Using a modified Delphi study, expert consensus recommendations for the management of systemic sclerosis‑associated interstitial lung disease (SSc-ILD) were developed. The recommendations were published in Respiratory Research.
The recommendations were developed by an expert panel of physicians, including 13 rheumatologists and 12 pulmonologists.
Based on literature review and clinical experience, the panelists reviewed statements related to the management of SSc-ILD. They rated their agreement with each statement on a Likert scale from -5 (complete disagreement) to +5 (complete agreement). Consensus was defined by a mean Likert scale score between -2.5 and +2.5. Overall, consensus was achieved for 109 of 239 reviewed statements (45.6%).
Based on the expert consensus, recommendations for SSc-ILD management were developed.
- The panelists recommended screening all patients with SSc for ILD, especially patients older than 70 years, those with respiratory symptoms, and those at high risk for ILD, including those with a high modified Rodnan skin score.
- Recommended screening methods should include chest auscultation, full pulmonary function tests (PFTs), including spirometry with diffusing capacity of the lungs for carbon dioxide (DLCO), high-resolution computed tomography (HRCT), and autoantibody testing.
- The panelists also recommended routine screening for pulmonary hypertension in patients with SSc, particularly among those with shortness of breath not explained by progression of ILD.
- The panelists recommended the initiation of treatment for patients with abnormal or progressive findings on HRCT or forced vital capacity (FVC) less than 80%.
- Treatment should be initiated in patients with FVC greater than 80%, if it is accompanied by ILD in a high-risk patient, dyspnea, a notable decline in FVC, or peripheral capillary oxygen desaturation (SpO2) on exercise.
- Immediate treatment was recommended for patients with moderate to severe ILD on HRCT (or >20% lung involvement), FVC and/or DLCO below normal limits, rapidly progressing diffuse cutaneous SSc, moderate to severe symptoms, hypoxemia at rest, or desaturation during exercise.
- The panelists recommended against treatment for patients with long disease duration, stable PFTs, and no progression in ILD.
- The panelists recommended mycophenolate mofetil (MMF) as first-line treatment for patients with SSc-ILD at a target dose of 2000 to 3000 mg daily.
- Nintedanib therapy was recommended for patients with longstanding (>5 years) SSc-ILD and evidence of progression for whom immunosuppression is not recommended.
- Nintedanib has been considered an appropriate add-on therapy along with MMF and cyclophosphamide (CYC) following an inadequate response or intolerance with MMF or CYC or with tocilizumab.
- The panelists recommended against using methotrexate as initial therapy.
- Based on a general consensus, antifibrotic drugs may be considered in patients with a decline in PFTs and/or HRCT.
- Generally, antifibrotics were not recommended as monotherapy but have been considered for use in combination with MMF or CYC.
- The panelists agreed that tocilizumab should be considered in patients with early SSc and ILD with elevated acute-phase reactants. However, consensus was not reached on the use of tocilizumab in patients with an inadequate response to initial therapy.
- Tocilizumab was also recommended as an option for patients unable to tolerate MMF, CYC, or antifibrotics due to adverse effects.
Follow-Up and Managing Progression
- Consensus was reached that patients should be followed-up with for changes in PFTs, symptoms over time, changes on HRCT, and exertional hypoxia.
- The panelists recommended the addition of another therapeutic agent in patients with ILD progression after initial therapy.
- “Success” was defined as the stabilization or improvement of FVC, DLCO, HRCT chest scans, symptoms, and/or oxygen saturation during exercise.
- Tapering/weaning therapy was recommended for patients with no symptom progression for more than 2 years.
- The panelists recommended that tapering/weaning take place over a period of 1 to 2 years with monitoring PFTs every 6 months.
“These differences highlight the importance of collaborative management of patients and the clinical impact of multidisciplinary discussion groups,” the study authors wrote. “Findings from this study provide a management algorithm that will be critical for treating patients with SSc-ILD and help expand on the latest guidelines with clinical expertise and consideration of recently published trials in SSc-ILD,” they added.
Disclosure: Several study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of authors’ disclosures.
Rahaghi FF, Hsu VM, Kaner RJ, et al. Expert consensus on the management of systemic sclerosis-associated interstitial lung disease. Respir Res. 2023;24(1):6. doi:10.1186/s12931-022-02292-3