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Pulmonary Involvement in Sjögren Syndrome.
Jake G Natalini1, Chadwick Johr2, Maryl Kreider1
1Division of Pulmonary, Allergy and Critical Care, Perelman School of Medicine, University of Pennsylvania, 3400 Spruce Street, 836 W. Gates Building, Philadelphia, PA 19104, USA.
Sjögren syndrome (SS) can affect the lungs, leading to conditions like airway and interstitial lung diseases. Current treatment guidelines for SS-related lung issues are limited, highlighting a need for further research and evidence-based recommendations.
Area of Science:
- Rheumatology
- Pulmonology
- Autoimmune Diseases
Background:
- Sjögren syndrome (SS) is a chronic autoimmune disorder primarily affecting exocrine glands, leading to dryness of eyes and mouth.
- Chronic lymphocytic infiltration of lacrimal and salivary glands is characteristic of SS.
- Extraglandular manifestations are common in SS, with significant pulmonary involvement reported.
Purpose of the Study:
- To review the prevalence and types of lung involvement in patients with Sjögren syndrome.
- To highlight the common pulmonary complications associated with SS.
- To identify the lack of evidence-based guidelines for managing SS-related lung disease.
Main Methods:
- Literature review of studies defining and reporting lung involvement in Sjögren syndrome.
- Analysis of prevalence estimates based on respiratory symptoms and diagnostic tests (radiography, pulmonary function tests).
- Identification of common pulmonary diseases associated with SS, such as airway and interstitial lung diseases.
Main Results:
- Lung involvement in Sjögren syndrome is estimated to occur in 9% to 22% of cases when defined by specific clinical and diagnostic criteria.
- The most frequent pulmonary manifestations include airways disease and interstitial lung disease.
- There is a notable absence of evidence-based guidelines to direct the treatment of lung complications in SS.
Conclusions:
- Pulmonary involvement is a significant extraglandular manifestation of Sjögren syndrome.
- Airways disease and interstitial lung disease are the predominant lung conditions observed in SS patients.
- The lack of established treatment guidelines necessitates further research to improve patient care for SS-related lung disease.
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