Traditional Chinese medicine (TCM) has been applied by a number of practitioners in China. There is substantial evidence that TCM improved symptoms and even laboratory parameters of SS patients. It was shown by Guo et al. that herbs such as Pueraria lobota, Tripterygium wilfordii Hook. f. (TWH), Dendrobium, Fructus lycii, and

Adenophora are efÞcacious in improving dryness of mouth and eyes [9]. TWH has been used for many years to treat autoimmune diseases as anti-inßammatory and immunosuppressive agent. A number of studies have suggested that TCM might regulate immune responses by raising CD4(+)CD25(+) T cells and by promoting the Foxp3 mRNA expression [10]. Increased evidence over the last three decades indicates that TCM can be used clinically based on the symptoms and severity of the diseases [11]. Collectively, TCM is potentially therapeutic in SjšgrenÕs syndrome and more studies should be carried out to evaluate its clinical value and underlying mechanism.

References

1.Bowman SJ, Ibrahim GH, Holmes G, et al. Estimating the prevalence among Caucasian women

of primary Sjšgren syndrome in two general practices in Birmingham, UK. Scand J Rheumatol. 2004;33:39Ð43.

2.Zhang NZ, Shi QS, Yao QP, et al. Epidemiological studies on primary Sjšgren syndrome. Chin J Intern Med. 1993;32(8):522Ð3.

3.He J, Liu X, Jia Y. Survey of clinical patterns of Sjšgren syndrome in China. Chin J Misdiagnos. 2009;9(20):4799Ð4780.

4.Huang JM, Zhang XW, Jia Y, Mu R, Li ZG. Thyroid disease in primary Sjšgren syndrome. Chin J Rheumatol. 2002;6(6):4780Ð99.

5.An Y, Zhang XW, Li ZG. Clinical features of primary Sjšgren syndrome in youth. J Peking Univ. 2009;41(3):324Ð7.

6.Jia Y, Liu X, Liu CH, Li ZG. The clinical and laboratory features of patients with Sjšgren syndrome associated with systemic lupus erythematosus. Chin J Rheumatol. 2006;10(1):23Ð6.

7.Xu D, Tian X, Zhang W, et al. Sjšgren syndromeonset lupus patients have distinctive clinical manifestations and benign prognosis: a caseÐcontrol study. Lupus 2009;18:1Ð4.

8.Fox RI. Sjšgren syndrome. Lancet 2005;366:321Ð31.

9.Guo FQ. The progress of Chinese medicine treatment in Sjšgren syndrome. J Modem Stomatol. 2003;17(4):359Ð61.

10.Li S, Xu KL, Li ZY. Effects of Triptergium hypoglaucun Hutch extraction on regulatory T cells in mice after allogeneic bone marrow transplantation. Zhongguo Zhong Xi Yi Jie He Za Zhi. 2009;29(10):910Ð14.

11.Liu F, Liu H, Wang CL. Sjšgren syndrome. J Practical Traditional Chin Int Med. 2009;23(4):18Ð9.

SjšgrenÕs syndrome (SS) is probably one of the commonest systemic autoimmune rheumatic disorders seen in Chinese. However, it has attracted disproportionately less academic interest compared with conditions such as systemic lupus erythematosus (SLE). Most of the studies on SS carried out in Chinese have been observational. The prevalence of primary SS (pSS) has been estimated to be between 0.45 and 0.77% in Chinese [1, 2], with the peak age of onset

C.-s. Lau ( )

Division of Rheumatology and Clinical Immunology, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong, China

e-mail: cslau@hku.hk

between 40 and 50 years of age. As with patients of other ethnic origins, there is a female predominance with a female to male patient ratio of 9:1. A large number of patients suffering from rheumatoid arthritis, SLE, and other connective tissue disorders may also be complicated by secondary SS. The frequency of secondary SS has not been reported previously but an early study showed that extra-articular manifestations, including sicca symptoms, are uncommon in southern Chinese with rheumatoid arthritis [3].

