scholarly journals Interstitial lung disease in systemic sclerosis

2016 ◽  
Vol 67 (4) ◽  
Author(s):  
E.A. Renzoni
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Lung Fibrosis ◽  
Immunosuppressive Agents ◽  
Significant Proportion ◽  
Clinical Markers ◽  
Significant Toxicity ◽  
Clinically Significant

Systemic sclerosis (SSc) is a connective tissue disease characterised by fibrosis of the skin and internal organs, autoimmune abnormalities and widespread vasculopathy. A degree of interstitial lung involvement is present in the majority of patients, although clinically significant lung fibrosis is present in approximately a third. Autoantibodies are significant clinical markers; anti-topoisomerase is tightly linked to lung fibrosis, whereas anti-centromere antibodies are protective. Further evaluation of markers of progression of lung fibrosis, such as markers of epithelial permeability, will be crucial in clinical management. The clinical course of SSc-associated interstitial lung disease is highly variable, with stability observed in a significant proportion of patients. Therefore, the decision of whether to treat is a challenging one, and should be based on evaluation of disease severity (on the basis of CT extent and lung function) and longitudinal disease behaviour. Two recently published placebo controlled randomized trials have shown a significant, if small, effect of cyclophosphamide on preventing FVC decline. However, because of the significant toxicity of cyclophosphamide, the assessment of alternative, less toxic, immunosuppressive agents for the long term management of SSc-associated interstitial lung disease is needed.

scholarly journals Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database

2020 ◽  
pp. annrheumdis-2020-217455 ◽  
Author(s):  
Anna-Maria Hoffmann-Vold ◽  
Yannick Allanore ◽  
Margarida Alves ◽  
Cathrine Brunborg ◽  
Paolo Airó ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Organ Damage ◽  
Disease Course ◽  
Lung Function Decline ◽  
Mixed Effect ◽  
Progression Patterns

ObjectivesTo identify overall disease course, progression patterns and risk factors predictive for progressive interstitial lung disease (ILD) in patients with systemic sclerosis-associated ILD (SSc-ILD), using data from the European Scleroderma Trials And Research (EUSTAR) database over long-term follow-up.MethodsEligible patients with SSc-ILD were registered in the EUSTAR database and had measurements of forced vital capacity (FVC) at baseline and after 12±3 months. Long-term progressive ILD and progression patterns were assessed in patients with multiple FVC measurements. Potential predictors of ILD progression were analysed using multivariable mixed-effect models.Results826 patients with SSc-ILD were included. Over 12±3 months, 219 (27%) showed progressive ILD: either moderate (FVC decline 5% to 10%) or significant (FVC decline >10%). A total of 535 (65%) patients had multiple FVC measurements available over mean 5-year follow-up. In each 12-month period, 23% to 27% of SSc-ILD patients showed progressive ILD, but only a minority of patients showed progression in consecutive periods. Most patients with progressive ILD (58%) had a pattern of slow lung function decline, with more periods of stability/improvement than decline, whereas only 8% showed rapid, continuously declining FVC; 178 (33%) experienced no episode of FVC decline. The strongest predictive factors for FVC decline over 5 years were male sex, higher modified Rodnan skin score and reflux/dysphagia symptoms.ConclusionSSc-ILD shows a heterogeneous and variable disease course, and thus monitoring all patients closely is important. Novel treatment concepts, with treatment initiation before FVC decline occurs, should aim for prevention of progression to avoid irreversible organ damage.

scholarly journals Increased Red Blood Cell Distribution Width in the First Year after Diagnosis Predicts Worsening of Systemic Sclerosis-Associated Interstitial Lung Disease at 5 Years: A Pilot Study

Diagnostics ◽  
2021 ◽  
Vol 11 (12) ◽  
pp. 2274
Author(s):  
Satoshi Ebata ◽  
Ayumi Yoshizaki ◽  
Takemichi Fukasawa ◽  
Asako Yoshizaki-Ogawa ◽  
Yoshihide Asano ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Blood Cell ◽  
Lung Disease ◽  
Red Blood Cell ◽  
First Year ◽  
Cell Distribution ◽  
Distribution Width ◽  
Red Blood Cell Distribution

The course of systemic sclerosis-associated interstitial lung disease (SSc-ILD) varies among individuals. Red blood cell distribution width (RDW) has been reported to be a predictor of idiopathic pulmonary fibrosis. However, there are no studies on the relationship between RDW and SSc-ILD. We conducted a retrospective study of 28 patients who were diagnosed with SSc-ILD on their first visit to our hospital and were followed-up for 5 years. The correlation between the changes in RDW, KL-6, and SP-D (ΔRDW, ΔKL-6, ΔSP-D) and the changes in percent-predicted forced lung volume and % carbon monoxide diffusion (Δ%FVC, Δ%DLco) was investigated. ΔRDW at 1 year after diagnosis was significantly inversely correlated with Δ%FVC at 5 years after diagnosis (r = −0.51, p < 0.001) and Δ%DLco at 5 years after diagnosis (r = −0.47, p < 0.001), whereas ΔKL-6 and ΔSP-D at 1 year were not correlated with Δ%FVC or Δ%DLco at 5 years. In the group of SSc-ILD patients with RDW increase in the first year after diagnosis, %FVC and %DLco were significantly lower than baseline at 3-, 4-, and 5-year assessments. In the group of patients without RDW increase in the first year, %FVC and %DLco did not decrease during the follow-up period. In conclusion, the changes in RDW in the first year after diagnosis may be useful surrogate markers to predict the long-term course of SSc-ILD.

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