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.
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Lower facial appearance of a 14-year-old adolescent girl with Sjogren syndrome. She exhibits both parotid and submandibular gland enlargement and chapped lips.
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Intraoral view of a 14-year-old adolescent girl with Sjogren syndrome. Hyposalivation results in erythema of the mucosa, gingivitis, decalcification or white spot lesions of the teeth at the cervical margin, and dental caries with extensive restorations of the posterior teeth.
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Erythema of the labial mucosa with enlargement of the minor salivary glands and superficial mucoceles.
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The dorsal surface of the tongue demonstrates generalized atrophy of the filiform papillae, mild fissuring, and median rhomboid glossitis.
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A 14-year-old adolescent girl with Sjogren syndrome with painful unilateral swelling of the knee and hyperpigmentation of the overlying skin.
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The dorsal tongue demonstrates hyperplastic candidiasis with focal erosions and a brown hairy tongue. Ulcerated fissures are observed on the corners of the mouth that represent angular cheilitis.
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Biopsy of the minor salivary glands of the lower lip may be useful in the diagnosis of Sjögren syndrome. A 1.5- to 2-cm incision of normal-appearing mucosa allows for the harvesting of 5 or more salivary gland lobules.
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Low-power photomicrograph of a minor salivary gland lobule showing multiple lymphocytic foci that are replacing the acinar structures (hematoxylin-eosin, 40 X).
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Intermediate-power photomicrograph demonstrating a chronic inflammatory aggregate of more than 50 lymphocytes and plasma cells with a periductal pattern. The inflammatory focus is adjacent to normal appearing acini (hematoxylin-eosin, 200 X).
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High-power photomicrograph of the chronic inflammatory aggregate consists of lymphocytes and plasma cells around a ductal structure (hematoxylin-eosin, 400 X).
