Research ArticleArticle
Pulmonary Manifestations and Progression of Lung Disease in Juvenile-onset Mixed Connective Tissue Disease
Siri Opsahl Hetlevik, Berit Flatø, Trond Mogens Aaløkken, May Brit Lund, Silje Reiseter, Georg Karl Mynarek, Ellen Nordal, Marite Rygg and Vibke Lilleby
The Journal of Rheumatology August 2018, jrheum.180019; DOI: https://doi.org/10.3899/jrheum.180019
Siri Opsahl Hetlevik
From the Department of Rheumatology, the Department of Radiology and Nuclear Medicine, and the Department of Respiratory Medicine, Oslo University Hospital, Rikshospitalet; Faculty of Medicine, Institute of Clinical Medicine, University of Oslo, Oslo; Department of Pediatrics, University Hospital of North Norway; Institute of Clinical Medicine, University of Tromsø, Tromsø; Department of Pediatrics, St. Olavs Hospital; Institute of Laboratory Medicine, Children’s and Women’s Health, Faculty of Medicine, Norwegian University of Science and Technology, Trondheim, Norway. This study was funded by The Norwegian Rheumatism Association and made possible by the Norwegian ExtraFoundation for Health and Rehabilitation. S.O. Hetlevik, MD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet; B. Flatø, MD, PhD, Professor, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; T.M. Aaløkken, MD, PhD, Department of Radiology and Nuclear Medicine, Oslo University Hospital; M.B. Lund, MD, PhD, Professor, Department of Respiratory Medicine, Oslo University Hospital, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; S. Reiseter, MD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; G.K. Mynarek, MD, Department of Radiology and Nuclear Medicine, Oslo University Hospital; E. Nordal, MD, PhD, Department of Pediatrics, University Hospital of North Norway, and Institute of Clinical Medicine, University of Tromsø; M. Rygg, MD, PhD, Professor, Department of Pediatrics, St. Olavs Hospital, and Institute of Laboratory Medicine, Children’s and Women’s Health, Faculty of Medicine, Norwegian University of Science and Technology; V. Lilleby, MD, PhD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet. Address correspondence to Dr. S.O. Hetlevik, Oslo University Hospital – Rikshospitalet, Pb 4950 Nydalen, 0424 Oslo, Norway. E-mail: siri.opsahl{at}gmail.com. Accepted for publication May 10, 2018.
Berit Flatø
From the Department of Rheumatology, the Department of Radiology and Nuclear Medicine, and the Department of Respiratory Medicine, Oslo University Hospital, Rikshospitalet; Faculty of Medicine, Institute of Clinical Medicine, University of Oslo, Oslo; Department of Pediatrics, University Hospital of North Norway; Institute of Clinical Medicine, University of Tromsø, Tromsø; Department of Pediatrics, St. Olavs Hospital; Institute of Laboratory Medicine, Children’s and Women’s Health, Faculty of Medicine, Norwegian University of Science and Technology, Trondheim, Norway. This study was funded by The Norwegian Rheumatism Association and made possible by the Norwegian ExtraFoundation for Health and Rehabilitation. S.O. Hetlevik, MD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet; B. Flatø, MD, PhD, Professor, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; T.M. Aaløkken, MD, PhD, Department of Radiology and Nuclear Medicine, Oslo University Hospital; M.B. Lund, MD, PhD, Professor, Department of Respiratory Medicine, Oslo University Hospital, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; S. Reiseter, MD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; G.K. Mynarek, MD, Department of Radiology and Nuclear Medicine, Oslo University Hospital; E. Nordal, MD, PhD, Department of Pediatrics, University Hospital of North Norway, and Institute of Clinical Medicine, University of Tromsø; M. Rygg, MD, PhD, Professor, Department of Pediatrics, St. Olavs Hospital, and Institute of Laboratory Medicine, Children’s and Women’s Health, Faculty of Medicine, Norwegian University of Science and Technology; V. Lilleby, MD, PhD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet. Address correspondence to Dr. S.O. Hetlevik, Oslo University Hospital – Rikshospitalet, Pb 4950 Nydalen, 0424 Oslo, Norway. E-mail: siri.opsahl{at}gmail.com. Accepted for publication May 10, 2018.
