Trial Lecture – time and place
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Adjudication committee
- First opponent: Post doc Helga Bergljot Midtbø, University of Bergen and Haukeland University Hospital
- Second opponent: Head of dept., Ivan Foeldvari, Hamburg Centre for Pediatric and Adolescence Rheumatology, Germany and Semmelweis Medical School, Budapest, Hungary
- Third member and chair of the evaluation committee: Professor II Espen Andre Haavardsholm, Faculty of Medicine, University of Oslo
Chair of the Defence
Professor Emeritus Emilia Kerty, Faculty of Medicine, University of Oslo
Principal Supervisor
MD, PhD Helga Sanner, Oslo University Hospital
Summary
Juvenile Dermatomyositis (JDM) is a rare autoimmune disease that leads to inflammation in the skin and muscles. The disease is characterised by systemic vasculopathy that may affect several organ systems, including the heart and lungs. Clinically important heart disease is rarely seen in patients with JDM, but subclinical cardiac involvement is often reported. Pulmonary involvement of clinical relevance is a rare, but potentially fatal complication in JDM.
Our main aim was to assess autonomic function and microvascular involvement in patients with medium- to long-tem JDM, and to compare with age- and sex-matched controls. Autonomic function was assessed by calculating heart rate variability (HRV) from Holter recordings. Impaired autonomic control was found in patients compared with controls, and decreased HRV was associated with elevated levels of inflammatory markers. Microvascular involvement was assessed by nailfold capillaroscopy (NFC). Compared with controls, patients presented microvascular remodelling, including decreased nailfold capillary density (NCD) and more prevalent scleroderma and neovascular pattern. When comparing patients with active vs inactive disease, active patients presented more decreased HRV and NCD.
We also aimed to elaborate on the linkage between impaired autonomic control and microvascular remodelling, and clinical outcomes including disease activity, and cardiac and pulmonary involvement in JDM. Impaired vagal control was associated with elevated levels of cytokines and cardiac dysfunction. Decreased NCD and neovascular pattern were associated with higher disease activity. Patients with low NCD presented impaired pulmonary function and more prevalent HRCT abnormalities than patients with normal NCD. No association was found between microvascular remodelling of the nailfolds and cardiac involvement.
Our results suggest impaired autonomic control and microvascular remodelling in JDM patients after medium- to long-term disease duration. Impaired vagal modulation seems to be linked to cardiac dysfunction. Microvascular remodelling of the nailfolds is associated with pulmonary, but not with cardiac involvement.
Additional information
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