Natural variability in the disease course of SSc-ILD: implications for treatment

Vonk, Madelon C.; Walker, Ulrich A.; Volkmann, Elizabeth R.; Kreuter, Michael; Allanore, Yannick

doi:10.1183/16000617.0340-2020

Cited by 25 publications

(13 citation statements)

References 85 publications

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“…Variation of disease course is well known in SSc, where patients can have a rapid progression, stability of disease, and even improvement. Our data confirmed that most patients’ lung function remained stable over the 2-year period; some of them even improved similarly to the Scleroderma Lung Study (SLS) I and II trials and SENSCIS ( Volkmann et al, 2017 ), ( Vonk et al, 2021 ).…”

Section: Discussionsupporting

confidence: 78%

Autoimmune Progressive Fibrosing Interstitial Lung Disease: Predictors of Fast Decline

Nagy

Kolonics-Farkas

et al. 2021

Front. Pharmacol.

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A subset of interstitial lung diseases (ILDs) with autoimmune traits—including connective tissue disease-associated ILD (CTD-ILD) and interstitial pneumonia with autoimmune features (IPAF)—develops progressive fibrosing (PF)-ILD. The aim of our study was to evaluate the clinical characteristics and predictors of longitudinal lung function (LF) changes in autoimmune PF-ILD patients in a real-world setting. All ILD cases with confirmed or suspected autoimmunity discussed by a multidisciplinary team (MDT) between January 2017 and June 2019 (n = 511) were reviewed, including 63 CTD-ILD and 44 IPAF patients. Detailed medical history, LF test, diffusing capacity of the lung for carbon monoxide (DLCO), 6-min walk test (6MWT), blood gas analysis (BGA), and high-resolution computer tomography (HRCT) were performed. Longitudinal follow-up for functional parameters was at least 2 years. Women were overrepresented (70.1%), and the age of the IPAF group was significantly higher as compared to the CTD-ILD group (p < 0.001). Dyspnea, crackles, and weight loss were significantly more common in the IPAF group as compared to the CTD-ILD group (84.1% vs. 58.7%, p = 0.006; 72.7% vs. 49.2%, p = 0.017; 29.6% vs. 4.8%, p = 0.001). Forced vital capacity (FVC) yearly decline was more pronounced in IPAF (53.1 ± 0.3 vs. 16.7 ± 0.2 ml; p = 0.294), while the majority of patients (IPAF: 68% and CTD-ILD 82%) did not deteriorate. Factors influencing progression included malignancy as a comorbidity, anti-SS-A antibodies, and post-exercise pulse increase at 6MWT. Antifibrotic therapy was administered significantly more often in IPAF as compared to CTD-ILD patients (n = 13, 29.5% vs. n = 5, 7.9%; p = 0.007), and importantly, this treatment reduced lung function decline when compared to non-treated patients. Majority of patients improved or were stable regarding lung function, and autoimmune-associated PF-ILD was more common in patients having IPAF. Functional decline predictors were anti-SS-A antibodies and marked post-exercise pulse increase at 6MWT. Antifibrotic treatments reduced progression in progressive fibrosing CTD-ILD and IPAF, emphasizing the need for guidelines including optimal treatment start and combination therapies in this special patient group.

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Section: Discussionsupporting

confidence: 78%

Autoimmune Progressive Fibrosing Interstitial Lung Disease: Predictors of Fast Decline

Nagy

Kolonics-Farkas

et al. 2021

Front. Pharmacol.

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“…Another significant cardiopulmonary complication accounting for additional high mortality is pulmonary hypertension (PH) [ 3 , 4 ]. The clinical course of this disease shows a high variability in progression [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

Clinical Predictors of Lung-Function Decline in Systemic-Sclerosis-Associated Interstitial Lung Disease Patients with Normal Spirometry

et al. 2022

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Interstitial lung disease (ILD) is the leading cause of mortality in systemic sclerosis (SSc). Progressive pulmonary fibrosis (PPF) is defined as progression in 2 domains including clinical, radiological or lung-function parameters. Our aim was to assess predictors of functional decline in SSc-ILD patients and compare disease behavior to that in idiopathic pulmonary fibrosis (IPF) patients. Patients with normal forced vital capacity (FVC > 80% predicted; SSc-ILD: n = 31; IPF: n = 53) were followed for at least 1 year. Predictors of functional decline including clinical symptoms, comorbidities, lung-function values, high-resolution CT pattern, and treatment data were analyzed. SSc-ILD patents were significantly younger (59.8 ± 13.1) and more often women (93 %) than IPF patients. The median yearly FVC decline was similar in both groups (SSc-ILD = −67.5 and IPF = −65.3 mL/year). A total of 11 SSc-ILD patients met the PPF criteria for functional deterioration, presenting an FVC decline of −153.9 mL/year. Cough and pulmonary hypertension were significant prognostic factors for SSc-ILD functional progression. SSc-ILD patients with normal initial spirometry presenting with cough and PH are at higher risk for showing progressive functional decline.

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“…SSc-ILD has a variable natural history [ 20 ]. Some patients have relatively stable disease for a prolonged period, while others develop a progressive phenotype characterized by worsening fibrosis on HRCT, progressive decline in lung function, worsening dyspnea, and an increase in mortality [ 3 ].…”

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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Natural variability in the disease course of SSc-ILD: implications for treatment

Cited by 25 publications

References 85 publications

Autoimmune Progressive Fibrosing Interstitial Lung Disease: Predictors of Fast Decline

Autoimmune Progressive Fibrosing Interstitial Lung Disease: Predictors of Fast Decline

Clinical Predictors of Lung-Function Decline in Systemic-Sclerosis-Associated Interstitial Lung Disease Patients with Normal Spirometry

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

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