Features of usual interstitial pneumonia in patients with primary Sjögren׳s syndrome compared with idiopathic pulmonary fibrosis

Yasunori Enomoto; Tamiko Takemura; Eri Hagiwara; Tae Iwasawa; Koji Okudela; Noriyo Yanagawa; Tomohisa Baba; Fumikazu Sakai; Yuh Fukuda; Shouhei Nagaoka; Takashi Ogura

doi:10.1016/j.resinv.2014.02.003

Features of usual interstitial pneumonia in patients with primary Sjögren׳s syndrome compared with idiopathic pulmonary fibrosis

Respir Investig. 2014 Jul;52(4):227-35. doi: 10.1016/j.resinv.2014.02.003. Epub 2014 Apr 13.

Authors

Yasunori Enomoto¹, Tamiko Takemura², Eri Hagiwara³, Tae Iwasawa⁴, Koji Okudela⁵, Noriyo Yanagawa⁶, Tomohisa Baba⁷, Fumikazu Sakai⁸, Yuh Fukuda⁹, Shouhei Nagaoka¹⁰, Takashi Ogura¹¹

Affiliations

¹ Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1, Tomioka-Higashi, Kanazawa-ku, Yokohama 236-0051, Japan. Electronic address: yasuyasuyasu29@yahoo.co.jp.
² Department of Pathology, Japanese Red Cross Medical Center, 4-1-22, Hiroo, Shibuya-ku, Tokyo 150-8935, Japan. Electronic address: byori@med.jrc.or.jp.
³ Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1, Tomioka-Higashi, Kanazawa-ku, Yokohama 236-0051, Japan. Electronic address: hagiwara@kanagawa-junko.jp.
⁴ Department of Radiology, Kanagawa Cardiovascular and Respiratory Center, 6-16-1, Tomioka-Higashi, Kanazawa-ku, Yokohama 236-0051, Japan. Electronic address: tae_i_md@wb3.so-net.ne.jp.
⁵ Department of Pathobiology, Yokohama City University Graduate School of Medicine, 3-9, Fukuura, Kanazawa-ku, Yokohama 236-0004, Japan. Electronic address: krascv@yahoo.co.jp.
⁶ Department of Radiology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, 3-18-22, Honkomagome, Bunkyo-ku, Tokyo 113-8677, Japan. Electronic address: noriyo_yana@ybb.ne.jp.
⁷ Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1, Tomioka-Higashi, Kanazawa-ku, Yokohama 236-0051, Japan. Electronic address: baba@kanagawa-junko.jp.
⁸ Department of Diagnostic Radiology, Saitama International Medical Center, Saitama Medical University, 1298, Yamane, Hidaka, Saitama 350-1298, Japan. Electronic address: fmksakai@yahoo.co.jp.
⁹ Department of Diagnostic Pathology, Itabashi Chuo Medical Center, 2-12-7, Azusawa, Itabashi-ku, Tokyo 174-0051, Japan. Electronic address: fukuda@nms.ac.jp.
¹⁰ Department of Rheumatology, Yokohama Minami Kyosai Hospital, 1-21-1, Mutsuura-Higashi, Kanazawa-ku, Yokohama 236-0037, Japan. Electronic address: nagaokascrt@msn.com.
¹¹ Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1, Tomioka-Higashi, Kanazawa-ku, Yokohama 236-0051, Japan. Electronic address: takashiogurajunko@yahoo.co.jp.

PMID: 24998369
DOI: 10.1016/j.resinv.2014.02.003

Abstract

Background: The different characteristics of usual interstitial pneumonia in patients with primary Sjögren׳s syndrome (UIP/pSS) compared with idiopathic pulmonary fibrosis (UIP/IPF) are not fully understood. This study aimed to compare characteristics, prognosis, and treatment responses in these patients.

Methods: Among 129 consecutive patients who underwent surgical lung biopsy to diagnose diffuse lung diseases at Kanagawa Cardiovascular and Respiratory Center between 1998 and 2002, we identified 10 and 19 patients with UIP/pSS and UIP/IPF, respectively. Baseline characteristics, chest high-resolution computed tomography (HRCT) and pathological findings, and the clinical course were compared between the two groups. Responses to immunosuppressive therapy were analyzed by comparing pulmonary function and clinical status before and one year after treatment initiation.

Results: More patients in the UIP/pSS group tended to be female and older than those in the UIP/IPF group (mean age, 68 years vs. 62 years). In addition, they more commonly exhibited enlarged mediastinal lymph nodes and bronchial wall thickening on HRCT. Pathologically, in the UIP/pSS group, interstitial inflammation, plasma cell infiltration, lymphoid follicles with germinal centers, cysts, bronchiolitis, and pleuritis were significantly more prominent, whereas smooth muscle hyperplasia and fibroblastic foci were milder (all P<0.05). The prognosis was better for UIP/pSS compared with UIP/IPF patients (P=0.01). In addition, immunosuppressive therapy provided better disease control for those with UIP/pSS (83%, 5/6) compared UIP/IPF (7%, 1/15).

Conclusion: This study identified distinct clinical, radiological, and pathological characteristics of UIP/pSS compared with UIP/IPF. Immunosuppressive treatment could be a therapeutic option for UIP/pSS.

Keywords: Idiopathic pulmonary fibrosis; Immunosuppressive treatment; Sjögren׳s syndrome; Usual interstitial pneumonia.

Publication types

Comparative Study
Research Support, Non-U.S. Gov't

MeSH terms

Age Factors
Aged
Female
Humans
Idiopathic Pulmonary Fibrosis / diagnostic imaging
Idiopathic Pulmonary Fibrosis / drug therapy
Idiopathic Pulmonary Fibrosis / pathology*
Immunosuppressive Agents / therapeutic use
Lung Diseases, Interstitial / diagnostic imaging
Lung Diseases, Interstitial / drug therapy
Lung Diseases, Interstitial / etiology*
Lung Diseases, Interstitial / pathology*
Male
Middle Aged
Prognosis
Sex Factors
Sjogren's Syndrome / complications*
Survival Rate
Time Factors
Tomography, X-Ray Computed
Treatment Outcome

Substances

Immunosuppressive Agents