Scleroderma-related interstitial lung disease

Suliman, Sally; Harash, Abdalhamid Al; Roberts, W N; Perez, Rafael L.; Roman, Jesse

doi:10.1016/j.rmcr.2017.07.007

Cited by 17 publications

(17 citation statements)

References 28 publications

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“…Considering the potentially progressive fibrosing phenotype of SSc-ILD and the risk of rapid deterioration particularly evident in the first 3 years of disease onset, effective and early disease monitoring is of utmost importance 14. In addition to this, monitoring treatment response is important.…”

Section: Monitoring and Predicting The Progression Of Ild In Patientsmentioning

confidence: 99%

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<p>Interstitial Lung Disease in Systemic Sclerosis: Focus on Early Detection and Intervention</p>

Fischer

Patel

Volkmann

2019

OARRR

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Systemic sclerosis (SSc) is a progressive and often devastating disease characterized by autoimmune dysfunction, vasculopathy, and fibrosis. Interstitial lung disease (ILD) is identified in the majority of patients with SSc and is the leading cause of SSc-related mortality. Although clinical manifestations and ILD severity vary among patients, lung function typically declines to the greatest extent during the first 3–4 years after disease onset. We aim to provide an overview of SSc-associated ILD (SSc-ILD) with a focus on current and emerging tools for early diagnosis of ILD and current and novel treatments under investigation. Early detection of ILD provides the opportunity for early therapeutic intervention, which could improve patient outcomes. Thoracic high-resolution computed tomography is the most effective method of identifying ILD in patients with SSc; it enables detection of mild lung abnormalities and plays an important role in monitoring disease progression. Cyclophosphamide and mycophenolate mofetil are the most commonly prescribed treatments for SSc-ILD. Recently, nintedanib (an antifibrotic) was approved by the Food and Drug Administration for patients with SSc-ILD; it is indicated for slowing the rate of decline in pulmonary function. However, there is a need for additional effective and well-tolerated disease-modifying therapy. Ongoing studies are evaluating other antifibrotics and novel agents. We envision that early detection of lung involvement, combined with the emergence and integration of novel therapies, will lead to improved outcomes in patients with SSc-ILD.

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Section: Monitoring and Predicting The Progression Of Ild In Patientsmentioning

confidence: 99%

“…Irrespective of the drug used, the factor that predicted significant improvement in lung function was the initiation of treatment at an early stage in the disease process 13. In line with this, rigorous screening programs to facilitate early diagnosis of SSc-ILD and, hopefully, early initiation of treatment are of paramount importance 14…”

Section: Introductionmentioning

confidence: 99%

<p>Interstitial Lung Disease in Systemic Sclerosis: Focus on Early Detection and Intervention</p>

Fischer

Patel

Volkmann

2019

OARRR

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“…The pathogenesis of SSc-ILD is believed to involve persistent and repeated bouts of injury to endothelial cells, activation of the immune response, and fibroblast recruitment and activation that results in fibrosis or scarring. 5 Patients with SSc-ILD usually present with exertional dyspnea, fatigue, nonproductive cough, and bilateral basilar inspiratory crackles on auscultation. 7,8 Systemic sclerosis-associated interstitial lung disease can be asymptomatic in the early stages, and symptoms are likely to emerge as the disease progresses.…”

Section: Pathogenesismentioning

confidence: 99%

“…The 2 most common pulmonary manifestations are pulmonary hypertension and interstitial lung disease (ILD), accounting for ~60% of SSc-associated mortality. 1,[3][4][5][6] In this review, we will focus on the diagnosis, treatment, and clinician and patient perspectives on ILD.…”

Section: Introductionmentioning

confidence: 99%

Patient and Physician Perspectives on Systemic Sclerosis–Associated Interstitial Lung Disease

