Public Defence: Siri Opsahl Hetlevik – Rheumatology Siri Opsahl Hetlevik at the Institute of Clinical Medicine will be defending the thesis “Long-term outcome and clinical manifestations in juvenile-onset mixed connective tissue disease” for the degree of PhD.

Foto: Ine Eriksen

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Adjudication Committee

  • First opponent: Professor Ingrid Lundberg, Karolinske institutet
  • Second opponent: Professor Espen Haavardsholm, University of Oslo
  • Third member of the adjudication committee: Professor Karin C. Lødrup Carlsen, University of Oslo

Chair of the Defence

Professor Tillmann Uhlig, University of Oslo

Principal Supervisor Vibke Lilleby, Oslo University Hospital


Mixed connective tissue disease (MCTD) is a rare connective tissue disease characterized by the presence of anti-U1 RNP autoantibodies, Raynaud phenomenon and symptoms and findings such as puffy hands, arthritis, myositis, interstitial lung disease (ILD) and sclerodactyly. Disease onset during childhood occurs in about 7-23% of all cases, and data on juvenile onset MCTD (JMCTD) are scarce.

The aim of this study was to describe clinical manifestations and long-term outcome in JMCTD, with special regards to pulmonary and cardiac outcome in a cohort thought to be representative of patients in Norway.

Out of 52 examined patients with the diagnosis, we found persistently active disease in 67% after mean 16 years disease duration. A positive rheumatoid factor at time of diagnosis was a predictor for ongoing active disease. During the disease course, all patients exhibited anit-U1 RNP antibodies and Raynaud phenomenon. Other common manifestations were arthritis (94%), puffy hands (77%) and pulmonary manifestations (58%).

Mortality was 5.1%; standard mortality ratio was not increased compared to the general population. Only 3% of patients had PH, a feared complication of the disease. Compared to a control group, the patients had reduced lung function, and 27% had interstitial lung disease shown on high resolution CT. Lung function decreased over 10 years, but the ILD did not progress. The patients also had impaired left- and right ventricular function. Cardiac dysfunction was associated with high disease activity and longer treatment with prednisolone. This study gives valuable information to treating physicians and patients regarding long-term outcome and prognosis in JMCTD.

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Published Nov. 23, 2018 11:22 AM - Last modified Dec. 6, 2018 10:58 AM