Elsevier

Mayo Clinic Proceedings

Volume 64, Issue 8, August 1989, Pages 920-931
Mayo Clinic Proceedings

Pulmonary Lymphoma in Sjögren's Syndrome

https://doi.org/10.1016/S0025-6196(12)61219-1Get rights and content

Sjögren's syndrome (SS) is an immunologie disease characterized by progressive destruction of the exocrine glands that causes mucosal and conjunctival dryness. In addition to the common pulmonary complications of diffuse interstitial lung disease, airways obstruction, desiccation of the upper respiratory tract, localized parenchymal nodules, recurrent tracheobronchitis, bronchiectasis, interstitial pneumonitis, and pleural effusion, patients with SS have a high risk of developing non-Hodgkin's lymphoma that may affect the lungs. Among 50 patients with SS and associated lymphoma, 10 had pulmonary involvement by lymphoma. The mean age of these 10 patients was 59.7 years, and 8 were women. The mean duration of SS was 7.2 years, and the mean interval between the onset of SS and lymphoma was 5.4 years. Cough and slowly progressive dyspnea were the most common pulmonary symptoms, and chest roentgenographic findings varied. Lung biopsies revealed a spectrum of low- to high-grade lymphomas, and high-grade lymphomas were associated with increased mortality. Of the 10 patients, 4 died from 8 to 48 months after lymphoma was diagnosed. We conclude that pulmonary involvement is common in patients with lymphoma associated with SS; thus, lymphoma should be considered in the differential diagnosis of pulmonary lesions in patients with SS.

Section snippets

MATERIAL AND METHODS

We reviewed the records of all Mayo Clinic patients with the diagnosis of SS and lymphoma. Only the records of patients with an unequivocal diagnosis of SS (sicca complex only, without evidence of other causes of “sicca-like” symptoms, or sicca complex associated with rheumatoid arthritis or other collagen disease and a clinical course suggestive of SS) and associated lymphoma were selected for study. The diagnosis of SS was based primarily on clinical findings and occasionally on laboratory

RESULTS

Both SS and lymphoma were diagnosed in 50 patients, and pulmonary lymphoma developed in 10 of these patients. Three patients had been included in an earlier Mayo Clinic report on the pulmonary manifestations in SS.13 The clinical features of the 10 patients, 8 of whom were women, are outlined in Table 1. The mean age of these patients was 59.7 years (range, 42 to 74 years). Seven patients had primary SS, and three had secondary SS in association with rheumatoid arthritis. The duration of SS

DISCUSSION

Among the autoimmune collagen disorders, SS is the second most commonly encountered (rheumatoid arthritis is the most frequent).7 The prevalence of SS is estimated to be between 0.5 and 2.0% of the general population; most patients are in the age group of 43 to 54 years at the time of diagnosis, and the female-to-male ratio is 9 to 1.9, 28, 29 Although various clinical features and laboratory tests have been recommended for the diagnosis of SS, no established criteria have been uniformly

CONCLUSION

This study demonstrates that patients with SS may have pulmonary lymphoma. The presence of pulmonary infiltrates in patients with SS should prompt the consideration of involvement of the lungs by lymphoma.

ACKNOWLEDGMENT

We thank Peter M. Banks, M.D., formerly of the Division of Pathology, for the review of pathologic specimens for this study.

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