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Pulmonary outcome in juvenile dermatomyositis: a case-control study.
MedLine Citation:
PMID:  20805295     Owner:  NLM     Status:  In-Process    
Abstract/OtherAbstract:
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 (r(s)=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.
Authors:
Helga Sanner; Trond Mogens Aaløkken; Jan Tore Gran; Ivar Sjaastad; Bjørn Johansen; Berit Flatø
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-08-30
Journal Detail:
Title:  Annals of the rheumatic diseases     Volume:  70     ISSN:  1468-2060     ISO Abbreviation:  Ann. Rheum. Dis.     Publication Date:  2011 Jan 
Date Detail:
Created Date:  2010-12-17     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0372355     Medline TA:  Ann Rheum Dis     Country:  England    
Other Details:
Languages:  eng     Pagination:  86-91     Citation Subset:  IM    
Affiliation:
Institute of Clinical Medicine, University of Oslo, Oslo, Norway. helga.sanner@medisin.uio.no
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