CAPILLARY SCLERODERMA PATTERN AND DISEASE DAMAGE IN CHILDHOOD-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS: IMPORTANT LESSONS FROM LONGITUDINAL FOLLOW-UP
- Dieneke Schonenberg-Meinema (Netherlands)
Abstract
Background and Aims
To observe if nailfold capillary patterns in childhood-onset SLE (cSLE) change over time and if a scleroderma pattern is associated with higher disease activity, -damage or scleroderma-like features.
Methods
Prospective data from cSLE-patients (onset < 18 years) were analyzed. Disease activity was defined by SLEDAI score and disease damage by SDI index. A scleroderma pattern was defined according to the ‘Fast track algorithm’ from the EULAR Study Group on Microcirculation in Rheumatic Diseases. An abnormal capillary pattern, not matching a scleroderma pattern, was defined as ‘microangiopathy’.
Results
We studied 53 cSLE patients with median disease onset of 14 years (IQR 12.5-15.5 years), median SLEDAI score at diagnosis was 11 (IQR 8-16). N=9 (17%) showed a scleroderma pattern (ever) and n=37 (70%) showed microangiopathy. N=30 patients had follow-up capillaroscopy of which 24/30 (80%) showed no changes in capillary pattern over time. Median SLEDAI score at diagnosis did not differ between capillary pattern groups. A scleroderma pattern in cSLE was significantly associated with the presence of anti-RNP antibodies and with occurrence of disease damage. Patients with a scleroderma pattern did not develop clinical features for systemic sclerosis during follow-up (range 1-10 years after diagnosis), but showed significantly increased risk for SLE-related disease damage (Hazard ratio 9.4, p=0.003).
Conclusions
This prospective study shows that the majority of abnormal capillary patterns in cSLE do not change over time, irrespective of treatment and SLE disease activity. Capillary scleroderma pattern in cSLE might reflect a more severe disease course and seems a prognostic red flag for disease damage.