• Autoimmune disorder
  
• Commonly associated with other connective tissue diseases (secondary SS)
  
• Affects middle aged females
  
• Widespread tissue infiltration by polyclonal B-lymphocytes
  
• Keratoconjunctivitis sicca, xerostomia, recurrent swelling of the parotid gland
  
• Radiography: interstitial lung disease (UIP, LIP), cystic lung disease, recurrent pneumonias, airway disease
  
• Increased risk for pseudolymphoma and lymphoma

Chest radiography

• CXR abnormal (< 33%)
  
• Reticulonodular pattern, basal predominance (most common)
  
• Bronchial wall thickening, bronchiectasis
  
• Recurrent bronchopneumonias
  
• Atelectasis
  
• Pleural effusion or thickening (uncommon)
  
• Pulmonary artery hypertension (uncommon)
  
• Lymphadenopathy suggests pseudolymphoma or malignant lymphoma
  

CT/HRCT

• Basal predominance
  
• Bronchiolectasis, centrilobular nodules or branching opacities
  
• Mosaic attenuation, air trapping
  
• Thin-walled cysts (5 – 30 mm)
  
• Linear opacities (septal and nonseptal)
  
• Airspace opacities – bronchopneumonia or pseudolymphoma
  
• Pleural effusion or thickening (uncommon)

• UIP
  
• Collagen vascular disease
  
• Drug reaction
  
• Asbestosis
  
• Cystic disease
• Langerhans cell granulomatosis
  
• Tuberous sclerosis
  
• Lymphangiomyomatosis
  
• Pneumocystis carinii pneumonia

• Autoimmune process
  
• Possible viral etiology: EB, hepatitic C, herpes, retrovirus
  
• Infiltration of tissues with polyclonal B-lymphocytes
  
• Lymphocytic infiltration, glandular atrophy, impaired secretion of lacrimal, salivary, airway mucous glands
• Pleuropulmonary abnormalities (30%):
  
  • Airway disease
    
    • Tracheobronchitis
      
    • Follicular bronchiolitis (peribronchiolar lymphoplasmacytic infiltrates)
      
    • BOOP
      
  • Recurrent pneumonias
    
  • Interstitial lung disease (33%)
    
  • LIP (diffuse)
    
  • Pseudolymphoma (localized)
    
  • UIP
    
  • Pleuritis with or without effusion, pleural thickening (10%)
    
  • Lymphadenitis
    
  • Plexogenic pulmonary arteriopathy (rare)
    
  • Amyloidosis
    
• Systemic necrotizing vasculitis, small and large vessels
  
• Risk for non-Hodgkins lymphoma
  

• Females (90%), mean age 57 years
  
• Sicca syndrome: dry eyes, mouth, nose
  
• Lacrimal, submandibular and parotid gland enlargement
  
• Hoarseness, cough, pleuritic pain, dyspnea
  
• Recurrent infections, bronchitis, pneumonia (secondary to impaired secretions)
  
• Associated with other autoimmune diseases: chronic active hepatitis, primary biliary cirrhosis, Hashimoto’s thyroiditis, myasthenia gravis, celiac disease, renal tubular disorders, myopathy, neuropathy, CNS disorders, Raynaud’s, vasculitis,purpura, polyarthropathy, thrombocytopenic purpura (rare) hypothyroidism, splenomegaly
  
• (50-67%) have connective tissue disease (secondary SS)
  
• Secondary SS associated with RA, PSS, SLE, polymyositis
  
• Positive RF (90%), ANA (70%)
  
• Lymphopenia, leukopenia, polyclonal gammopathy IgG, IgM
  
• Diagnosis
  
  • Sicca syndrome
    
  • Abnormal Schirmer’s test/Rose Bengal test
    
  • Biopsy of minor salivary glands
    
  • Parotid sialography
    
  • Detection of antibodies to extractable nuclear antigens (SS-A, SS-B)
    
• PFTs - obstructuve, restrictive, or diffusion abnormalities; hyperresponsive airway
  
• BAL – lymphocytosis
  
• Both primary and secondary SS – increased risk for pseudolymphoma, lymphoma
  
• Prognosis, poorly described
  
  • Primary SS, may progress rapidly

Strimlan CV, Rosenow EC, 3rd, Divertie MB, et al. Pulmonary manifestations of Sjogren's syndrome Chest 70:354-361, 1976
Meyer CA, Pina JS, Taillon D, et al. Inspiratory and expiratory high-resolution CT findings in a patient with Sjogren's syndrome and cystic lung disease AJR 168:101-103, 1997