Treatment Of Systemic Sclerosis-Associated Interstitial Lung Disease (Ssc-ILD)

Publication Date: September 29, 2023
Last Updated: January 19, 2024

Treatment

The recommendation for the use of the following agent for the treatment of SSc-ILD is strong:
  • Mycophenolate
(Very low, Strong Recommendation)
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The recommendation for the use of the following agents for the treatment of SSc-ILD is conditional:
Cyclophosphamide (Low, Conditional Recommendation)
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Rituximab (Very low, Conditional Recommendation)
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Tocilizumab (Very low, Conditional Recommendation)
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Nintedanib (Very low, Conditional Recommendation)
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Nintednaib plus mycophenolate (Very low, Conditional Recommendation)
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The recommendation for further research due to insufficient evidence was made for the following agents for the treatment of SSc-ILD and is strong:
Pirfenidone (Very low, Strong Recommendation)
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Pirfenidone plus mycophenolate (Very low, Strong Recommendation)
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Caution must be taken in using systemic corticosteroids in patients with SSc with or without SSc-ILD. Whenever possible, the daily dose should not exceed the equivalent of 15 mg prednisone (Best Practice Statement). (, )
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Video

Recommendation Grading

Disclaimer

The information in this patient summary should not be used as a substitute for professional medical care or advice. Contact a health care provider if you have questions about your health.

Overview

Title

Treatment Of Systemic Sclerosis-Associated Interstitial Lung Disease (Ssc-ILD)

Authoring Organization

Publication Month/Year

September 29, 2023

Last Updated Month/Year

February 14, 2024

Document Type

Guideline

Country of Publication

US

Target Patient Population

Patients with SSc-ILD

Inclusion Criteria

Male, Female, Adult, Older adult

Health Care Settings

Ambulatory

Intended Users

Nurse, nurse practitioner, physician, physician assistant

Scope

Treatment

Diseases/Conditions (MeSH)

D017563 - Lung Diseases, Interstitial, D011014 - Pneumonia, D008171 - Lung Diseases, D011658 - Pulmonary Fibrosis, D054988 - Idiopathic Interstitial Pneumonias, D000092124 - Organizing Pneumonia

Keywords

rituximab, tocilizumab, cyclophosphamide, interstitial lung disease (ILD), systemic sclerosis (SSc), micro-aspiration, proton pump inhibitors (PPI), H2-blockers, prokinetic agents, reticulation, traction bronchiectasis, traction bronchiolectasis, honeycomb cyst, interstitial pneumonia, pleuroparenchymal fibroelastosis, Mycophenolate, Nintedanib, Pirfenidone, systemic corticosteroids

Source Citation

Raghu G, Montesi SB, Silver RM, Hossain T, Macrea M, Herman D, Barnes H, Adegunsoye A, Azuma A, Chung L, Gardner GC, Highland KB, Hudson M, Kaner RJ, Kolb M, Scholand MB, Steen V, Thomson CC, Volkmann ER, Wigley FM, Kemper KA, Knight SL, Ghazipura M. Treatment of Systemic Sclerosis-associated Interstitial Lung Disease: Evidence-based Recommendations. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med. 2023 Sep 29. doi: 10.1164/rccm.202306-1113ST. Epub ahead of print. PMID: 37772985.

Supplemental Methodology Resources

Data Supplement