Nonspecific interstitial pneumonia (NSIP) is most common ILD pattern in patients with SS (~ 45%)
Airways disease
Small airways disease
Chronic bronchiolitis
Follicular bronchiolitis,
Constrictive bronchiolitis
Upper airway inflammation and dryness
Lymphoproliferative disorders
Increased risk of non-Hodgkin lymphoma (NHL)
Marginal zone B cell lymphoma and mucosa-associated lymphoid tissue (MALT) lymphoma or MALToma: Most common subtypes in SS
IMAGING
Radiographic Findings
CT Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Heus A et al: Pulmonary involvement in primary Sjögren's syndrome, as measured by the ESSDAI. Scand J Rheumatol. 49(1):38-46, 2020
Luppi F et al: Lung complications of Sjogren syndrome. Eur Respir Rev. 29(157), 2020
Chung A et al: Pulmonary and bronchiolar involvement in Sjogren's syndrome. Semin Respir Crit Care Med. 40(2):235-54, 2019
Natalini JG et al: Pulmonary involvement in Sjögren syndrome. Clin Chest Med. 40(3):531-44, 2019
Ohno Y et al: State-of-the-art imaging of the lung for connective tissue disease (CTD). Curr Rheumatol Rep. 17(12):69, 2015
Ruano CA et al: Thoracic manifestations of connective tissue diseases. Curr Probl Diagn Radiol. 44(1):47-59, 2015
Kreider M et al: Pulmonary involvement in Sjögren syndrome. Semin Respir Crit Care Med. 35(2):255-64, 2014
Egashira R et al: CT findings of thoracic manifestations of primary Sjögren syndrome: radiologic-pathologic correlation. Radiographics. 33(7):1933-49, 2013
Stojan G et al: Pulmonary manifestations of Sjögren's syndrome. Curr Allergy Asthma Rep. 13(4):354-60, 2013
Tsai Y et al: Thymoma associated with myasthenia gravis and Sjögren syndrome. West Indian Med J. 62(3):264-5, 2013
Borie R et al: Severe chronic bronchiolitis as the presenting feature of primary Sjögren's syndrome. Respir Med. 105(1):130-6, 2011
Shi JH et al: Pulmonary manifestations of Sjögren's syndrome. Respiration. 78(4):377-86, 2009
Related Anatomy
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
Sjögren syndrome (SS)
Synonyms
Sicca syndrome
Gougerot-Sjögren disease
Definitions
Autoimmune disease characterized by dryness of eyes and mouth
Caused by chronic lymphocytic infiltration of exocrine glands and other extraglandular sites
Second most common autoimmune disorder after rheumatoid arthritis
SS can be primary or associated with other connective tissue diseases, most commonly rheumatoid arthritis
Clinically significant pulmonary involvement in 9-24% of affected patients and more common in secondary than in primary SS
Nonspecific interstitial pneumonia (NSIP) is most common ILD pattern in patients with SS (~ 45%)
Airways disease
Small airways disease
Chronic bronchiolitis
Follicular bronchiolitis,
Constrictive bronchiolitis
Upper airway inflammation and dryness
Lymphoproliferative disorders
Increased risk of non-Hodgkin lymphoma (NHL)
Marginal zone B cell lymphoma and mucosa-associated lymphoid tissue (MALT) lymphoma or MALToma: Most common subtypes in SS
IMAGING
Radiographic Findings
CT Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Heus A et al: Pulmonary involvement in primary Sjögren's syndrome, as measured by the ESSDAI. Scand J Rheumatol. 49(1):38-46, 2020
Luppi F et al: Lung complications of Sjogren syndrome. Eur Respir Rev. 29(157), 2020
Chung A et al: Pulmonary and bronchiolar involvement in Sjogren's syndrome. Semin Respir Crit Care Med. 40(2):235-54, 2019
Natalini JG et al: Pulmonary involvement in Sjögren syndrome. Clin Chest Med. 40(3):531-44, 2019
Ohno Y et al: State-of-the-art imaging of the lung for connective tissue disease (CTD). Curr Rheumatol Rep. 17(12):69, 2015
Ruano CA et al: Thoracic manifestations of connective tissue diseases. Curr Probl Diagn Radiol. 44(1):47-59, 2015
Kreider M et al: Pulmonary involvement in Sjögren syndrome. Semin Respir Crit Care Med. 35(2):255-64, 2014
Egashira R et al: CT findings of thoracic manifestations of primary Sjögren syndrome: radiologic-pathologic correlation. Radiographics. 33(7):1933-49, 2013
Stojan G et al: Pulmonary manifestations of Sjögren's syndrome. Curr Allergy Asthma Rep. 13(4):354-60, 2013
Tsai Y et al: Thymoma associated with myasthenia gravis and Sjögren syndrome. West Indian Med J. 62(3):264-5, 2013
Borie R et al: Severe chronic bronchiolitis as the presenting feature of primary Sjögren's syndrome. Respir Med. 105(1):130-6, 2011
Shi JH et al: Pulmonary manifestations of Sjögren's syndrome. Respiration. 78(4):377-86, 2009
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