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Pulmonary outcome in juvenile dermatomyositis: a case-control study
  1. Helga Sanner1,2,
  2. Trond Mogens Aaløkken3,
  3. Jan Tore Gran1,2,
  4. Ivar Sjaastad1,4,5,
  5. Bjørn Johansen1,6,
  6. Berit Flatø1,2
  1. 1Institute of Clinical Medicine, University of Oslo, Oslo, Norway
  2. 2Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Oslo, Norway
  3. 3Department of Radiology, Oslo University Hospital, Rikshospitalet, Oslo, Norway
  4. 4Institute for Experimental Medical Research, Oslo University Hospital, Oslo, Norway
  5. 5Department of Cardiology, Oslo University Hospital, Oslo, Norway
  6. 6Department of Respiratory Medicine, Oslo University Hospital, Rikshospitalet, Oslo, Norway
  1. Correspondence to Dr Helga Sanner, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, 0027 Oslo, Norway; helga.sanner{at}medisin.uio.no

Abstract

Objectives To compare pulmonary function in patients with juvenile dermatomyositis (JDM) with that of matched controls; and to examine associations between pulmonary function impairment, high-resolution CT (HRCT) abnormalities and other disease variables in patients with JDM.

Methods A total of 59 patients with JDM clinically examined a median 16.8 years (range 2–38 years) after disease onset were compared with 59 age-matched and sex-matched controls. Pulmonary function tests included spirometry, diffusing capacity for carbon monoxide (DLCO) and body plethysmography. In patients with JDM, HRCT scans were performed and cumulative organ damage and patient-reported health status assessed.

Results Patients with JDM had lower total lung capacity (TLC) and DLCO compared to controls (p=0.003 and <0.001, respectively). A low TLC was found in 26% of patients versus 9% of controls (p=0.026), and a low DLCO in 49% of patients versus 9% of controls (p<0.001). HRCT abnormalities were found in 37% of patients, and included interstitial lung disease (ILD) (14%), chest wall calcinosis (14%) and airway disease (15%). Three patients were diagnosed as having ILD prior to the follow-up visit. A low TLC was more often found in patients with than without abnormal HRCT (50% vs 12%, p=0.002). HRCT abnormality correlated with cumulative organ damage (rs=0.346, p=0.008) and patient-reported health status at follow-up (p<0.005).

Conclusions Patients with JDM had smaller lung volumes than controls; a restrictive ventilatory defect was found in 26% and HRCT abnormality in 37% of the patients, and these findings were associated. Although mostly subclinical, the relatively high frequency of pulmonary involvement highlights the systemic nature of JDM.

  • Accepted 10 July 2010

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  • Accepted 10 July 2010
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Footnotes

  • Funding The project received financial support form Dr Olga Imerslunds foundation, Oslo, Norway

  • Competing interests None.

  • Ethics approval This study was carried out in compliance with the declaration of Helsinki and was approved by the Regional Ethics Committee, (Helse Sør-Øst).

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

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