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1. The highest risk of disease progression usually occurs within the first _____ years of diagnosis?
2. Which of the following diagnostic tests is the most accurate indicator in diagnosing SSc-ILD and predictor of progression?
3. Lung function measures: DLco, FVC, FEV1:FVC ratio, 6MWT, and TLC should be employed to__________.
4. Risk of progression to severe lung fibrosis is often associated with ___________?
5. Kate, a 56-year-old African American woman presents to your office complaining of becoming slightly more breathless walking up one flight of stairs. She was diagnosed with limited systemic sclerosis 5 years ago. Her current medications include a calcium channel blocker and H-2 receptor blocker. Her cardiac and musculoskeletal exams are normal. Upon further examination, you detect bibasilar Velcro-like crackles in both lungs. An HRCT is ordered and the findings are a bibasilar fibrotic NSIP. Kate has now been diagnosed with SSc-ILD. Kate is concerned about the safety and efficacy of her treatment options.
Given Kate’s disease course and concerns, what treatment should be given as first-line therapy?
6. Both nintedanib and pirfenidone are being studied in clinical trials for SSc-ILD. Pirfenidone will be studied in the phase 2 Scleroderma Lung Study III, which is now recruiting patients, while nintedanib is being evaluated in the soon-to-be completed phase 3 SENSCIS trial.
What is the primary endpoint of the SENSCIS trial?
7. Managing patient expectations for treatment is critical and strong patient engagement can be a factor in improving adherence. Which of the following is true and should be employed in patient counseling?