Thoracic manifestations | Prevalence | Peculiar aspects in Sjögren's syndrome | Treatment |
Airway disease | |||
Cough | 41–61%# | Secretagogues (pilocarpine) Nebulised saline solution | |
BHR | 42–60%# | Inhaled corticosteroids | |
Bronchiolitis | 12–24%# | Mainly follicular bronchiolitis | Steroids Rituximab Macrolides |
Bronchiectasis | 7–54%# | Mainly cylindrical bronchiectasis | |
Pulmonary infections | 10–35%# | ||
Interstitial lung disease | |||
Nonspecific interstitial pneumonia | 45%¶ | Steroids Hydroxychloroquine Azathioprine Cyclophosphamide Rituximab | |
Usual interstitial pneumonia | 16%¶ | No benefit of immunosuppressive drugs | |
Lymphocytic interstitial pneumonitis | 15%¶ | Steroids Azathioprine Cyclophosphamide Chlorambucil Rituximab | |
Organising pneumonitis | 11%# | Steroids Azathioprine Cyclosporine Infliximab Rituximab Tocilizumab | |
Others pulmonary manifestations in Sjögren's syndrome | |||
Pulmonary amyloidosis | Rare | 96.5% female | Steroids |
Pulmonary lymphoma | 2%¶ | Specific haematological treatment | |
Pulmonary embolism and pulmonary hypertension | Rare | Risk of venous thrombosis or pulmonary embolism in Sjögren's syndrome patients is greater than in the general population |
BHR: bronchial hyperresponsiveness. #: in Sjögren's syndrome patients; ¶: of interstitial lung disease in Sjögren's syndrome.