Pediatric Sjogren Syndrome Guidelines

Updated: Sep 09, 2021
  • Author: Marietta Morales De Guzman, MD; Chief Editor: Lawrence K Jung, MD  more...
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Guidelines

Guidelines Summary

Pulmonary disease in Sjögren syndrome

Guidelines on the evaluation and management of pulmonary disease in Sjögren syndrome were published in 2021 by the Sjögren's Foundation. [36] These are some of the highlights of the guidelines.

Assessing and managing upper and lower airway disease in Sjögren syndrome

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

Encourage all patients with Sjögren syndrome to stop smoking.

Bronchoscopic biopsy is not recommended in patients with Sjögren syndrome who have symptomatic small airway disease.

Perform complete pulmonary function testing in patients with Sjögren syndrome who have symptomatic small airway disease.

Treatment for patients with Sjögren syndrome who have 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 syndrome

High-resolution CT with expiratory views and oximetry testing are recommended in patients with Sjögren syndrome who have suspected ILD.

All patients with Sjögren syndrome must be immunized against influenza and pneumococcal infection.

Long-term oxygen therapy is recommended for patients with Sjögren syndrome who have suspected ILD and clinically significant hypoxemia.

Consider mycophenolate mofetil or azathioprine in patients with Sjögren syndrome who have 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.