2017
DOI: 10.1016/j.rmcr.2017.07.007
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Scleroderma-related interstitial lung disease

Abstract: Scleroderma-related interstitial lung disease (SSc-ILD) is a pulmonary fibrosing disorder characterized by systemic inflammation and progressive scarring of the lungs that leads to respiratory failure. Although certain immunosuppressive therapies may slow disease progression, current treatment strategies are not curative; consequently, SSc-ILD continues to be a major cause of morbidity and mortality. We present four cases of SSc-ILD that emphasize the importance of early screening and detection, close follow-u… Show more

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Cited by 17 publications
(17 citation statements)
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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%
See 1 more Smart Citation
“…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%
“…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%
“…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%
“…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%