Juvenile Dermatomyositis: Association between Nail Fold Capillary End Row Loops Area Under the Curve and Disease Damage Indicators.

BACKGROUND

Juvenile Dermatomyositis (JDM) is a rare autoimmune disease characterized by skin and muscle inflammation. The loss of nail fold capillary end row loops (ERL) is evidence of small vessel involvement in JDM. This study aimed to examine the association of ERL over the disease course and evidence of disease damage.

METHODS

We analyzed data from 68 initially treatment-naïve JDM children who had been observed for at least five years with multiple ERL density assessments. The JDM disease courses were categorized into monocyclic short, monocyclic long, polycyclic, and chronic. The ERL capillary count was cumulatively evaluated using the area under the curve (AUC) method.

RESULTS

The mean ERL density for the treatment-naive JDM was significantly lower than that of their healthy controls (4.8±1.6 /mm vs. 7.9±0.9 /mm; p <0.0001). The ERL AUC was significantly lower in children with chronic disease course compared to those with monocyclic short (p =0.001) or monocyclic long disease course (p =0.013). JDM patients with lipodystrophy had lower ERL AUC than those without lipodystrophy (p =0.04). There was no association between ERL AUC and calcifications or fractures.

CONCLUSION

Persistently decreased ERL capillary density, evident by low ERL AUC, is associated with chronic disease course and lipodystrophy in JDM. Despite medical therapy, the mean ERL count remained below normal even after five years, particularly in polycyclic and chronic cases. Therefore, the goal of restoring normal capillary density in children with JDM might be challenging and require novel therapeutic strategies targeting their underlying endothelial dysfunction.