Pulmonary Manifestations of Primary Sjögren's Syndrome

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Sjögren's syndrome (SS) is a systemic disease with a predilection for the exocrine glands. It also is considered to be an autoimmune epitheliitis, and, as the respiratory system is lined throughout with epithelial cells, it should not be surprising that patients who have SS may develop pulmonary disease. This article describes these manifestations.

Section snippets

Airway disease

The respiratory system starts at the nose and includes the associated nasal cavities. The nose is lined with hairs that filter the incoming air, trapping unwanted particles, and secretions from the nasal columnar epithelial cells help to moisten and remove these inhaled particles. Reduced secretions from these epithelial cells can result in nasal crusting, epistaxis, and recurrent sinusitis.

Further down the airways, dryness in the trachea can produce a dry irritating nonproductive cough and

Interstitial lung disease

ILD can be a manifestation of pulmonary disease in various connective tissue diseases.48 In some patients, the lung disease may be an initial presentation, occurring before the other more usual clinical complaints are apparent,25, 26 and it has been estimated that 15% to 20% of patients presenting with chronic ILD have an occult connective tissue disease,49 whereas other studies have suggested that approximately 8% of primary SS patients have ILD.50 Patients who have SS may develop various

Lymphoma

Patients who have SS are at risk for developing lymphomas, usually a low-grade, non-Hodgkin's MALT-associated lymphoma.30, 31, 68, 69, 70 The low-grade nature of most of these tumors and the currently available treatment have led some to conclude that the diagnosis of a low-grade MALT lymphoma in a Sjögren's patient has no effect on longevity for that patient. Recent studies have determined that certain characteristics are predictive for developing lymphoma, and these include: a low C4,

Pulmonary Hypertension

Pulmonary hypertension (PAH) is a rare complication of primary SS.79, 80, 81 In a recent review by Launay and colleagues,80 nine new cases were presented, and the 19 fully documented cases appearing in the English language literature were reviewed, although only 8 of these 19 other cases had a right heart catheterization, making a total of 17 patients with confirmed PAH. Exertional dyspnea was the initial symptom in 16 of the 17 patients, with syncope the initial complaint in the remaining

Anatomic Tests

Chest radiographs may be abnormal even in patients who are asymptomatic. In a study comparing patients who had primary and secondary SS, some of whom were without significant pulmonary complaints, Vitali and colleagues34 determined that approximately 27% of patients who had primary disease and 75% of patients who had secondary disease had abnormal chest radiographs. Chest radiographs, however, may not be particularly helpful for detecting subtle abnormalities in patients who have SS.34, 38, 84

Summary

SS is a systemic disease with a predilection for the exocrine glands. It also is considered to be an autoimmune epitheliitis, and as the respiratory system is lined throughout with epithelial cells, it should not be surprising that patients who have SS may develop pulmonary disease. Pulmonary disease may be the initial presentation of SS in some patients,25, 26 and previous studies have suggested that patients presenting with idiopathic diffuse lung disease should be evaluated for an underlying

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