieve Vrouwe Gasthuis, Amsterdam, The Netherlands

8. A. Elisabeth Hak

   Amsterdam University Medical Centers · University of Amsterdam

   Affiliation as printed

   Department of Rheumatology and Clinical Immunology, Amsterdam University Medical Centres, University of Amsterdam, Amsterdam Rheumatology and Immunology Center, Amsterdam, The Netherlands

9. Petra C. E. Hissink Muller Aachen Department of Paediatric Rheumatology

   Leiden University Medical Center · Willem-Alexander Kinderziekenhuis

   Affiliation as printed

   Department of Paediatric Rheumatology, Willem Alexander Children’s Hospital, Leiden University Medical Center, Leiden, The Netherlands

   Department of Paediatric Rheumatology, Willem Alexander Children's Hospital, Leiden University Medical Center, Leiden, The Netherlands

10. Marieke van Onna

    Amsterdam University Medical Centers · University of Amsterdam

    Affiliation as printed

    Department of Rheumatology and Clinical Immunology, Amsterdam University Medical Centres, University of Amsterdam, Amsterdam Rheumatology and Immunology Center, Amsterdam, The Netherlands

11. Joost Frans Swart

    Utrecht University · University Medical Center Utrecht · Wilhelmina Children's Hospital

    Affiliation as printed

    Department of Paediatric Immunology, Wilhelmina Children's Hospital, University of Utrecht, University Medical Center Utrecht, Utrecht, The Netherlands

12. Taco Willem Kuijpers

    Emma Kinderziekenhuis · Amsterdam University Medical Centers · University of Amsterdam

    Affiliation as printed

    Department of Pediatric Immunology, Rheumatology and Infectious diseases, Emma Children's Hospital, Amsterdam University Medical Centres, University of Amsterdam, Amsterdam, The Netherlands

13. Sylvia S M Kamphuis

    Erasmus MC · Erasmus MC - Sophia Children’s Hospital

    Affiliation as printed

    Department of Paediatric Rheumatology, Sophia Children's Hospital, Erasmus Medical Center, Rotterdam, The Netherlands

14. Vanessa M. Smith

    Ghent University Hospital · Ghent University

    Affiliation as printed

    Department of Rheumatology, Ghent University Hospital, Ghent, Belgium

    Faculty of Internal Medicine, Ghent University, Ghent, Belgium

15. J. Merlijn van den Berg

    Emma Kinderziekenhuis · Amsterdam University Medical Centers · University of Amsterdam

    Affiliation as printed

    Department of Pediatric Immunology, Rheumatology and Infectious diseases, Emma Children's Hospital, Amsterdam University Medical Centres, University of Amsterdam, Amsterdam, The Netherlands