Trond Mogens Aaløkken
From the Department of Rheumatology, the Department of Radiology and Nuclear Medicine, and the Department of Respiratory Medicine, Oslo University Hospital, Rikshospitalet; Faculty of Medicine, Institute of Clinical Medicine, University of Oslo, Oslo; Department of Pediatrics, University Hospital of North Norway; Institute of Clinical Medicine, University of Tromsø, Tromsø; Department of Pediatrics, St. Olavs Hospital; Institute of Laboratory Medicine, Children’s and Women’s Health, Faculty of Medicine, Norwegian University of Science and Technology, Trondheim, Norway. This study was funded by The Norwegian Rheumatism Association and made possible by the Norwegian ExtraFoundation for Health and Rehabilitation. S.O. Hetlevik, MD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet; B. Flatø, MD, PhD, Professor, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; T.M. Aaløkken, MD, PhD, Department of Radiology and Nuclear Medicine, Oslo University Hospital; M.B. Lund, MD, PhD, Professor, Department of Respiratory Medicine, Oslo University Hospital, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; S. Reiseter, MD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; G.K. Mynarek, MD, Department of Radiology and Nuclear Medicine, Oslo University Hospital; E. Nordal, MD, PhD, Department of Pediatrics, University Hospital of North Norway, and Institute of Clinical Medicine, University of Tromsø; M. Rygg, MD, PhD, Professor, Department of Pediatrics, St. Olavs Hospital, and Institute of Laboratory Medicine, Children’s and Women’s Health, Faculty of Medicine, Norwegian University of Science and Technology; V. Lilleby, MD, PhD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet. Address correspondence to Dr. S.O. Hetlevik, Oslo University Hospital – Rikshospitalet, Pb 4950 Nydalen, 0424 Oslo, Norway. E-mail: siri.opsahl{at}gmail.com. Accepted for publication May 10, 2018.
May Brit Lund
From the Department of Rheumatology, the Department of Radiology and Nuclear Medicine, and the Department of Respiratory Medicine, Oslo University Hospital, Rikshospitalet; Faculty of Medicine, Institute of Clinical Medicine, University of Oslo, Oslo; Department of Pediatrics, University Hospital of North Norway; Institute of Clinical Medicine, University of Tromsø, Tromsø; Department of Pediatrics, St. Olavs Hospital; Institute of Laboratory Medicine, Children’s and Women’s Health, Faculty of Medicine, Norwegian University of Science and Technology, Trondheim, Norway. This study was funded by The Norwegian Rheumatism Association and made possible by the Norwegian ExtraFoundation for Health and Rehabilitation. S.O. Hetlevik, MD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet; B. Flatø, MD, PhD, Professor, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; T.M. Aaløkken, MD, PhD, Department of Radiology and Nuclear Medicine, Oslo University Hospital; M.B. Lund, MD, PhD, Professor, Department of Respiratory Medicine, Oslo University Hospital, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; S. Reiseter, MD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; G.K. Mynarek, MD, Department of Radiology and Nuclear Medicine, Oslo University Hospital; E. Nordal, MD, PhD, Department of Pediatrics, University Hospital of North Norway, and Institute of Clinical Medicine, University of Tromsø; M. Rygg, MD, PhD, Professor, Department of Pediatrics, St. Olavs Hospital, and Institute of Laboratory Medicine, Children’s and Women’s Health, Faculty of Medicine, Norwegian University of Science and Technology; V. Lilleby, MD, PhD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet. Address correspondence to Dr. S.O. Hetlevik, Oslo University Hospital – Rikshospitalet, Pb 4950 Nydalen, 0424 Oslo, Norway. E-mail: siri.opsahl{at}gmail.com. Accepted for publication May 10, 2018.
