Pulmonary physiology is poorly associated with radiological extent of disease in systemic sclerosis-associated interstitial lung disease

Jhajj, Amrit; Gill, Harinder Pal; Hague, Cameron; Murphy, Darra; Elicker, Brett M.; Soon, Jeanette; Sulaiman, Nada; Castillo-Saldana, Daniela; Wilcox, Pearce; Dunne, James V.; Lee, Joyce; Wolters, Paul J.; Ley, Brett; Jones, Kirk D.; Morisset, Julie; Ryerson, Christopher J.

doi:10.1183/13993003.02182-2018

Cited by 11 publications

(2 citation statements)

References 15 publications

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“…A seminal study at a UK center found that extensive ILD, defined as > 30% extent of fibrosis on HRCT (based on a mean score of five sections, each estimated to the nearest 5%) or an extent of fibrosis on HRCT of 10–30% with an FVC < 70% predicted, was strongly predictive of mortality (hazard ratio 3.46) [ 14 ]. While most studies have focused on analyses of thresholds, any increase in the extent of fibrotic abnormalities on HRCT has prognostic implications [ 3 , 26 ]. Automated post-processing software is available to quantify the extent of fibrotic changes on CT [ 27 ] but is rarely used in a routine clinical environment.…”

Section: Progression and Monitoring Of Ssc-ildmentioning

confidence: 99%

Detection and Monitoring of Interstitial Lung Disease in Patients with Systemic Sclerosis

2022

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Purpose of Review Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis (SSc). We explore the importance of early detection, monitoring, and management of SSc-ILD. Recent Findings All patients with SSc are at risk of ILD and should be screened for ILD at diagnosis using a high-resolution computed tomography (HRCT) scan. Some patients with SSc-ILD develop a progressive phenotype characterized by worsening fibrosis on HRCT, decline in lung function, and early mortality. To evaluate progression and inform treatment decisions, regular monitoring is important and should include pulmonary function testing, evaluation of symptoms and quality of life, and, where indicated, repeat HRCT. Multidisciplinary discussion enables comprehensive evaluation of the available information and its implications for management. The first-line treatment for SSc-ILD is usually immunosuppression. The antifibrotic drug nintedanib has been approved for slowing lung function decline in patients with SSc-ILD. Summary Optimal management of patients with SSc-ILD requires a multidisciplinary and patient-centered approach.

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Section: Progression and Monitoring Of Ssc-ildmentioning

confidence: 99%

Detection and Monitoring of Interstitial Lung Disease in Patients with Systemic Sclerosis

2022

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“…In 2019, a large multicenter study of the EU-STAR group -European Scleroderma Trials and Research Study Group -covering over 1,000 patients was published. It revealed that the progression of skin lesions within one year were associated with worsening of lung function tests and worse survival in patients with generalized SSc [9,10].…”

Section: Can the Prognosis Of Interstitial Lung Disease In The Coursementioning

confidence: 99%

Clinical and Serological Parameters of Progression And Prognosis in Patients With Systemic Sclerosis – A State of the Art Review

Wielosz

Majdan

2020

Wiad Lek

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Systemic sclerosis (SSc) is a multi-organ connective tissue disease that leads to the dysfunction and the impaired morphology of blood vessels due to non-specific inflammation and progressive fibrosis. Due to the diversity of SSc and even though the factors predisposing to the severe course of SSc are known, it is not always possible to predict the disease progression and to determine the prognosis. Ideally, the group of patients with faster progression of organ lesions and a worse course of the disease should be identified and the early intensive treatment should be instituted. The aim of the article, is an attempt to identify the factors that worsen the prognosis in the course of SSc. The analysis of numerous studies demonstrated that patients with short-lasting SSc, with the presence of anti-RNA polymerase III antibodies, with a generalized type of SSc with quickly progressing skin lesions and males should be most strictly monitored. Moreover, vascular complications, tendon ruptures and fast capillaries loss observed in nailfold capillaroscopy are the factors deteriorating the prognosis in SSc. In conclusion, despite the known, the factors that worsen the prognosis, it is difficult to predict the course of systemic sclerosis. Due to its incompletely elucidated etiopathology as well as the diverse and unpredictable nature of the disease, reliable markers to determine the prognosis in SSc have not been found.

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Lung involvement in systemic sclerosis

Campochiaro

Bruni

Tomassetti

et al. 2022

Handbook of Systemic Autoimmune Diseases

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Pulmonary physiology is poorly associated with radiological extent of disease in systemic sclerosis-associated interstitial lung disease

Cited by 11 publications

References 15 publications

Detection and Monitoring of Interstitial Lung Disease in Patients with Systemic Sclerosis

Detection and Monitoring of Interstitial Lung Disease in Patients with Systemic Sclerosis

Clinical and Serological Parameters of Progression And Prognosis in Patients With Systemic Sclerosis – A State of the Art Review

Lung involvement in systemic sclerosis

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