Adjudication committee
- First opponent: Professor Annet van Royen-Kerkhof, University Medical Center Utrecht, The Netherlands
- Second opponent: Professor Jan Kristian Damås, NTNU – Norwegian University of Science and Technology
- Third member and chair of the evaluation committee: Professor Emeritus Ingebjørg Seljeflot, University of Oslo
Chair of the Defence
Professor Stine Marie Ulven, University of Oslo
Principal Supervisor
Professor II Helga Sanner, ONH – Oslo New University College
Summary
Juvenile Dermatomyositis (JDM) is a rare autoimmune disease of childhood. It involves vasculopathy and chronic inflammation, leading to distinct skin rash, muscle weakness and potential organ involvement, such as reduced cardiac and pulmonary function. Unfortunate adipose tissue distribution may exacerbate the risk of developing cardiac diseases through a proinflammatory cyto- and adipokine profile.
This thesis explores the long-term effects of JDM on adipose tissue distribution, cardio-metabolic syndrome, and pulmonary structures and function. All compared with controls. Further how cytokines are associated with these organ-outcomes.
Our findings revealed a significant 2.4-fold increase in abdominal adipose tissue among patients, with 23% occurrence of cardio-metabolic syndrome. Cardiac dysfunction was three times more prevalent in patient, and independent associations were identified with lipodystrophy, and male gender.
In all patients leptin levels were increased and patients with active disease had elevated levels of apelin-12, and visfatin. Adiponectin and apelin-12 showed associations with improved cardiac pumping and adiponectin with better cardiac relaxation. Resistin and lipocalin-2 showed adverse associations. All associations were most evident in patients with active disease.
In the lungs, a reduced capacity for oxygen exchange was found in active compared with inactive disease. Cytokines such as IP-10, eotaxin, and MCP-1 were found to be associated with structural changes in active disease. Whereas in inactive disease growth factors and Il-17 were linked to various functional measures.
In sum, after long-term disease, patients exhibited increased abdominal fat and adipokine level imbalances impacting cardiac function, especially in active disease. Cytokines showed diverse associations with pulmonary outcomes underscoring the complexity of the interplay between immune responses and disease manifestations.
