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Extended Report
Prevalence and severity of interstitial lung disease in mixed connective tissue disease: a nationwide, cross-sectional study
  1. Ragnar Gunnarsson1,2,
  2. Trond Mogens Aaløkken3,
  3. Øyvind Molberg1,2,
  4. May Brit Lund2,4,
  5. Georg Karl Mynarek3,
  6. Åse Stavland Lexberg5,
  7. Kari Time6,
  8. Alvilde Sofie Strand Dhainaut7,
  9. Liv-Turid Bertelsen8,
  10. Øyvind Palm9,
  11. Karen Irgens10,
  12. Andrea Becker-Merok11,
  13. Jan Leidulf Nordeide12,
  14. Villy Johnsen13,
  15. Sonja Pedersen14,
  16. Anne Prøven15,
  17. Lamya Samir Noori Garabet9,
  18. Jan Tore Gran1,2
  1. 1Rheumatology unit, Oslo University Hospital Rikshospitalet, Oslo, Norway
  2. 2Institute of Clinical Medicine, University of Oslo, Oslo, Norway
  3. 3Department of Radiology, Oslo University Hospital Rikshospitalet, Oslo, Norway
  4. 4Department of Respiratory Medicine, Oslo University Hospital Rikshospitalet, Oslo, Norway
  5. 5Department of Rheumatology, Buskerud Hospital, Drammen, Norway
  6. 6Haugesund Sanitetsforeningens Revmatismesykehus, Haugesund, Norway
  7. 7Department of Rheumatology, St Olavs Hospital, Trondheim University Hospital, Trondheim, Norway
  8. 8Department of Rheumatology, Haukeland University Hospital, Bergen, Norway
  9. 9Department of Rheumatology, Østfold Hospital, Moss, Norway
  10. 10Department of Rheumatology, Ålesund Hospital, Ålesund, Norway
  11. 11Department of Rheumatology, Institute of Clinical Medicine, University of Tromsø, Tromsø, Norway
  12. 12Department of Rheumatology, Førde Central Hospital, Førde, Norway
  13. 13Department of Rheumatology, Sørlandet Hospital, Kristiansand, Norway
  14. 14Department of Rheumatology, Nordland Hospital, Bodø, Norway
  15. 15Department of Rheumatology, Martina Hansens Hospital, Bærum, Norway
  1. Correspondence to Ragnar Gunnarsson, Rheumatology Unit, Oslo University Hospital Rikshospitalet, PO Box 4950 Nydalen, 0424 Oslo, Norway; ragunnar{at}


Background Mixed connective tissue disease (MCTD) is an immune-mediated, systemic disorder of unknown cause.

Objective To assess the prevalence, pattern and severity of interstitial lung disease (ILD) in a cross-sectional study of the nationwide, Norwegian MCTD cohort.

Methods 126 patients with MCTD were systematically examined for ILD by high-resolution CT (HRCT), pulmonary function tests (PFT), 6 min walk test (6MWT) and by the New York Heart Association (NYHA) functional classification of dyspnoea. The extent and type of HRCT lung abnormalities were scored according to the CT criteria of ILD recommended by the Fleischner Society.

Results All 126 patients were Caucasian, 75% women. At the time of the cross-sectional ILD study, the patients had a mean disease duration of 9.0 years. 52% of the patients had abnormal HRCT findings, most commonly reticular patterns consistent with lung fibrosis (35%). Lung fibrosis was quantified as minor in 7%, moderate in 9% and severe in 19% of the patients. Fibrosis was uniformly concentrated in the lower parts of the lungs and was not associated with smoking. Patients with severe lung fibrosis had lower PFT values, shorter 6MWT and a higher mean NYHA functional class. After a mean 4.2 years' follow-up, overall mortality was 7.9%. Mortality in patients with normal HRCT was 3.3%, as compared with 20.8% in patients with severe lung fibrosis (p<0.01).

Conclusions Severe lung fibrosis is common in MCTD, has an impact on pulmonary function and overall physical capacity and is associated with increased mortality.

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  • Funding Grants from the Norwegian Rheumatism Association and the Scandinavian Rheumatology Research Foundation.

  • Patient consent Obtained.

  • Ethics approval The study was approved by the regional committee for research ethics (REK) in the Southern and Eastern Norway Regional Health Authority and the Norwegian Social Science Data Services (NSD).

  • Provenance and peer review Not commissioned; externally peer reviewed.