Verlauf der interstitiellen Lungenerkrankung bei systemischer Sklerose

2021 ◽  
pp. 1-2
Author(s):  
Martin Aringer

<b>Objectives:</b> To 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. <b>Methods:</b> Eligible 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. <b>Results:</b> 826 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 &#x3e;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. <b>Conclusion:</b> SSc-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.

2020 ◽  
pp. 1-2
Author(s):  
Martin Aringer

<b>Objectives:</b> To 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. <b>Methods:</b> Eligible 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. <b>Results:</b> 826 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 &#x3e;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. <b>Conclusion:</b> SSc-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.


2020 ◽  
pp. annrheumdis-2020-217455 ◽  
Author(s):  
Anna-Maria Hoffmann-Vold ◽  
Yannick Allanore ◽  
Margarida Alves ◽  
Cathrine Brunborg ◽  
Paolo Airó ◽  
...  

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.


2014 ◽  
Vol 16 (12) ◽  
pp. 1001-1006 ◽  
Author(s):  
Katherine M Simpson ◽  
Luisa De Risio ◽  
Anita Theobald ◽  
Laurent Garosi ◽  
Mark Lowrie

All previous studies on feline ischaemic myelopathy (IM) have reported an acute onset of a single event with no recurrence of clinical signs. This study aimed to evaluate clinical and long-term follow-up data in cats presumptively diagnosed with cervical IM in the territory of the ventral spinal artery (VSA). Eight cats (four females and four males) were included with a mean age of 14 years and 2 months. Neurological status at the time of presentation ranged from ambulatory tetraparesis to tetraplegia with nociception present. Six cats had marked cervical ventroflexion. All eight cats were diagnosed with one or more concurrent medical conditions, including chronic kidney disease (n = 2), hypertrophic cardiomyopathy (n = 2) and hypertension (n = 6). Median time to ambulation was 5.7 days (range 2–14 days). Long-term follow-up ranged from 7 months to 3 years and 3 months (median 1 year and 2 months). Five cats had no reported recurrence of clinical signs and 3/8 had a chronic relapsing disease course. One cat had an acute recurrence of clinical signs 4 months after the first event and was euthanased. Two cats had acute onsets of suspected intracranial infarctions, one of which had further suspected intracranial infarcts every 3 months and was euthanased after one of these. This study highlights the importance of performing ancillary diagnostic tests in older cats presenting with IM, particularly when VSA embolisation is suspected.


2018 ◽  
Vol 54 (7) ◽  
pp. 378-385 ◽  
Author(s):  
Xavier Muñoz ◽  
María José Álvarez-Puebla ◽  
Ebymar Arismendi ◽  
Lourdes Arochena ◽  
María del Pilar Ausín ◽  
...  

2006 ◽  
Vol 155 (suppl_1) ◽  
pp. S59-S64
Author(s):  
Jorn Olsen

The concept that many chronic diseases has an early, even fatal, etiology has inspired funding agencies to support large and long-term follow-up studies starting as early in life as possible. These cohort studies provide new opportunities for studying childhood cancer using data that are less biased than those from case-control studies. However, these studies have to be coordinated to reach sufficient sample sizes and a number of novel ethical concerns have to be solved.


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