TABLE 1.

Thoracic manifestations of Sjögren's syndrome

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.