Bronchoalveolar lavage cellular profiles in patients with systemic sclerosis–associated interstitial lung disease are not predictive of disease progression

2007 ◽  
Vol 56 (6) ◽  
pp. 2005-2012 ◽  
Author(s):  
Nicole S. L. Goh ◽  
Srihari Veeraraghavan ◽  
Sujal R. Desai ◽  
Derek Cramer ◽  
David M. Hansell ◽  
...  
Respiration ◽  
2009 ◽  
Vol 78 (1) ◽  
pp. 56-62 ◽  
Author(s):  
N. Damjanov ◽  
P. Ostojic ◽  
O. Kaloudi ◽  
S. Alari ◽  
S. Guiducci ◽  
...  

2020 ◽  
Vol 55 (5) ◽  
pp. 1902026 ◽  
Author(s):  
Oliver Distler ◽  
Shervin Assassi ◽  
Vincent Cottin ◽  
Maurizio Cutolo ◽  
Sonye K. Danoff ◽  
...  

Systemic sclerosis (SSc) is a systemic autoimmune disease affecting multiple organ systems, including the lungs. Interstitial lung disease (ILD) is the leading cause of death in SSc.There are no valid biomarkers to predict the occurrence of SSc-ILD, although auto-antibodies against anti-topoisomerase I and several inflammatory markers are candidate biomarkers that need further evaluation. Chest auscultation, presence of shortness of breath and pulmonary function testing are important diagnostic tools, but lack sensitivity to detect early ILD. Baseline screening with high-resolution computed tomography (HRCT) is therefore necessary to confirm an SSc-ILD diagnosis. Once diagnosed with SSc-ILD, patients' clinical courses are variable and difficult to predict, although certain patient characteristics and biomarkers are associated with disease progression. It is important to monitor patients with SSc-ILD for signs of disease progression, although there is no consensus about which diagnostic tools to use or how often monitoring should occur. In this article, we review methods used to define and predict disease progression in SSc-ILD.There is no valid definition of SSc-ILD disease progression, but we suggest that either a decline in forced vital capacity (FVC) from baseline of ≥10%, or a decline in FVC of 5–9% in association with a decline in diffusing capacity of the lung for carbon monoxide of ≥15% represents progression. An increase in the radiographic extent of ILD on HRCT imaging would also signify progression. A time period of 1–2 years is generally used for this definition, but a decline over a longer time period may also reflect clinically relevant disease progression.


Respirology ◽  
2020 ◽  
Author(s):  
Carmel J.W. Stock ◽  
Rachel K. Hoyles ◽  
Cecile Daccord ◽  
Maria Kokosi ◽  
Dina Visca ◽  
...  

2021 ◽  
Vol 0 (0) ◽  
pp. 0-0
Author(s):  
Kazumasa Kase ◽  
Satoshi Watanabe ◽  
Keigo Saeki ◽  
Yuko Waseda ◽  
Hazuki Takato ◽  
...  

2019 ◽  
Vol 5 (2_suppl) ◽  
pp. 31-40 ◽  
Author(s):  
Elizabeth R Volkmann

The natural history of interstitial lung disease in patients with systemic sclerosis is highly variable. Historical observational studies have demonstrated that the greatest decline in lung function in systemic sclerosis occurs early in the course of the disease; however, not all patients experience a decline in lung function even in the absence of treatment. Furthermore, among patients who do experience a decline in lung function, the rate of decline can be either rapid or slow. The most common clinical phenotypes of systemic sclerosis–related interstitial lung disease are, therefore, as follows: (1) rapid progressors, (2) gradual progressors, (3) stabilizers, and (4) improvers. This review summarizes the features of systemic sclerosis–related interstitial lung disease patients who are more likely to experience rapid progression of interstitial lung disease, as well as those who are more likely not to experience interstitial lung disease progression. Understanding the clinical, biological, and radiographic factors that consistently predict interstitial lung disease–related outcomes in systemic sclerosis is central to our ability to recognize those patients who are at heightened risk for interstitial lung disease progression. With new options available for treating patients with systemic sclerosis–related interstitial lung disease, it is more important than ever to accurately identify patients who may derive the most benefit from aggressive systemic sclerosis–related interstitial lung disease therapy. Early therapeutic intervention in patients with this progressive fibrosing phenotype may ultimately improve morbidity and mortality outcomes in patients with systemic sclerosis–related interstitial lung disease.


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