2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Treatment of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases

Abstract

Objective: We provide evidence-based recommendations regarding the treatment of interstitial lung disease (ILD) in adults with systemic autoimmune rheumatic diseases (SARDs).

Methods: We developed clinically relevant population, intervention, comparator, and outcomes questions. A systematic literature review was then performed, and the available evidence was rated using the Grading of Recommendations, Assessment, Development, and Evaluation methodology. A panel of clinicians and patients reached consensus on the direction and strength of the recommendations.

Results: Thirty-five recommendations were generated (including two strong recommendations) for first-line SARD-ILD treatment, treatment of SARD-ILD progression despite first-line ILD therapy, and treatment of rapidly progressive ILD. The strong recommendations were against using glucocorticoids in systemic sclerosis-ILD as a first-line ILD therapy and after ILD progression. Otherwise, glucocorticoids are conditionally recommended for first-line ILD treatment in all other SARDs.

Conclusion: This clinical practice guideline presents the first recommendations endorsed by the American College of Rheumatology and American College of Chest Physicians for the treatment of ILD in people with SARDs.

Figure 1.

Initial treatment options for the treatment of ILD associated with systemic autoimmune rheumatic diseases of interest. * Decisions on GC dose and use of oral versus intravenous therapy depend on severity of disease. GCs should be used cautiously in patients with MCTD with an SSc phenotype who may be at increased risk of renal crisis. Short-term is defined as 3 months or less. ^† Treatments are listed in order based on a hierarchy established by head-to-head votes, although the panel noted that decisions on which first-line therapy to use were dependent on specific situations and patient factors. In all diseases, mycophenolate was conditionally recommended over the other listed therapies. Therapies are divided into “preferred” and “additional” options based on the rank-order hierarchy. CNI, calcineurin inhibitor; GC, glucocorticoid; ILD, interstitial lung disease; JAKi, JAK inhibitor; MCTD, mixed connective tissue disease; SSc, systemic sclerosis.

Figure 2.

Management of SARD-ILD with progression of ILD despite first ILD therapy. * If intolerance leads to suboptimal dosing of first-line therapy, consider switching to an alternative first-line therapy. ^† Therapies are generally listed in order based on a hierarchy established by head-to-head votes, but decisions depend on specific clinical situations. Decision on whether to switch therapy or add to current therapy depends on current therapy and on which therapy is being initiated. Cyclophosphamide is not typically used in combination with other therapies, whereas others may be used individually or in combination. ^‡ Decision on use of nintedanib vs immunosuppression depends on pace of progression and amount of fibrotic disease or presence of a usual interstitial pneumonia pattern on CT chest. ^§ JAKi conditionally recommended as an option particularly in patients with anti–MDA-5. ^¶ Short-term glucocorticoids may be of use in some patients with disease flares or as a bridge when switching therapy. AHSCT, autologous hematopoietic stem cell transplantation; CNI, calcineurin inhibitor; CT, computed tomography; GC, glucocorticoid; ILD, interstitial lung disease; IVIG, intravenous immunoglobulin; JAKi, JAK inhibitor; MCTD, mixed connective tissue disease; MDA-5, melanoma differentiation-associated protein 5; SARD, systemic autoimmune rheumatic disease.

Figure 3.

Management of SARD with rapidly progressive ILD. * In rare patients with systemic sclerosis with rapidly progressive ILD, there was no consensus on whether or not to use glucocorticoids; if used, patients should be monitored closely for evidence of renal crisis. ^† Rituximab and cyclophosphamide recommended over IVIG, but IVIG may be preferred if there is high concern for infection. ILD, interstitial lung disease; IV, intravenous; IVIG, intravenous immune globulin; JAKi, JAK inhibitor; MDA-5, melanoma differentiation-associated protein 5; SARD, systemic autoimmune rheumatic disease. Color figure can be viewed in the online issue, which is available at http://onlinelibrary.wiley.com/doi/10.1002/acr.25348/abstract.