Severe chronic bronchiolitis as the presenting feature of primary Sjögren's syndrome.
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.
Assistance Publique-Hôpitaux de Paris, Hôpital Bichat, Service de Pneumologie A, Centre de Compétence maladies rares pulmonaires, Paris, France.
Raphael
R
Borie
Sophie
S
Schneider
Marie-Pierre
MP
Debray
Homa
H
Adle-Biasssette
Claire
C
Danel
Anne
A
Bergeron
Xavier
X
Mariette
Michel
M
Aubier
Thomas
T
Papo
Bruno
B
Crestani
D000328:Adult; D000368:Aged; D000402:Airway Obstruction / Q000000981:diagnostic imaging / Q000209:etiology*; D001988:Bronchiolitis / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012859:Sjogren's Syndrome / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology; D014057:Tomography, X-Ray Computed