Pulmonary manifestations of primary Sjogren's syndrome: a clinical, radiologic, and pathologic study.
BACKGROUND
Clinicopathologic pulmonary manifestations associated with primary Sjogren's syndrome have yet to be reviewed in a large series since the recognition of nonspecific interstitial pneumonia (NSIP) as a distinct histologic pattern.


OBJECTIVE
To determine clinical presentations, high-resolution computed tomographic (HRCT) and histologic findings of the lung disease associated with primary Sjogren's syndrome in the light of NSIP, and to analyze prognosis of the disease.


METHODS
On the basis of 33 cases (31 surgical lung biopsies and 2 autopsies) collected consecutively from multiple centers, we have retrospectively evaluated clinical, radiologic, and pathologic manifestations of the disease. Prognostic factors were identified by univariate and multivariate analysis.


RESULTS
We found that NSIP was the most frequently seen histologic pattern (20 of 33 cases [61%], 19 fibrosing and 1 cellular). Bronchiolar diseases and amyloid and malignant lymphoma were seen less frequently. HRCT-pathologic correlation resulted in a 94% positive predictive value of CT-NSIP pattern for pathologic diagnosis of NSIP, whereas the diagnostic value of HRCT was low (15%) with an HRCT pattern other than NSIP, data that may influence the decision to biopsy. The 5-year survival rate was 84% overall and 83% in patients with NSIP. Multivariate analysis on all patients showed that low Pa(O(2)) (p = 0.02) and presence of microscopic honeycombing (p = 0.04) were independently associated with survival. Patients with NSIP showed lower vital capacity (mean +/- SD: 68.5 +/- 16.6%pred) than patients without NSIP (92.5 +/- 18.6%pred; p < 0.001).


CONCLUSIONS
Among a diversity of pulmonary lesions in primary Sjogren's syndrome, NSIP was the commonest histologic pattern and had a favorable prognosis.
Ito
Isao
I
Department of Respiratory Medicine, Kyoto University Hospital, 54 Shogoin-kawaharacho, Sakyo, Kyoto, Japan.
Nagai
Sonoko
S
Kitaichi
Masanori
M
Nicholson
Andrew G
AG
Johkoh
Takeshi
T
Noma
Satoshi
S
Kim
Dong Soon
DS
Handa
Tomohiro
T
Izumi
Takateru
T
Mishima
Michiaki
M
D001706:Biopsy; D005260:Female; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging* / Q000473:pathology; D017563:Lung Diseases, Interstitial / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011237:Predictive Value of Tests; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D012859:Sjogren's Syndrome / Q000150:complications*; D016019:Survival Analysis; D015996:Survival Rate; D013997:Time Factors; D014057:Tomography, X-Ray Computed