Severe chronic bronchiolitis as the presenting feature of primary Sjögren’s syndrome

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• 作者：Raphael Borie ; Sophie Schneider ; Marie-Pierre Debray ; Homa Adle-Biasssette ; Claire Danel ; Anne Bergeron ; Xavier Mariette ; Michel Aubier ; Thomas Papo ; Bruno Crestani
• 关键词：Obstructive ; Sicca syndrome ; Macrolides ; Erythromycin ; Therapy
• 刊名：Respiratory Medicine
• 出版年：2011
• 出版时间：January 2011
• 年：2011
• 卷：105
• 期：1
• 页码：130-136
• 全文大小：357 K

文摘

Summary

Sjögren’s syndrome is a frequent auto-immune disorder with a pulmonary location in almost 10 % of the patients. Although bronchial involvement is very common, most patients only complain of cough and this involvement rarely results in severe symptoms or chronic respiratory failure are rarely observed.

We describe here 5 patients with severe chronic bronchiolitis revealing primary Sjögren’s syndrome. The lung involvement resulted in chronic bronchorrhea, recurrent sinusitis, diffuse bronchiolar nodules with bronchiectasis on the CT scan, and a severe obstructive airway pattern on lung function tests. Improvement was obtained in 4 patients with combination of inhaled corticosteroids, inhaled long acting beta-agonists, and a low dose of erythromycin.