Silje Reiseter
From the Department of Rheumatology, the Department of Radiology and Nuclear Medicine, and the Department of Respiratory Medicine, Oslo University Hospital, Rikshospitalet; Faculty of Medicine, Institute of Clinical Medicine, University of Oslo, Oslo; Department of Pediatrics, University Hospital of North Norway; Institute of Clinical Medicine, University of Tromsø, Tromsø; Department of Pediatrics, St. Olavs Hospital; Institute of Laboratory Medicine, Children’s and Women’s Health, Faculty of Medicine, Norwegian University of Science and Technology, Trondheim, Norway. This study was funded by The Norwegian Rheumatism Association and made possible by the Norwegian ExtraFoundation for Health and Rehabilitation. S.O. Hetlevik, MD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet; B. Flatø, MD, PhD, Professor, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; T.M. Aaløkken, MD, PhD, Department of Radiology and Nuclear Medicine, Oslo University Hospital; M.B. Lund, MD, PhD, Professor, Department of Respiratory Medicine, Oslo University Hospital, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; S. Reiseter, MD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; G.K. Mynarek, MD, Department of Radiology and Nuclear Medicine, Oslo University Hospital; E. Nordal, MD, PhD, Department of Pediatrics, University Hospital of North Norway, and Institute of Clinical Medicine, University of Tromsø; M. Rygg, MD, PhD, Professor, Department of Pediatrics, St. Olavs Hospital, and Institute of Laboratory Medicine, Children’s and Women’s Health, Faculty of Medicine, Norwegian University of Science and Technology; V. Lilleby, MD, PhD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet. Address correspondence to Dr. S.O. Hetlevik, Oslo University Hospital – Rikshospitalet, Pb 4950 Nydalen, 0424 Oslo, Norway. E-mail: siri.opsahl{at}gmail.com. Accepted for publication May 10, 2018.
Georg Karl Mynarek
From the Department of Rheumatology, the Department of Radiology and Nuclear Medicine, and the Department of Respiratory Medicine, Oslo University Hospital, Rikshospitalet; Faculty of Medicine, Institute of Clinical Medicine, University of Oslo, Oslo; Department of Pediatrics, University Hospital of North Norway; Institute of Clinical Medicine, University of Tromsø, Tromsø; Department of Pediatrics, St. Olavs Hospital; Institute of Laboratory Medicine, Children’s and Women’s Health, Faculty of Medicine, Norwegian University of Science and Technology, Trondheim, Norway. This study was funded by The Norwegian Rheumatism Association and made possible by the Norwegian ExtraFoundation for Health and Rehabilitation. S.O. Hetlevik, MD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet; B. Flatø, MD, PhD, Professor, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; T.M. Aaløkken, MD, PhD, Department of Radiology and Nuclear Medicine, Oslo University Hospital; M.B. Lund, MD, PhD, Professor, Department of Respiratory Medicine, Oslo University Hospital, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; S. Reiseter, MD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; G.K. Mynarek, MD, Department of Radiology and Nuclear Medicine, Oslo University Hospital; E. Nordal, MD, PhD, Department of Pediatrics, University Hospital of North Norway, and Institute of Clinical Medicine, University of Tromsø; M. Rygg, MD, PhD, Professor, Department of Pediatrics, St. Olavs Hospital, and Institute of Laboratory Medicine, Children’s and Women’s Health, Faculty of Medicine, Norwegian University of Science and Technology; V. Lilleby, MD, PhD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet. Address correspondence to Dr. S.O. Hetlevik, Oslo University Hospital – Rikshospitalet, Pb 4950 Nydalen, 0424 Oslo, Norway. E-mail: siri.opsahl{at}gmail.com. Accepted for publication May 10, 2018.
