Pulmonary disease in Sjögren’s Clinical Practice Guidelines (SF, 2021)

Sjögren’s Foundation

These are some of the highlights of the guidelines without analysis or commentary. For more information, go directly to the guidelines by clicking the link in the reference.

March 02, 2021

Guidelines on the evaluation and management of pulmonary disease in Sjögren's were published in February 2021 by the Sjögren's Foundation (SF) in Chest.[1]

Assessing and Managing Upper and Lower Airway Disease in Sjögren's

Evaluate Sjögren's patients with dry bothersome cough and absence of lower airway or parenchymal lung disease for treatable or preventable etiologies other than xerotrachea.

Encourage all Sjögren's patients to stop smoking.

Bronchoscopic biopsy is not recommended in Sjögren's patients with symptomatic small airway disease.

Perform complete pulmonary function testing in Sjögren's patients with symptomatic small airway disease.

Treatment for Sjögren's patients with clinically relevant bronchiectasis may include mucolytic agents/expectorants, nebulized or hypertonic saline, oscillatory positive expiratory pressure, postural drainage, mechanical high-frequency chest wall oscillation therapies, chronic macrolides (in those without mycobacterium colonization or infection).

Interstitial Lung Disease (ILD) in Sjögren's

High-resolution CT with expiratory views and oximetry testing are recommended in Sjögren's patients with suspected ILD.

All Sjögren's patients must be immunized against influenza and pneumococcal infection. 

Long-term oxygen therapy is recommended for Sjögren's patients with suspected ILD and clinically significant hypoxemia.

Consider mycophenolate mofetil or azathioprine in Sjögren's patients with symptomatic ILD when long-term steroid use is contemplated and steroid-sparing immunosuppressive therapy is required. Please note that there are cautions for each of these medications.

For more information, please go to Sjogren Syndrome.

For more Clinical Practice Guidelines, please go to Guidelines.

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