Thoracic Cancer ISSN 1759-7706
ORIGINAL ARTICLE
The Prevalence and clinical characteristics of primary Sjogren’s syndrome patients with lung cancer: An analysis of ten cases in China and literature review Yan Xu1*, Yunyun Fei2*, Wei Zhong1, Li Zhang1, Jing Zhao1, Longyun Li1 & Mengzhao Wang1 1 Department of Respiratory Disease, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China 2 Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China
Keywords Diffuse parenchymal lung disease; lung cancer; Primary Sjogren’s syndrome. Correspondence Wang Mengzhao, Department of Respiratory Disease, Peking Union Medical College Hospital, No.1 Shuaifuyuan Road, Dongcheng District, 100730, Beijing, China. Tel: +86 10 69158950 Fax: +86 10 65231169 Email:
[email protected] *Both authors contributed equally to this work. Received: 31 August 2014; Accepted: 5 November 2014. doi: 10.1111/1759-7714.12216 Thoracic Cancer 6 (2015) 475–479
Abstract Background: A retrospective analysis of clinical data from primary Sjogren’s syndrome (pSS) patients with lung cancer was conducted in order to guide further clinical work. Methods: From January 1993 to December 2010, 10 pSS patients with lung cancer were hospitalized at the Peking Union Medical College Hospital. One hundred and three pSS patients were recruited as the controls. Patients’ clinical data was retrospectively analyzed. Result: The incidence rate of lung cancer in pSS patients was 0.477%. The duration from diagnosis of pSS to lung cancer in the 10 patients was 0.92∼42 years. The pSS patients with lung cancer were significantly older than those without lung cancer (60.8 ± 8.9 vs. 44.9 ± 12.1, P < 0.001). Eight of the pSS patients with lung cancer (80%) were female nonsmokers; five pSS patients with lung cancer (50%) had lung involvement, exhibiting diffuse parenchymal lung disease. Adenocarcinoma was the most common pathological type of lung cancer (90%). Tumor node metastasis staging of lung cancer included: four cases of Ib (40%), one case of IIIb (10%), four cases of IV (40%) and one case undetermined (10%). Conclusion: The incidence rate of lung cancer in pSS patients was higher with respect to the normal population. Physicians should closely observe lung cancer incidence during the pSS follow-up period. Adenocarcinoma was the most frequently seen lung cancer pathology in pSS patients. Clinical stages and individualized treatment for pSS patients with lung cancer should be carefully determined.
Introduction The incidence rate of malignancy is high for patients with systematic connective tissue disease (CTD). Lung cancer is one of common malignancies.1 High incidence rates of lung cancer have been reported in patients with scleroderma, rheumatoid arthritis, and dermatomyositis.2 CTD is one of the independent risk factors of lung cancer. The mechanisms involved in the development of cancer in CTD patients are still poorly understood. There are several possible pathogenic mechanisms, including: abnormal autoimmunity of CTD patients, long-term oral immunosuppressant treatment resulting in a secondary onset of immunosuppression, and CTD lung involvement resulting
in interstitial lung disease. Sjogren’s syndrome is a chronic, slowly progressive autoimmune disease, characterized by lymphocytic infiltration of the exocrine glands resulting in xerostomia and dry eyes. Approximately one-third of patients present with systemic manifestations. The incidence rate of malignant tumor in primary Sjogren’s syndrome (pSS) patients is higher than in the normal population; the incidence rate of lymphoma is 15.57∼37.46 times higher than in the normal population. Solid malignant tumors, such as cervical and colon cancers, have been reported in the literature;3,4 however, there are few reports on pSS patients with lung cancer. pSS patients with lung cancer are rare, with a lower incidence rate than other CTDs1,2 of 0.15%5∼1.79%.4
Thoracic Cancer 6 (2015) 475–479 © 2015 The Authors. Thoracic Cancer published by Tianjin Lung Cancer Institute and Wiley Publishing Asia Pty Ltd 475 This is an open access article under the terms of the Creative Commons Attribution-NonCommercial License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.
Sjogren’s syndrome and lung cancer
Y. Xu et al.
Table 1 Clinical characteristics of pSS with lung cancer and pSS patient groups Variable
pSS with lung cancer group
pSS group
P † value
Female: male Age (year) Organ involvement Xerostomia Keratoconjunctivitis sicca Hematologic involvement Liver function abnormality Diffuse parenchymal lung disease
8:2 60.8 ± 8.9
20:1 44.9 ± 12.1
0.226
