Background Nailfold capillaroscopy is a useful investigation to identify patients with Raynaud's phenomenon secondary to systemic sclerosis (SSc) because typical capillary changes can be clearly demonstrated in adults as well as in children. However, in juvenile-onset SSc the overall capillaroscopic pattern is called “scleroderma pattern”, the classification in “early”, “active” and “late” pattern (1) has never been applied, and differences in microvascular abnormalities between juvenile- and adult-onset SSc have never been explored.
Objectives Microvascular abnormalities described as qualitative overall patterns by nailfold capillaroscopy in patients with juvenile- and adult-onset systemic sclerosis (SSc) were studied in adulthood using data from the EULAR Scleroderma Trials and Research (EUSTAR) database.
Methods Data collected between June 2004 and April 2013 in the EUSTAR registry were examined with focus on capillaroscopy. In this case-control study, adult patients with juvenile-onset SSc with available data on capillaroscopy were matched with corresponding patients with adult-onset SSc having the same gender and autoantibody profile. Patients aged ≥65 year-old at disease onset were excluded.
Results 30 adult patients with juvenile-onset SSc and 2108 patients with adult-onset SSc were included in the analysis. The mean age at the visit of patients with adult- and juvenile-onset SSc was 52.91±12.6 and 29.56±10.71 year-old respectively. Mean age at the onset of Raynaud's phenomenon was 38.60±12.23 year-old in adult-onset and 11.36±5.12 year-old in juvenile-onset SSc. Similar distribution of modified Rodnan skin score between adult- and juvenile-onset SSc was observed (8.85±7.88 and 10.25±9.74 respectively). The majority of patients had a scleroderma pattern and it was equally distributed among early, active and late pattern in juvenile-onset SSc and adult-onset SSc. Results of univariate analysis showed that in patients with juvenile-onset SSc showed the presence of scleroderma pattern more frequently than adult-onset SSc (OR=2.44, p=0.17), even if not significant. No difference was observed in the distribution of early, active and late pattern between the two groups (OR estimates are near 1.0). Juvenile-onset SSc and adult-onset SSc shared similar capillaroscopy patterns and organ involvement, except for a slightly decreased frequency of oesophageal involvement (46.66% vs 66.22%) and increased lung fibrosis assessed by HRCT (18.13% vs 42.85%) in juvenile-onset SSc (OR=0.46 p=0.04 and OR 2.97, p=0.009 respectively).
Conclusions This study is the largest series of adult patients with juvenile-onset SSc in which capillaroscopy has been performed and classified using the three qualitative patterns usually applied in adult-onset SSc. We showed that the microvascular involvement documented by nailfold capillaroscopy patterns in adulthood was similar in patients with juvenile- and adult-onset SSc. The possibility to use the same classification in three patterns applied in adults may be useful to standardise this examination.
Cutolo M, Pizzorni C, Tuccio M, Burroni A, Craviotto C, Basso M, et al. Nailfold videocapillaroscopic patterns and serum autoantibodies in systemic sclerosis. Rheumatology (Oxford). 2004;43(6):719-26.
Disclosure of Interest None declared