Ellen Nordal
From the Department of Rheumatology, the Department of Radiology and Nuclear Medicine, and the Department of Respiratory Medicine, Oslo University Hospital, Rikshospitalet; Faculty of Medicine, Institute of Clinical Medicine, University of Oslo, Oslo; Department of Pediatrics, University Hospital of North Norway; Institute of Clinical Medicine, University of Tromsø, Tromsø; Department of Pediatrics, St. Olavs Hospital; Institute of Laboratory Medicine, Children’s and Women’s Health, Faculty of Medicine, Norwegian University of Science and Technology, Trondheim, Norway. This study was funded by The Norwegian Rheumatism Association and made possible by the Norwegian ExtraFoundation for Health and Rehabilitation. S.O. Hetlevik, MD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet; B. Flatø, MD, PhD, Professor, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; T.M. Aaløkken, MD, PhD, Department of Radiology and Nuclear Medicine, Oslo University Hospital; M.B. Lund, MD, PhD, Professor, Department of Respiratory Medicine, Oslo University Hospital, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; S. Reiseter, MD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; G.K. Mynarek, MD, Department of Radiology and Nuclear Medicine, Oslo University Hospital; E. Nordal, MD, PhD, Department of Pediatrics, University Hospital of North Norway, and Institute of Clinical Medicine, University of Tromsø; M. Rygg, MD, PhD, Professor, Department of Pediatrics, St. Olavs Hospital, and Institute of Laboratory Medicine, Children’s and Women’s Health, Faculty of Medicine, Norwegian University of Science and Technology; V. Lilleby, MD, PhD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet. Address correspondence to Dr. S.O. Hetlevik, Oslo University Hospital – Rikshospitalet, Pb 4950 Nydalen, 0424 Oslo, Norway. E-mail: siri.opsahl{at}gmail.com. Accepted for publication May 10, 2018.
Marite Rygg
From the Department of Rheumatology, the Department of Radiology and Nuclear Medicine, and the Department of Respiratory Medicine, Oslo University Hospital, Rikshospitalet; Faculty of Medicine, Institute of Clinical Medicine, University of Oslo, Oslo; Department of Pediatrics, University Hospital of North Norway; Institute of Clinical Medicine, University of Tromsø, Tromsø; Department of Pediatrics, St. Olavs Hospital; Institute of Laboratory Medicine, Children’s and Women’s Health, Faculty of Medicine, Norwegian University of Science and Technology, Trondheim, Norway. This study was funded by The Norwegian Rheumatism Association and made possible by the Norwegian ExtraFoundation for Health and Rehabilitation. S.O. Hetlevik, MD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet; B. Flatø, MD, PhD, Professor, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; T.M. Aaløkken, MD, PhD, Department of Radiology and Nuclear Medicine, Oslo University Hospital; M.B. Lund, MD, PhD, Professor, Department of Respiratory Medicine, Oslo University Hospital, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; S. Reiseter, MD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; G.K. Mynarek, MD, Department of Radiology and Nuclear Medicine, Oslo University Hospital; E. Nordal, MD, PhD, Department of Pediatrics, University Hospital of North Norway, and Institute of Clinical Medicine, University of Tromsø; M. Rygg, MD, PhD, Professor, Department of Pediatrics, St. Olavs Hospital, and Institute of Laboratory Medicine, Children’s and Women’s Health, Faculty of Medicine, Norwegian University of Science and Technology; V. Lilleby, MD, PhD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet. Address correspondence to Dr. S.O. Hetlevik, Oslo University Hospital – Rikshospitalet, Pb 4950 Nydalen, 0424 Oslo, Norway. E-mail: siri.opsahl{at}gmail.com. Accepted for publication May 10, 2018.