Cheema

Young

Rabold

et al. 2020

Clin Med Insights Circ Respir Pulm Med

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Systemic sclerosis–associated interstitial lung disease is challenging to diagnose and treat. Patients and physicians can perceive the disease differently and have different views on its management. Communication issues between them can lead to suboptimal disease management. Despite a clear need for improvement in the speed and accuracy of the diagnostic workup, the heterogeneity of clinical symptoms renders the process long and challenging. When considering treatment options, physicians may be more focused on the evidence supporting a particular treatment or on a patient’s pulmonary function test results, as opposed to the realities of the patient’s difficulties with symptoms or the psychosocial effects of systemic sclerosis–associated interstitial lung disease. Disease management plans should be determined by the patient’s own preferences and goals as well as the objective clinical situation. Health care providers must consider their patients as partners on a journey in which treatment decisions are reached jointly. This review will focus on the perspectives of physicians and patients in relation to the diagnosis and management of systemic sclerosis–associated interstitial lung disease. Similarities and differences in these perspectives will be identified, and strategies for achieving optimal disease management will be proposed.

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“…The standard MMF therapeutic regimen consists of an initial dose of 500 mg twice daily, increased monthly, until a maximum dose of 1.5 g twice daily is reached, administered for 24 months [5][6][7][8]. Due to the potential progressive fibrosing phenotype of SSc-ILD and the risk of rapid deterioration in the patient's health, particularly evident in the first 3 years of disease onset, effective and early disease monitoring is extremely important [9].…”

Section: Introductionmentioning

confidence: 99%

Mycophenolate Mofetil Improves Exercise Tolerance in Systemic Sclerosis Patients with Interstitial Lung Disease: A Pilot Study

Vaiarello

Schiavetto²,

Foti

et al. 2020

Rheumatol Ther

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Introduction: Systemic sclerosis (SSc) is an autoimmune disease characterized by the overproduction of collagen leading to fibrosis of the skin and internal organs. Interstitial lung disease (ILD) is one of the major causes of death in patients with SSc. Exercise tolerance can be investigated by cardio-pulmonary exercise testing (CPET). First-line therapies in patients with SSc associated with ILD (SSc-ILD) include cyclophosphamide and mycophenolate mofetil (MMF). The aim of this study was to evaluate the response of patients with SSc-ILD to MMF by means of CPET. Methods: Ten consecutive SSc patients were enrolled in this study. All SSc patients underwent clinical evaluation, echocardiography, pulmonary function tests, high-resolution computed tomography (HRCT) and CPET at baseline and after 2 years of therapy with MMF. Results: After 24 months of treatment with MMF (target dose 1500 mg twice daily), forced vitality capacity, diffusing capacity of the lungs for carbon monoxide and systolic pulmonary arterial pressure had not improved significantly and there were no significant differences in HRCT findigns. In addition, peak oxygen uptake (V 0 O 2 peak) and ventilatory equivalents for carbon dioxide production (V 0 E/V 0 CO 2 slope) had not improved significantly. In contrast, there was a significant improvement from baseline to 24 months of treatment in the respiratory exchange ratio [median (interquartile range): 1.07 (0.92-1.22) vs. 1.26 (1.22-1.28), respectively; p \ 0.01] and in the Borg scale for leg discomfort [median (interquartile range): 5 (5-7) vs. 4 (3-4), respectively; p \ 0.01]. Conclusion: These data from our pilot study on a small cohort of SSc patients are the first to demonstrate that treatment with MMF can improves exercise tolerance and leg discomfort in patients with SSc-ILD. These preliminary results need to be confirmed in large randomized studies.

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Scleroderma-related interstitial lung disease

Cited by 17 publications

References 28 publications

<p>Interstitial Lung Disease in Systemic Sclerosis: Focus on Early Detection and Intervention</p>

<p>Interstitial Lung Disease in Systemic Sclerosis: Focus on Early Detection and Intervention</p>

Patient and Physician Perspectives on Systemic Sclerosis–Associated Interstitial Lung Disease

Mycophenolate Mofetil Improves Exercise Tolerance in Systemic Sclerosis Patients with Interstitial Lung Disease: A Pilot Study

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