POS1311 SCLERODERMA PATTERN IN NAILFOLD CAPILLARIES OF (CHILDHOOD-ONSET) SYSTEMIC LUPUS ERYTHEMATOSUS: LESSONS FROM LONGITUDINAL FOLLOW-UP
Authors: D. Schonenberg-Meinema, S. Bergkamp, A. Nassar-Sheikh Rashid, M. Gruppen, A. E. Hak, P. C. E. Hissink Muller, M. Middelkamp, M. Van Onna, T. Kuijpers, V. Smith, J. M. Van den Berg
Journal: Annals of the Rheumatic Diseases (2021)

Background: It has been suggested that a capillary scleroderma pattern in patients with systemic lupus erythematosus (SLE)-patients predisposes for clinical signs of systemic sclerosis (SSc) or overlap disease (1). However, this was previously shown not to be the case in a cross-sectional study in childhood-onset SLE (cSLE) (2). Objectives: To assess if nailfold capillary patterns in cSLE change over time and if a capillary scleroderma pattern is associated with prospective development of clinical SSc-features, higher SLE disease activity or –damage. Methods: Prospective clinical and capillaroscopy data were used from a longitudinal cohort of cSLE patients. Patients with a disease onset before the age of 18 years and diagnosis according to SLICC 2012 criteria were included. Disease activity was defined by SLEDAI score and disease damage by the SLICC damage index. Nailfold images from eight fingers (excluding thumbs) were obtained by videocapillaroscopy. A scleroderma pattern was defined according to the ‘fast track algorithm’ (3). An abnormal capillary pattern that did not match the criteria for a scleroderma pattern was defined as ‘microangiopathy’. Results: Our longitudinal study cohort consisted of n=53 cSLE patients with a median disease onset of 14 years (IQR 12.5-15.5 years) and a median SLEDAI score at diagnosis of 11 (IQR 8-15.5). Clinical follow-up data were available from 0.5-16 years after disease onset. Median disease duration at first capillaroscopy was 17 months (IQR 5.5-51.5 months) and 17/53 (32.1%) of patients had Raynaud’s phenomenon. In total, n=9 (17%) showed a scleroderma pattern (at some point in time), n=37 (70%) had microangiopathy and n=7 (13%) showed a normal capillary pattern. N=27 patients had follow up with capillaroscopy (1-7 times over 1-5 years). In most patients (23/27) we did not observe any change in capillary pattern during follow-up. Two patients showed changes from microangiopathy to a scleroderma pattern, one patient from a scleroderma pattern to microangiopathy and one patient from microangiopathy to a normal pattern. Raynaud’s phenomenon was equally distributed among patients with different capillaroscopy patterns (p=0.487), as was median SLEDAI score at diagnosis (p=0.285). Follow-up patients with a capillary scleroderma pattern did not show any clinical features for SSc over time (follow-up over 1-5 years, Table 1 below. Patients with a capillary scleroderma pattern showed significantly more disease damage (Chi-square, p=0.008), also indicated by a survival analysis for disease damage (Figure 1, p=0.042). Conclusion: Our study indicates that a capillary scleroderma pattern in cSLE correlates with disease damage but not with clinical SSc features during follow-up period. References: [1]S. Pavlov-Dolijanovic et al. Is there a difference in systemic lupus erythematosus with and without Raynaud’s phenomenon? Rheum Int 2013;33: 859–865 [2]D. Schonenberg-Meinema et al. Nailfold capillary abnormalities in childhood-onset systemic lupus erythematosus. Revised manuscript after review re-submitted to Lupus in January 2021 [3]V. Smith et al. Fast track algorithm. Autoimm Rev 2019 Nov;18(11): 102394 Table 1. Clinical characteristics of cSLE-patients (n=9/53) with a nailfold capillary scleroderma pattern Butterfly rash Photosensitive rash Lupus nephritis Autoimmune cytopenia Positive Coombs Low C3/C4 Serositis Damage Type of auto-antibodies Follow-up in years 1 + + - + + + - + ANA, Anti-ds-DNA, anti-Sm, anti-RNP, anti-Ro52, anti-SS-A 5 2 + + - + + + - + ANA, Anti-ds-DNA, anti-Sm, anti-RNP, anti-SS-A 5 3 - + + + + + + + ANA, Anti-ds-DNA, anti-Sm, anti-RNP, anti-SS-A 10 4 - + + - + + + + ANA, Anti-ds-DNA, anti-Sm, anti-RNP, anti-Ro52, anti-SS-A 9 5 - + - - + - + - ANA 1 (lost) 6 - + + + + + + - ANA, Anti-ds-DNA, anti-c1q 3 7 - - - + - - - - ANA, Anti-ds-DNA, anti-Sm, anti-RNP 1 8 + + - + - - - - ANA 9 9 - + + + + + + + ANA, Anti-ds-DNA, anti-RNP, anti-SS-A 8 Figure 1. Occurrence of disease damage (with survival as ‘no damage’) in cSLE patients with microangiopathy (blue) and a capillary scleroderma pattern (red) Disclosure of Interests: None declared