Download European Respiratory Society Annual Congress 2013

European Respiratory Society
Annual Congress 2013
Abstract Number: 1781
Publication Number: P2340
Abstract Group: 1.5. Diffuse Parenchymal Lung Disease
Keyword 1: Interstitial lung disease (connective tissue disease) Keyword 2: Idiopathic pulmonary fibrosis
Keyword 3: No keyword
Title: Prognostic factors of interstitial lung disease associated with primary Sjögren's syndrome
Dr. Yasunori 15465 Enomoto [email protected] MD 1, Dr. Tamiko 15466 Takemura
[email protected] MD 2, Dr. Tae 15467 Iwasawa [email protected] MD 3, Dr. Yuh 15468
Fukuda [email protected] MD 4, Dr. Noriyo 15469 Yanagawa [email protected] MD 5, Dr. Fumikazu
15470 Sakai [email protected] MD 6, Dr. Tomohisa 15471 Baba [email protected] MD 1, Dr.
Eri 15472 Hagiwara [email protected] MD 1 and Dr. Takashi 15495 Ogura
[email protected] MD 1. 1 Department of Respiratory Medicine, Kanagawa Cardiovascular and
Respiratory Center, Yokohama, Japan ; 2 Department of Pathology, Japanese Red Cross Medical Center,
Tokyo, Japan ; 3 Department of Radiology, Kanagawa Cardiovascular and Respiratory Center, Yokohama,
Japan ; 4 Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan ; 5 Department of
Radiology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, Tokyo, Japan
and 6 Department of Diagnostic Radiology, Saitama International Medical Center, Saitama Medical
University, Saitama, Japan .
Body: Objectives: Interstitial lung disease associated with primary Sjögren's syndrome (pSS-ILD) shows a
variety of patterns such as nonspecific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP).
The prognostic factors in pSS-ILD remain unknown. Our objectives were to confirm whether UIP in pSS-ILD
could be associated with poor prognosis as recognized in idiopathic interstitial pneumonias, and to
determine the prognostic factors. Methods: A retrospective review of medical records identified 33
consecutive patients with pSS-ILD pathologically proven by surgical lung biopsy between November 1998
and November 2008. Clinical, radiological, pathological characteristics and survival rate were analyzed, and
compared between patients with NSIP and UIP. The prognostic factors were assessed with Cox
proportional hazard model. Results: NSIP (n=22) was the most predominant ILD pattern, and the others
were all diagnosed as UIP (n=11). The median follow-up period was 110 months. The five-year survival rate
of all patients was 87.3%. The prognosis of patients with UIP was not significantly different from that with
NSIP. Multivariate analysis revealed that PaCO2 (hazard ratio [HR] 1.68 per 1 mm Hg increase, p<0.01), the
extent of reticular abnormality on high-resolution CT (HR 4.17 per 1-grade increment, p=0.03), and the
severity of fibroblastic foci (HR 9.26 per 1-grade increment, p<0.01) were independent and statistically
significant prognostic factors. Conclusions: Survival outcome in UIP was not poorer than in NSIP. Retention
of PaCO2, large extent of reticular abnormality on high-resolution CT, and severe fibroblastic foci were
associated with poor prognosis in pSS-ILD.