Sjogren's Lung Study

  • STATUS
    Recruiting
  • End date
    Apr 1, 2030
  • participants needed
    500
  • sponsor
    Stanford University
Updated on 21 April 2021

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)
Clinical Study IdentifierNCT04843345
SponsorStanford University
Last Modified on21 April 2021

Eligibility

Yes No Not Sure

Inclusion Criteria

Group 1: Interstitial Lung Disease and Other Parenchymal Lung Diseases
Known or suspected interstitial lung disease regardless of radiographic pattern
Interstitial lung disease due to alternative autoimmune etiology
Sarcoidosis
Organizing pneumonia
Hypersensitivity pneumonitis absent known or suspected trigger
Primary pulmonary lymphoma
Other idiopathic pulmonary conditions at discretion of study team
Group 2: Refractory Airway Symptoms
Chronic cough despite treatment trials with albuterol, proton-pump inhibitors and anti-histamine and intranasal corticosteroids
Persistent bronchial hyperreactivity (defined as positive response to methacholine challenge on spirometry or subjective worsening after exposure to airway irritants such as tobacco, pollution, etc) or persistent asthma symptoms despite trial of inhaled corticosteroid and long acting bronchodilator
Unexplained persistent bronchial wall thickening on CT imaging
Recurrent or chronic bronchiolitis (including but not limited to chronic bronchiolitis, obliterative bronchiolitis, lymphocytic bronchiolitis, constrictive bronchiolitis associated with bronchiolar destruction, and panbronchiolitis)
Bronchiectasis
Lymphocytic alveolitis on bronchoalveolar lavage absent hypersensitivity pneumonitis with known trigger or HIV
Recurrent bacterial pneumonia (greater than 2 episodes in 1 year, confirmed by focal consolidative opacity on chest imaging and requiring antibiotic therapy)
Group 3: Other
Select patients outside the protocol testing schema who have one of the above
lung diseases and are found to have Sjogren's syndrome through the course of
their normal clinical care will be invited to participate in the data
collection portion of the study for analyzing longitudinal outcomes

Exclusion Criteria

Patients with interstitial lung disease due to a known or suspected trigger such as drug-induced (including but not limited to nitrofurantoin, amiodarone, methotrexate and other chemotherapies), inorganic dust exposure (including but not limited to asbestos, silica, hard metals, coal dust) or organic exposure (including but not limited to birds, hay, mold)
Patients who have taken a muscarinic antagonist or agonists within 7 days of planned testing
Patients who are unable to consent for themselves
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