Vibke Lilleby
From the Department of Rheumatology, the Department of Radiology and Nuclear Medicine, and the Department of Respiratory Medicine, Oslo University Hospital, Rikshospitalet; Faculty of Medicine, Institute of Clinical Medicine, University of Oslo, Oslo; Department of Pediatrics, University Hospital of North Norway; Institute of Clinical Medicine, University of Tromsø, Tromsø; Department of Pediatrics, St. Olavs Hospital; Institute of Laboratory Medicine, Children’s and Women’s Health, Faculty of Medicine, Norwegian University of Science and Technology, Trondheim, Norway. This study was funded by The Norwegian Rheumatism Association and made possible by the Norwegian ExtraFoundation for Health and Rehabilitation. S.O. Hetlevik, MD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet; B. Flatø, MD, PhD, Professor, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; T.M. Aaløkken, MD, PhD, Department of Radiology and Nuclear Medicine, Oslo University Hospital; M.B. Lund, MD, PhD, Professor, Department of Respiratory Medicine, Oslo University Hospital, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; S. Reiseter, MD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; G.K. Mynarek, MD, Department of Radiology and Nuclear Medicine, Oslo University Hospital; E. Nordal, MD, PhD, Department of Pediatrics, University Hospital of North Norway, and Institute of Clinical Medicine, University of Tromsø; M. Rygg, MD, PhD, Professor, Department of Pediatrics, St. Olavs Hospital, and Institute of Laboratory Medicine, Children’s and Women’s Health, Faculty of Medicine, Norwegian University of Science and Technology; V. Lilleby, MD, PhD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet. Address correspondence to Dr. S.O. Hetlevik, Oslo University Hospital – Rikshospitalet, Pb 4950 Nydalen, 0424 Oslo, Norway. E-mail: siri.opsahl{at}gmail.com. Accepted for publication May 10, 2018.
Abstract
Objective To assess the occurrence and extent of interstitial lung disease (ILD) in patients with juvenile mixed connective tissue disease (JMCTD), compare pulmonary function in patients and matched controls, study associations between ILD and disease-related variables, and examine progression of pulmonary manifestations over time.
Methods A cohort of 52 patients with JMCTD were examined in a cross-sectional study after a mean 16.2 (SD 10.3) years of disease duration with high-resolution computed tomography (HRCT) and pulmonary function tests (PFT) comprising spirometry, DLCO, and total lung capacity (TLC). Matched controls were examined with PFT. Previous HRCT and PFT were available in 37 and 38 patients (mean 8.8 and 10.3 yrs before study inclusion), respectively.
Results Compared to controls, patients with JMCTD had lower forced vital capacity (FVC), DLCO, and TLC (p < 0.01). The most frequent abnormal PFT was DLCO in 67% of patients versus 17% of controls (p < 0.001). Fourteen patients (27%) had ILD on HRCT. Most had ILD in < 10% of their lungs. ILD was associated with low values for FVC and TLC, but not with DLCO. HRCT findings did not progress significantly over time, but FVC declined (p < 0.01).
Conclusion Compared to controls, patients with JMCTD had impaired pulmonary function. ILD was present in 27% of patients after a mean 16 years of disease duration, mostly as mild disease, and did not progress. ILD seems to be less common in juvenile-onset than in adult-onset MCTD, and ILD in JMCTD seems mostly mild and stable over time.
In this issue
The Journal of Rheumatology
Vol. 52, Issue 12
1 Dec 2025
Pulmonary Manifestations and Progression of Lung Disease in Juvenile-onset Mixed Connective Tissue Disease
Siri Opsahl Hetlevik, Berit Flatø, Trond Mogens Aaløkken, May Brit Lund, Silje Reiseter, Georg Karl Mynarek, Ellen Nordal, Marite Rygg, Vibke Lilleby
The Journal of Rheumatology Aug 2018, jrheum.180019; DOI: 10.3899/jrheum.180019
Pulmonary Manifestations and Progression of Lung Disease in Juvenile-onset Mixed Connective Tissue Disease
Siri Opsahl Hetlevik, Berit Flatø, Trond Mogens Aaløkken, May Brit Lund, Silje Reiseter, Georg Karl Mynarek, Ellen Nordal, Marite Rygg, Vibke Lilleby
The Journal of Rheumatology Aug 2018, jrheum.180019; DOI: 10.3899/jrheum.180019
