The new American Thoracic Society guideline provides evidence to use specific immune modulating and antifibrotic medications to treat all patients with SSc-ILD, not just those with progressive disease.

A new American Thoracic Society clinical practice guideline recommends expanding antifibrotic treatment to all patients with systemic sclerosis associated with ILD (SSc-ILD), not just those with progressive disease.

Lead guidelines chair and editor Ganesh Raghu, MD, ATSF, noted the timeliness of the recommendations given the many immune-modulating agents clinicians already use to treat connective tissue diseases, including SSc, a rare connective tissue disease. In patients with SSc, interstitial lung disease contributes to 35% of deaths.

“The need to provide evidence-based clinical practice guidelines was evident,” he says in a release.

It was with this in mind that the 24-member international expert panel (that included pulmonologists, rheumatologists, methodologists, and patients with SSc) convened. Using the evidence-based Grading of Recommendations, Assessment, Development, and Evaluation framework, the expert panel made the following recommendations and suggestions for treating  patients with SSc-ILD:

  1. We recommend using mycophenolate to treat patients with SSc-ILD (strong recommendation, very low-quality evidence).
  2. We suggest using the following medications to treat patients with SSc-ILD:
    1. i : cyclophosphamide (conditional recommendation, low-quality evidence).
    2. ii : nintedanib (conditional recommendation, very low-quality evidence).
    3. iii: rituximab (conditional recommendation, very low-quality evidence).
    4. iv: tocilizumab (conditional recommendation, very low-quality evidence).
    5. v: the combination of nintedanib plus mycophenolate (conditional recommendation, very low-quality evidence).
  3. We recommend further research into the safety and efficacy of pirfenidone alone or in combination with mycophenolate to treat patients with SSc-ILD.

Regarding treatment with antifibrotics like nintedanib and pirfenidone, “We emphasize the importance of the shared decision process with patients and physicians, especially for the recommendations that are conditional,” adds Raghu in the release.

Watch the explainer video.

Photo 146108416 © Madamlead |