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

Abstract

ObjectivesTo 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.MethodsClinical 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’.ResultsOur 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.ConclusionThis 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.

Scleroderma-like capillaroscopic pattern in SLE is not a sign of overlap syndrome in both adults and children

“…Recently published reports by Schonenberg-Meinema et al also revealed the presence of a ‘scleroderma-like’ capillaroscopic pattern in patients with childhood-onset SLE without overlap with SSc and without anti-RNP antibody positivity. 13 14 Out of 41 patients with SLE with disease onset <18 years, a ‘scleroderma-like’ pattern was observed in 17.1% (7/41) of the cases without SSc-associated symptoms. Positivity of anti-RNP antibodies was not different in patients with and without a ‘scleroderma-like’ capillaroscopic pattern.…”

“…However, there was no association of the capillary pattern with disease activity and RP, though anti-RNP antibodies were detected significantly more frequently in patients with ‘scleroderma-like’ changes. During a 5-year follow-up after disease onset, patients with a ‘scleroderma-like’ pattern did not develop SSc symptoms, but more than half of them presented with SLE-related irreversible disease damage that could not be predicted by SLEDAI (SLE Disease Activity Index) at diagnosis or during the follow-up 13. These observations indicate that scleroderma-like microangiopathy could be observed in both children and adults without the presence of overlap syndrome with SSc and without association with anti-RNP antibody 5 9 10…”

“…To sum up, a ‘scleroderma-like’ pattern is a relatively non-specific morphological finding that could be found in different rheumatic diseases other than SSc and SSc-associated overlap syndromes (eg, SLE in adults and children, dermatomyositis, rheumatoid arthritis) 5 9 10 13 14. Additionally, it could be observed in CLE as a local skin pathology without evidence of systemic vasculopathy.…”

Microangiopathy in Rheumatic Diseases

“…It is characterized by the presence of giant capillaries, microhemorrhages, devascularization, and capillary derangement [ 7 ], and could be found in the vast majority of patients with overt SSc (70–90%) [ 8 , 9 ]. Analogous capillaroscopic changes, i.e., “scleroderma-like” pattern, could also be observed with varying frequency in other rheumatic diseases, such as dermatomyositis (63–89%) [ 10 ], undifferentiated connective tissue disease (in approximately half of patients with RP) [ 11 ], and overlap syndromes, as well as in SLE (2–17%) [ 8 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 ] and RA (14–20.9%) [ 13 , 20 , 21 , 22 ] without signs of overlap. In SSc there is validated staging of the “scleroderma”-type capillaroscopic changes suggested by Cutolo et al that includes three distinct phases, i.e., “early”, “active”, and “late.” The “early” phase is characterized by the appearance of a few giant capillaries; a few microhemorrhages but with preserved capillary density and architecture.…”

“…Previously, it was considered that “scleroderma-like” capillaroscopic changes in SLE are associated with anti-RNP antibodies and indicate subclinical overlap with SSc [ 19 , 25 ], while there is now increasing evidence that “scleroderma-like” pattern could be observed in SLE patients without features of overlap and without association with anti-RNP antibodies [ 12 , 13 , 14 , 15 ]. In an own study (2013), a “scleroderma-like” pattern has been observed in 13.3% of examined SLE patients without signs of overlap syndrome with SSc and without association with anti-RNP antibodies.…”