PMID- 22550317
OWN - NLM
STAT- MEDLINE
DCOM- 20130114
LR  - 20220408
IS  - 1468-2060 (Electronic)
IS  - 0003-4967 (Linking)
VI  - 71
IP  - 12
DP  - 2012 Dec
TI  - Prevalence and severity of interstitial lung disease in mixed connective tissue 
      disease: a nationwide, cross-sectional study.
PG  - 1966-72
LID - 10.1136/annrheumdis-2011-201253 [doi]
AB  - 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.
FAU - Gunnarsson, Ragnar
AU  - Gunnarsson R
AD  - Rheumatology Unit, Oslo University Hospital Rikshospitalet, PO Box 4950 Nydalen, 
      0424 Oslo, Norway. ragunnar@gmail.com
FAU - Aalokken, Trond Mogens
AU  - Aalokken TM
FAU - Molberg, Oyvind
AU  - Molberg O
FAU - Lund, May Brit
AU  - Lund MB
FAU - Mynarek, Georg Karl
AU  - Mynarek GK
FAU - Lexberg, Ase Stavland
AU  - Lexberg AS
FAU - Time, Kari
AU  - Time K
FAU - Dhainaut, Alvilde Sofie Strand
AU  - Dhainaut AS
FAU - Bertelsen, Liv-Turid
AU  - Bertelsen LT
FAU - Palm, Oyvind
AU  - Palm O
FAU - Irgens, Karen
AU  - Irgens K
FAU - Becker-Merok, Andrea
AU  - Becker-Merok A
FAU - Nordeide, Jan Leidulf
AU  - Nordeide JL
FAU - Johnsen, Villy
AU  - Johnsen V
FAU - Pedersen, Sonja
AU  - Pedersen S
FAU - Proven, Anne
AU  - Proven A
FAU - Garabet, Lamya Samir Noori
AU  - Garabet LS
FAU - Gran, Jan Tore
AU  - Gran JT
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20120501
PL  - England
TA  - Ann Rheum Dis
JT  - Annals of the rheumatic diseases
JID - 0372355
SB  - IM
MH  - Adult
MH  - Cohort Studies
MH  - Cross-Sectional Studies
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Lung Diseases, Interstitial/*mortality/pathology/*physiopathology
MH  - Male
MH  - Middle Aged
MH  - Mixed Connective Tissue Disease/*mortality/pathology/*physiopathology
MH  - Motor Activity
MH  - Norway/epidemiology
MH  - Prevalence
MH  - Pulmonary Fibrosis/mortality/pathology/physiopathology
MH  - *Severity of Illness Index
EDAT- 2012/05/03 06:00
MHDA- 2013/01/15 06:00
CRDT- 2012/05/03 06:00
PHST- 2012/05/03 06:00 [entrez]
PHST- 2012/05/03 06:00 [pubmed]
PHST- 2013/01/15 06:00 [medline]
AID - annrheumdis-2011-201253 [pii]
AID - 10.1136/annrheumdis-2011-201253 [doi]
PST - ppublish
SO  - Ann Rheum Dis. 2012 Dec;71(12):1966-72. doi: 10.1136/annrheumdis-2011-201253. 
      Epub 2012 May 1.