title: Interstitial lung disease in primary Sjögren's syndrome. Clinical-pathological evaluation and response to treatment.
abstract: Interstitial disease is a recognized form of lung involvement in primary Sjögren's syndrome in which frequency and severity have not yet been established. We studied 20 patients 61.2 +/- 36.4 mo after the first symptoms of the syndrome that fulfilled criteria for both xerostomia and xerophtalmia. Eighteen patients exhibited pulmonary symptoms; nine presented a restrictive functional pattern. The chest roentgenogram disclosed interstitial involvement in nine patients. Gallium-67 lung scan presented hypercaptation in 15 of 19 patients studied. Thirteen patients who submitted to bronchoalveolar lavage presented higher cell counts with increases of lymphocytes and/or polymorphonuclear cells. All patients had abnormal results in at least one of the above. Lung biopsy, undertaken in 12 patients, showed a whole spectrum of interstitial disease, from a follicular bronchiolitis to a lymphoid interstitial pneumonia and finally fibrosis with honeycombing. One patient presented with associated sarcoid granulomas. We treated 11 patients with an azathioprine-based regimen and found a significant improvement in the forced vital capacity (p < 0.05) after at least 6 mo when compared with nontreated patients. We conclude that the frequency of interstitial lung disease is high in Sjögren's syndrome and that an azathioprine-based treatment can favorably change the outcome in this population.
authors.affiliation: Pulmonary Division, Hospital das Clínicas, University of São Paulo, Brazil.
authors.forename: D
authors.initials: D
authors.lastname: Deheinzelin
authors.forename: V L
authors.initials: VL
authors.lastname: Capelozzi
authors.forename: R A
authors.initials: RA
authors.lastname: Kairalla
authors.forename: J V
authors.initials: JV
authors.lastname: Barbas Filho
authors.forename: P H
authors.initials: PH
authors.lastname: Saldiva
authors.forename: C R
authors.initials: CR
authors.lastname: de Carvalho
mesh_terms: D000328:Adult; D000368:Aged; D001379:Azathioprine / Q000627:therapeutic use*; D001992:Bronchoalveolar Lavage Fluid / Q000276:immunology; D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D012129:Respiratory Function Tests; D012859:Sjogren's Syndrome / Q000150:complications* / Q000473:pathology / Q000503:physiopathology
chemical_list: D001379:Azathioprine