Figures
- FIGURE 1
a) Axial computed tomography (CT) showing mild thickening of bronchial walls (arrows) in a woman with Sjögren's syndrome. b) Chronic cough and recurrent pulmonary infections associated with bronchiolitis in a 60-year-old woman with primary Sjögren's syndrome. High-resolution CT showing multiple ill-defined centrilobular nodules (arrowheads) with sparing of the subpleural region. c, d) Follicular bronchiolitis in a 54-year-old woman with primary Sjögren's syndrome. c) High-resolution CT showing an area of heterogeneous attenuation in the left lower lobe of the lung (a finding referred to as mosaic attenuation) and the presence of well-defined, round, thin-walled air cysts (arrows). d) Photomicrograph (haematoxylin–eosin stain) showing lymphoplasmacytic infiltration and lymphoid follicles in the bronchiolar wall (arrowheads) and mild inflammation in the adjacent alveolar area. These findings are indicative of follicular bronchiolitis. Original magnification ×40.
- FIGURE 2
a) Usual interstitial pneumonia in a 69-year-old woman with primary Sjögren's syndrome. High-resolution computed tomography (CT) showing bilateral reticular areas and honeycombing with peripheral and basal predominance (arrows). b) Combined pulmonary fibrosis and emphysema syndrome in a 68-year-old smoker with primary Sjögren's syndrome complicated by pulmonary hypertension. High-resolution CT shows bilateral reticular areas and honeycombing with posterior and basal predominance. Emphysema is predominant in apical areas. c, d) Lymphocytic interstitial pneumonia in a 59-year-old woman with primary Sjögren's syndrome and lymphocytic alveolitis. c) High-resolution CT shows thickening of interlobular septa with superimposition of intralobular reticulation. d) Photomicrograph (haematoxylin–eosin stain) shows diffuse thickening of alveolar septa and peribronchiolar infiltration with lymphocytes and plasma cells (arrowheads). Original magnification ×100.
- FIGURE 3
Nonspecific interstitial pneumonia in a woman a, c) at the time of Sjögren's syndrome diagnosis and b) after 3 years. a, b) High-resolution computed tomography (CT) show bilateral areas of ground-glass attenuation and traction bronchiectasis (arrowheads) with peripheral intralobular reticulation. c) Photomicrograph (haematoxylin–eosin stain) shows diffuse and homogenous collagenous fibrosis in the alveolar area. d) Organising pneumonia and primary Sjögren's syndrome revealed by acute onset of pleuropneumonia in a 64-year-old man. High-resolution CT images show bilateral patchy areas of consolidation (#) and areas of ground-glass opacity (*). The patient was diagnosed with nonspecific interstitial pneumonia 2 years later.
- FIGURE 4
a) Mucosa-associated lymphoid tissue lymphoma in a 45-year-old woman with primary Sjögren's syndrome. High-resolution computed tomography (CT) shows air space consolidation (*), mass (#) and interlobular septal thickening (arrowheads) in the right lower lobe. b) High-resolution CT obtained in a 64-year-old woman with lymphocytic interstitial pneumonitis and primary Sjögren's syndrome shows well-defined, round, thin-walled air cysts in the peribronchovascular regions (arrows), and areas of ground-glass and reticular attenuation.
Tables
- 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 solutionBHR 42–60%# Inhaled corticosteroids Bronchiolitis 12–24%# Mainly follicular bronchiolitis Steroids
Rituximab
MacrolidesBronchiectasis 7–54%# Mainly cylindrical bronchiectasis Pulmonary infections 10–35%# Interstitial lung disease Nonspecific interstitial
pneumonia45%¶ Steroids
Hydroxychloroquine
Azathioprine
Cyclophosphamide
RituximabUsual interstitial pneumonia 16%¶ No benefit of immunosuppressive drugs Lymphocytic interstitial
pneumonitis15%¶ Steroids
Azathioprine
Cyclophosphamide
Chlorambucil
RituximabOrganising pneumonitis 11%# Steroids
Azathioprine
Cyclosporine
Infliximab
Rituximab
TocilizumabOthers pulmonary manifestations in Sjögren's syndrome Pulmonary amyloidosis Rare 96.5% female Steroids Pulmonary lymphoma 2%¶ Specific haematological treatment Pulmonary embolism and
pulmonary hypertensionRare 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.
- TABLE 2
Screening pulmonary manifestations of Sjögren's syndrome
When Sjögren's syndrome is known, ILD screening should be systematic When ILD is known, Sjögren's syndrome screening must be systematic Investigate respiratory symptoms (dyspnoea, cough, crackles and squeaks, clubbing)
Exclude an infectious, medication (www.pneumotox.com) or cardiovascular cause
Confirm ILD by performing chest computed tomography
Consultation with a lung specialist to discuss bronchoscopy with BAL, histological sampling (transbronchial and surgical biopsy) and therapeutic careInvestigate extra-respiratory symptoms suggestive of Sjögren's syndrome (keratoconjunctivitis sicca, swelling of the salivary glands or hyposialia, arthritis, arthralgia), perform a minor salivary gland biopsy and do a urine analysis
Investigate antinuclear antibodies, rheumatoid factor, SSA/Ro and SSB/La, and perform serum protein electrophoresis
Exclude another connective tissue disease
Confirm dry eyes and dry mouthConsult a connective tissue disease expert to discuss therapeutic care ILD: interstitial lung disease; BAL: bronchoalveolar lavage.
Disclosures
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