In his research, Zoltán Barth has studied how autonomic function and microvascular involvement relate to cardiac function and disease activity in the rare skin/muscle disease juvenile dermatomyositis.

MAIN RESULTS:

1. Patients with juvenile dermatomyositis have reduced heart rate variabiliy.
2. Patients with juvenile dermatomyositis have reduced nailfold capillary density.
3. Reduced heart rate variability and nailfold capillary density in juvenile dermatomyositis relate to inflammation, cardiac function and/or disease activity.

THESIS DEFENCE:

Thesis: Microvascular Remodelling and Autonomic Dysregulation in Juvenile Dermatomyositis
Candidate: Zoltán Barth
Time: May 10, 2019 at 13:15
Place: Oslo University Hospital, Rikshospitalet: Seminar room A3 3067
Link to university website

SUMMARY:

(1) Young patients with juvenile dermatomyositis have reduced cardiac function, and Barth’s first article shows that these impairments associate with lower heart rate variability. Low heart rate variability also relates to increased inflammation. The study indicates disturbed autonomic control in juvenile dermatomyositis.

The study includes 55 patients aged 20 years old on average. Heart rate variability was assessed by 24-hour Holter monitoring. Two out of six measures of heart rate variability were lower than in the healthy control group. Furthermore, three of the heart rate variability measures were significantly associated with both systolic and diastolic heart function, and most measures of heart rate variability were linked to an increase in several inflammatory markers.

(2) Nailfold capillary density is reduced and linked to disease severity and impaired muscle function in patients with medium- to long-term juvenile dermatomyositis. 58 patients were included in the study and compared to matched healthy controls. The patients also had more prevalent scleroderma and neovascular pattern, and the latter was also related to disease activity and muscle function.

(3) Neither reduced nailfold capillary density nor neovascular pattern are associated with cardiac parameters in juvenile dermatomyositis. However, low nailfold capillary density is linked to subclinical reductions in lung function and airway disease.

REFERENCES:

(1) Barth, Z., Nomeland Witczak, B., Schwartz, T., Gjesdal, K., Flatø, B., Koller, A., Sanner, H., & Sjaastad, I. (2015). In juvenile dermatomyositis, heart rate variability is reduced, and associated with both cardiac dysfunction and markers of inflammation: a cross-sectional study median 13.5 years after symptom onset. Rheumatology, 55(3), 535-543.

(2) Barth, Z., Witczak, B. N., Flatø, B., Koller, A., Sjaastad, I., & Sanner, H. (2018). Assessment of Microvascular Abnormalities by Nailfold Capillaroscopy in Juvenile Dermatomyositis After Medium‐to Long‐Term Followup. Arthritis care & research, 70(5), 768-776.

(3) Barth, Z., Schwartz, T., Flatø, B., Aaløkken, T. M., Koller, A., Lund, M. B., Sjaatad, I., & Sanner, H. (2019). Association Between Nailfold Capillary Density and Pulmonary and Cardiac Involvement in Medium to Longstanding Juvenile Dermatomyositis. Arthritis care & research, 71(4), 492-497.