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J Rheumatol. 2019 Jan;46(1):93-100. doi: 10.3899/jrheum.180019. Epub 2018 Aug 1.

Pulmonary Manifestations and Progression of Lung Disease in Juvenile-onset Mixed Connective Tissue Disease.

Author information

1
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. siri.opsahl@gmail.com.
2
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. siri.opsahl@gmail.com.
3
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.
4
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.

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.

KEYWORDS:

AUTOANTIBODIES; INTERSTITIAL LUNG DISEASE; MIXED CONNECTIVE TISSUE DISEASE; OUTCOME RESEARCH; PEDIATRIC RHEUMATOLOGY; PULMONARY FIBROSIS

PMID:
30068767
DOI:
10.3899/jrheum.180019

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