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. 2013 Sep 9;8(9):e73774.
doi: 10.1371/journal.pone.0073774. eCollection 2013.

Prognostic factors in interstitial lung disease associated with primary Sjögren's syndrome: a retrospective analysis of 33 pathologically-proven cases

Yasunori Enomoto  1 , Tamiko Takemura, Eri Hagiwara, Tae Iwasawa, Yuh Fukuda, Noriyo Yanagawa, Fumikazu Sakai, Tomohisa Baba, Shouhei Nagaoka, Takashi Ogura
Affiliations

Prognostic factors in interstitial lung disease associated with primary Sjögren's syndrome: a retrospective analysis of 33 pathologically-proven cases

Yasunori Enomoto et al. PLoS One. .

Abstract

Introduction: Interstitial lung disease associated with primary Sjögren's syndrome (pSS-ILD) shows several patterns such as nonspecific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP). Although UIP is a well-recognized prognostic determinant in idiopathic interstitial pneumonias, whether this is also the case in pSS-ILD is unclear. The objectives of this study were to evaluate the prognostic effect of UIP, and to identify the prognostic factors in pSS-ILD.

Methods: A retrospective review of medical records identified 33 consecutive patients with pathologically-proven pSS-ILD. Each patient was classified into each ILD pattern by multidisciplinary analysis. Baseline clinical-radiologic-pathologic characteristics and survival rates were compared between the ILD patterns. Finally, the prognostic factors in pSS-ILD were assessed by univariate and subsequent multivariate analyses using Cox's proportional hazards regression model.

Results: pSS-ILD patients were diagnosed with NSIP (n = 22) or UIP (n = 11). The median follow-up period was 110 months, and five-year survival rate was 87.3% in the total patient population. The prognosis of the UIP patients was not significantly different from that of the NSIP patients (NSIP to UIP, hazard ratio [HR]: 0.77, 95% confidence interval [CI]: 0.18-3.36, P = 0.73). Multivariate analysis identified PaCO2 (HR: 1.68 per 1 Torr increase, 95% CI: 1.24-2.28, P < 0.01), extent of reticular abnormality on high-resolution CT (HR: 4.17 per 1-grade increment, 95% CI: 1.18-14.73, P = 0.03), and severity of fibroblastic foci (HR: 9.26 per 1-grade increment, 95% CI: 1.74-49.35, P < 0.01) as prognostic factors in pSS-ILD.

Conclusions: UIP in pSS-ILD was not related to poorer prognosis than NSIP. Assessment of detailed clinical-radiologic-pathologic findings is more important than distinguishing UIP to evaluate prognosis in this disease.

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Conflict of interest statement

Competing Interests: The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. An example of high–resolution CT images.
Analysis of each finding was as follows. Honeycombing: negative; Bronchiectasis: positive; Dilatation of pulmonary artery: negative; Extent of ground glass attenuation: grade 1; Extent of consolidation: grade 1; Extent of reticular abnormality: grade 2; Extent of micronodules: grade 2. This case was diagnosed as fibrotic nonspecific interstitial pneumonia pattern. (a) at the aortic arch. (b) at the carina tracheae. (c) at the right pulmonary vein. (d) at the top of the right diaphragm.
Figure 2
Figure 2. Examples of pathological scoring (hematoxylin–eosin, × 100).
Typical images in each grade of fibroblastic foci (straight arrows) [(a): grade 1 and (b): grade 3] and bronchiolar fibrosis [(c): grade 1 and (d): grade 3] were listed.
Figure 3
Figure 3. Kaplan–Meier survival curves for patients with interstitial lung disease associated with primary Sjögren’s syndrome.
(a) Survival curve for the total patient population. Five-year survival rate was 87.3%. (b) Comparison of survival curves between the NSIP (n = 22) and UIP (n = 11) patients. Open circles or open squares show censored cases in NSIP group or UIP group, respectively. The prognosis between the two groups was not significantly different (P = 0.93 in log–rank test). UIP: usual interstitial pneumonia; NSIP: nonspecific interstitial pneumonia.
See this image and copyright information in PMC

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