Sjogren's Lung Study
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- STATUS
- Recruiting
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- End date
- Apr 1, 2030
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- participants needed
- 500
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- sponsor
- Stanford University
Summary
Lung involvement in Sjgren's syndrome is common and causes reduced quality of life and increased mortality. Sjgren's syndrome-related lung diseases (SS-RLD) are classified and treated as the primary lung diseases they resemble. Whether this approach is optimal has not been evaluated thoroughly. A critical gap in knowledge is knowing whether SS-RLDs have a unique clinical course and response to therapy. Given the underlying immune system dysfunction in Sjgren's syndrome, we hypothesize that patients with SS-RLD will be more likely to respond to immunosuppressive therapy than patients with the matching primary lung disease. To address this hypothesis, we will prospectively screen for Sjogren's syndrome in patients presenting to pulmonary clinics and compare the clinical course and response to therapy in Sjogren's syndrome positive and negative patients.
Description
Sjgren's syndrome is an autoimmune disease affecting at least 1% of adults characterized by hyperactive lymphocytes that damage exocrine glands leading to dry eyes and dry mouth. Although less well recognized, lung involvement in Sjgren's syndrome is common and causes reduced health-related quality of life and increased mortality. Sjgren's syndrome-related lung diseases are classified and treated as the primary lung diseases they resemble. Whether this approach is optimal has not been evaluated thoroughly.
Despite the potentially life-threatening consequence of Sjgren's syndrome-related lung disease, general medical education still promotes the false idea that Sjgren's syndrome is a nuisance disease. This leads many clinicians to overlook Sjgren's syndrome as a possible cause for respiratory symptoms. Even when Sjgren's syndrome is identified, there is no standard for attribution of the lung disease and little data on how to best treat it.
Only one study has compared interstitial lung disease patients with and without Sjgren's syndrome. Although it was a small retrospective study, it found that patients with usual interstitial pneumonia and Sjgren's syndrome were more likely to achieve stable lung function with immunosuppressive therapy as compared to the idiopathic cohort.1 This is striking as usual interstitial pneumonia is generally thought to not be responsive to immunosuppressive therapy.
A critical gap in knowledge is knowing whether Sjgren's syndrome-related lung diseases have a unique clinical course and response to therapy. Given the underlying immune system dysfunction in Sjgren's syndrome, we hypothesize that patients with Sjgren's syndrome-related lung disease will be more likely to respond to immunosuppressive therapy than patients with the matching primary lung disease.
Details
| Condition | Pulmonary Disease, Lung Disease, Bronchiectasis, Bronchiectasis, Sicca Syndrome, Sjogren's Syndrome, Rheumatoid Arthritis, Rheumatoid Arthritis, Rheumatoid Arthritis (Pediatric), Pulmonary Fibrosis, Pulmonary Fibrosis, Xerostomia, Dry Mouth, Interstitial lung disease, Rheumatoid Arthritis (Pediatric), Organizing Pneumonia, Sjogren's Syndrome, Dry Mouth, Lung Disease, interstitial lung diseases, pulmonary diseases, lung diseases, pulmonary disorders, Primary Pulmonary Lymphoma (Disorder), Chronic Bronchiolitis, Primary Pulmonary Lymphoma (Disorder) |
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| Clinical Study Identifier | NCT04843345 |
| Sponsor | Stanford University |
| Last Modified on | 21 April 2021 |
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