Capillaroscopy in childhood-onset systemic lupus erythematosus: a first systematic review

Abstract

Objectives: Recently, a systematic review indicated that, compared to healthy controls, adult patients with systemic lupus erythematosus (SLE) show a significantly more abnormal capillary morphology and greater number of haemorrhages in nailfold capillaroscopy and that these capillary changes are associated with disease activity. As yet, no systematic literature evaluation of capillaroscopy in childhood-onset SLE (cSLE) has been performed. Therefore, we aimed to systematically review the literature on nailfold capillary characteristics in cSLE.

Methods: Search terms "SLE or Lupus", "Capillaroscopy" and "Juvenile or Childhood or Paediatric or Child" were used in PubMed, Embase and Web of Science. Capillary findings were evaluated according to the current international consensus-based definitions for analysis of capillaroscopic characteristics from the European League against Rheumatism (EULAR) Study Group on Microcirculation in Rheumatic Diseases (SG MC/RD).

Results: After screening eighty search hits, six articles were retained, two of which were case-control studies and four case series. For capillary density, no difference was found between cSLE and healthy controls (one study). Differences in capillary diameter, capillary morphology, haemorrhages and semi-quantitative score were inconclusive or non-interpretable. A scleroderma pattern was not detected in the case control studies but was reported in a minority of cSLE patients in 3 out of 4 case series.

Conclusions: Literature on nailfold capillary findings in cSLE is scarce and inconclusive. To evaluate capillary characteristics in cSLE, prospective longitudinal studies are needed. Future studies should use uniform definitions for capillary characteristics and findings should be compared with healthy controls, matched for age and ethnicity. The EULAR SG MC/RD is stepping up to this need.