News Release

Systemic sclerosis-associated interstitial lung disease focus of latest clinical practice guideline

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

Peer-Reviewed Publication

American Thoracic Society

Clinical Practice Guideline


New guidance for clinicians treating patients with systemic sclerosis-associated interstitial lung disease.

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Credit: ATS

In patients with systemic sclerosis (SSc), a rare connective tissue disease, interstitial lung disease contributes to 35 percent of deaths. Published recently in the American Journal of Respiratory and Critical Care Medicine, the latest American Thoracic Society Clinical Practice Guideline makes a number of recommendations. One recommendation expands antifibrotic treatment to all patients with systemic sclerosis associated with ILD (SSc-ILD), not just those with progressive disease.

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Lead guidelines chair and editor, Ganesh Raghu, M.D., ATSF, noted the timeliness of the recommendations given the many immune modulating agents clinicians already use to treat connective tissue diseases, including SSc. “The need to provide evidence-based clinical practice guideline was evident,” he said.

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 thorough, evidence-based Grading of Recommendations, Assessment, Development and Evaluation or GRADE 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:

i :   cyclophosphamide (conditional recommendation, low- quality evidence).

ii :  nintedanib  (conditional recommendation, very low-quality evidence).

iii:   rituximab  (conditional recommendation, very low-quality evidence).

iv:   tocilizumab  (conditional recommendation, very low-quality evidence).

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,” added Dr. Raghu.

To read the latest clinical practice guideline on systemic sclerosis-associated interstitial lung disease in its entirety, visit us online.

For ATS guideline implementation tools and derivatives, go here.

To learn about clinical trials recruiting patients with SSc-ILD, visit the ATS Clinical Trials Resources page. *The ATS does not endorse any trial listed.


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