Predictors of interstitial lung disease in early systemic sclerosis: a prospective longitudinal study of the GENISOS cohort
IntroductionThe objective was to examine the association of baseline demographic and clinical characteristics with sequentially obtained measurements of forced vital capacity (FVC, expressed as a percentage of the predicted value) and to identify predictors of decline rate in FVC over time in the Genetics versus Environment in Scleroderma Outcome Study (GENISOS).
Methods: To date, 266 patients were enrolled in GENISOS, a prospective, observational cohort of patients with early systemic sclerosis. In addition to pulmonary function tests (PFT), clinical and laboratory data were obtained from each patient.
We analyzed 926 FVC measurements utilizing generalized linear mixed models. The predictive significance of baseline variables for the decline rate in FVC was investigated by the interaction term between the variable and follow up time within the first 3 years after enrollment as well as throughout the entire follow up time.
Results: The cohort consisted of 125 white, 54 African American, and 77 Hispanic patients with average disease duration of 2.5 years at enrollment.
The mean follow up time was 3.8 years, ranging up to 11.4 years. A number of baseline variables including antibody status, African American ethnicity, disease type, baseline PFT values, modified Rodnan Skin Score, fibrosis on chest radiograph, lung and skin subscores of Severity Index were associated with serially measured FVC levels.
However, only presence of anti-topoisomerase I antibodies (ATA) was associated with lower FVC levels (P<0.001) as well as accelerated decline rate in FVC within the first 3 years of follow up (P=0.02). None of the baseline variables predicted the rate of decline in FVC on long term follow up.
However, patients with rapidly progressive ILD were underrepresented in the long term follow up group because the accelerated rate of decline in FVC was associated with poor survival (P=0.001).
Conclusions: ATA was the only baseline variable, associated with differential FVC levels, predicting the rate of decline in FVC within the first three years of follow up. The association of faster decline in FVC with poor survival further emphasizes the need for identification of predictive biomarkers by collection of genetic information and serial blood samples in cohort studies.
Author: Shervin AssassiRoozbeh SharifRobert LaskyTerry McNearneyRosa Estrada-Y-MartinHilda DraegerDeepthi NairMarvin FritzlerJohn ReveilleFrank ArnettMaureen MayesThe GENISOS Study
Credits/Source: Arthritis Research &Therapy 2010, 12:R166
Published on: 2010-09-02
Copyright by the authors listed above - made available via BioMedCentral (Open Access). Please
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