From the Guidelines
Treatment for interstitial lung disease (ILD) associated with Sjögren's syndrome typically begins with corticosteroids, such as prednisone, and immunosuppressive agents like mycophenolate mofetil or azathioprine, as first-line therapy, according to the 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline 1.
First-Line Treatment Options
- Corticosteroids, such as prednisone, at a dose of 0.5-1 mg/kg/day for acute exacerbations, followed by a gradual taper over several months 1.
- Immunosuppressive agents, including mycophenolate mofetil (1-3 g/day in divided doses) or azathioprine (1-2.5 mg/kg/day), as maintenance therapy or steroid-sparing agents 1.
Additional Considerations
- Rituximab (1000 mg IV given two weeks apart) may be considered for refractory cases or as an alternative to immunosuppressive agents 1.
- Supportive care, including supplemental oxygen for patients with hypoxemia, pulmonary rehabilitation, vaccination against pneumococcal pneumonia and influenza, and smoking cessation, is essential 1.
- Regular monitoring with pulmonary function tests every 3-6 months and high-resolution CT scans annually or as clinically indicated is recommended to assess treatment response 1.
Disease Severity and Patient Comorbidities
- Treatment should be individualized based on ILD subtype, disease severity, and patient comorbidities 1.
- The 2023 ACR/CHEST guideline provides a framework for managing SARD-ILD, including Sjögren's syndrome, with consideration of disease severity and patient comorbidities 1.
From the Research
Treatment Options for Sjogren's Syndrome ILD
- Glucocorticoids are empirically used, usually in association with immunosuppressive drugs, such as cyclophosphamide and mycophenolate mofetil 2
- A trial showed the efficacy of the antifibrotic drug nintedanib in slowing progression of various interstitial lung diseases, including patients with connective tissue diseases 2
- Combination of systemic tacrolimus and abatacept has been successfully used in severe refractory SS and related ILD 3
- Azathioprine and mycophenolate therapy have been assessed in adult patients with pSS-ILD, with mycophenolate treatment associated with significant improvement in PFTs 4
- Immunosuppressive therapy has been successfully used to treat ILD in anti-SS-A and ACA double-positive cases 5
Treatment Outcomes
- The course of ILD can be improvement (15.8%), stabilization (47.4%), or deterioration (36.8%) 6
- Predictive parameters of ILD onset include older age, Raynaud's phenomenon, and esophageal involvement 6
- Factors associated with ILD deterioration include older age and esophageal involvement 6
Treatment Considerations
- Multidisciplinary discussion may be necessary in pSS cases with ambiguous clinical findings, when differential diagnosis with IIPs might be very difficult 2
- Systematic screening of pulmonary involvement is required in pSS patients, resulting in both diagnosis and management at an early stage of ILD 6
- Patients presenting predictive factors of ILD deterioration may need a closer follow-up and a more aggressive therapy 6