- Pulmonary Manifestations of Sjögren’s Syndrome
- 作者:施举红|发布时间:2013-06-20|浏览量:728次
abstract
background: primary sjögren’s syndrome (pss) is associat-
ed with various histological patterns of interstitial lung dis-
ease. although chest images and lung function studies
showed that lung involvement predominantly occurs in
small airways, pathological findings were not consistent北京协和医院呼吸内科施举红
with the results of high-resolution ct (hrct) and lung func-
tion tests. objectives: to investigate the pathological char-
acteristics of pss-associated interstitial lung disease (pss-
ild) and their relationship with hrct lung function tests.
methods: fourteen patients diagnosed as pss who under-
went surgical lung biopsy in peking union medical college
hospital from october 2000 to october 2006 were reviewed.
histopathologic findings, radiologic findings and lung func-
tion tests were analyzed. results: the study included 13
women. the median age was 46 years. most patients pre-
sented with dyspnea and cough. ct scans revealed bilateral
ground-glass, consolidative, reticular and nodular opacities
and cyst lesions. the histological patterns included nonspe-
cific interstitial pneumonia (nsip) cellular pattern associated
with organizing pneumonia (op), nsip mixed pattern associ-
ated with op, noncaseating granulomas, chronic bronchiol-
itis, follicular bronchiolitis, constrictive bronchiolitis, lym-
phocytic interstitial pneumonia associated with follicular
bronchiolitis, nsip mixed pattern associated with follicular
bronchiolitis, nsip mixed pattern coexisting with chronic
bronchiolitis, op associated with chronic bronchiolitis, and
noncaseating granulomas coexisting with op. treatment in-
cluded prednisone and cyclophosphamide. during the fol-
low-up period (median 38 months), most patients improved
or remained stable. the patient with constrictive bronchiol-
itis died from progression of primary disease. conclusions:
the histopathologic patterns of pss-ild included lung inter-
stitial involvement and small airway involvement or both.
corticosteroid therapy combined with cyclophosphamide
was administered with a favorable response in the majority
of patients.