From the Guidelines
Pulmonary involvement in Sjögren's syndrome is a potentially serious complication that affects approximately 16% of patients, leading to higher mortality and lower quality of life, and interstitial lung disease is the most common pulmonary manifestation. The pulmonary involvement in Sjögren's syndrome can manifest in several ways, including:
- Interstitial lung disease, with subtypes such as nonspecific interstitial pneumonia, lymphocytic interstitial pneumonia, usual interstitial pneumonia, and organizing pneumonia
- Other lung complications, including bronchiectasis, bronchiolitis, and pleural effusions
- Symptoms such as dry cough, shortness of breath, and decreased exercise tolerance, although some patients may remain asymptomatic despite radiographic abnormalities
Diagnosis of pulmonary involvement in Sjögren's syndrome typically involves:
- High-resolution CT scans
- Pulmonary function tests showing restrictive patterns with decreased diffusion capacity
- Occasionally, lung biopsies
Treatment of pulmonary involvement in Sjögren's syndrome depends on the severity and type of lung involvement, with:
- Mild cases often monitored without specific therapy
- Corticosteroids like prednisone (starting at 0.5-1 mg/kg/day with gradual tapering) as first-line treatment for progressive or symptomatic disease, often combined with immunosuppressants such as mycophenolate mofetil (1-3g daily), azathioprine (1-2.5 mg/kg/day), or cyclophosphamide for severe cases
- Rituximab (1000mg IV given twice, two weeks apart) may be considered for refractory cases
- Regular pulmonary function monitoring every 3-6 months is recommended to assess disease progression and treatment response, as emphasized by the consensus guidelines for evaluation and management of pulmonary disease in Sjögren's 1.
From the Research
Pulmonary Involvement in Sjögren's Syndrome
Pulmonary involvement in Sjögren's syndrome can manifest in various forms, including:
- Interstitial lung disease (ILD) 2, 3, 4, 5, 6
- Airways disease 3, 6
- Lymphocytic interstitial pneumonia 2, 4, 5, 6
- Nonspecific interstitial pneumonia (NSIP) 2, 5
- Usual interstitial pneumonia (UIP) 2, 5
- Organising pneumonia 2, 5
- Bronchiolitis and bronchiectasis 6
- Pulmonary hypertension 6
- Pleural disease 6
- Amyloidosis 5, 6
- Granulomatous lung disease 6
- Pseudolymphoma 6
- Primary pulmonary lymphoma 5, 6
Prevalence of Pulmonary Involvement
The prevalence of pulmonary involvement in Sjögren's syndrome is estimated to be around 9-22% 3, with some studies suggesting that up to 20% of patients may develop ILD 2. Subclinical lung disease is even more frequent, with evidence of small airways disease and airway inflammation often present 6.
Clinical Features and Diagnosis
Pulmonary involvement in Sjögren's syndrome can present with a range of clinical features, including:
- Respiratory symptoms such as dyspnea and cough 4, 5
- Radiographic abnormalities such as bilateral infiltrates and ground-glass opacities 5
- Pulmonary function test abnormalities such as a restrictive functional pattern 4
- Histopathologic findings on lung biopsy, including various patterns of ILD 4, 5
Treatment and Management
Treatment for pulmonary involvement in Sjögren's syndrome often involves the use of glucocorticoids and immunosuppressive agents such as azathioprine, cyclophosphamide, and mycophenolate mofetil 2, 4, 5. The antifibrotic drug nintedanib has also shown efficacy in slowing the progression of ILD in patients with connective tissue diseases, including Sjögren's syndrome 2. However, evidence-based guidelines for the treatment of pulmonary involvement in Sjögren's syndrome are largely lacking 3.