The spectrum of clinical manifestations of pSS in Chinese is probably similar to that reported in other ethnic groups. For example, the 2002 International ClassiÞcation Criteria for pSS [4] have been shown to be sensitive and speciÞc for

the diagnosis of this condition in Chinese in routine clinical practice [5]. Serologically, 70 and 40% of patients are positive for anti-SSA and anti-SSB antibodies, respectively. Rheumatoid factor (RF) is reported to be positive in 70Ð 90% of patients and cryoglobulin is said to be positive in 25% of patients. Patients who are anti-nuclear antibodies (ANAs) and/or RF positive appear to be younger, have more severe keratoconjunctivitis complex, and other organ involvement [6].

No previous studies have been carried out comparing the clinical manifestations, treatment responses, and outcome of primary SS in Chinese and patients of other ethnic origins. Clinical observation and previous descriptive studies tend to suggest that Chinese patients with pSS probably develop more severe organ disease. For example, in a retrospective study that involved 522 patients with pSS, 221 (42.3%) were reported to have developed some form of pulmonary complications after a median follow up of 48 months from disease onset. Of these patients, 23.3% had interstitial lung involvement, 12.5% pulmonary hypertension, 9.2% pulmonary bullae, 6.0% pleural effusion, and 5.6% pulmonary nodules. Patients with pulmonary disease were older, had more severe sicca symptoms, RaynaudÕs phenomenon and articular complaints, and higher levels of serum γ(gamma)-globulin. The mortality rate was 5.5 times higher in patients with pulmonary complications than those without lung disease [7]. A recent computed tomographic and histopathologic study of patients with interstitial lung disease showed features of non-speciÞc interstitial pneumonia with organizing pneumonia, non-caseating granulomas, chronic bronchiolitis, and lymphocytic interstitial pneumonia [8]. It therefore appears that regular monitoring with careful clinical assessment, pulmonary function tests, and radiological examination are warranted when following up on Chinese patients with pSS.

Cardiac disease is also thought to be common in Chinese patients with pSS, though many patients are asymptomatic [9]. In the retrospective study by Ye et al. [9], of the 124 patients examined, pericardial effusion, left ventricular diastolic dysfunction, and pulmonary arterial

hypertension were found in 20.2, 13.7, and 12.9% of patients, respectively.

Besides pulmonary and cardiac involvement, neurological manifestations have also been suggested to be commonly seen in Chinese patients with pSS. Peripheral neuropathy is commonly reported and up to 5% of patients may present with major central nervous disease including tonicÐclonic seizures, psychosis, and organic brain syndromes. Cerebrospinal ßuid examination may reveal increased white cell count and protein levels, while CT scan of the brain may show plague-like lesions. Similarly, renal diseases, including renal tubular acidosis, interstitial nephritis, and glomerulonephritis, are said to be commonly seen in Chinese SS patients. However, it is not sure whether the patients with glomerulonephritis may indeed have SLE and secondary SS rather than pSS.

Of the various organs involved in pSS, Chinese or oriental pSS patients may present with a characteristic variant of gastrointestinal diseaseÑprotein-losing enteropathy [10, 11]. These patients often present with periorbital and peripheral edema and hypoalbuminemia without marked features of keratoconjunctivitis complex. Serologically, there is hypergammaglobulinemia and positive RF, ANA, and anti-SSA and anti-SSB antibodies. Excessive intestinal protein loss may be conÞrmed by albumin-labeled scan or stool α(alpha)-1 anti-trypsin clearance measurement. Treatment with systemic corticosteroid often results in an improvement and the prognosis is generally good with maintenance immunosuppressive therapy such as azathioprine.

As with all patients with pSS, xerostomia presents a major challenge in the management of this condition in Chinese. Few studies have evaluated the extent of xerostomia and its impact on patientsÕ health-related quality of life (HRQOL) and oral health in Chinese because of a lack of appropriate and culturally validated measurement tools. Recently, however, questionnaires that measure general health-related QOL [12, 13] and oral health-related QOL in southern Chinese have been validated [14]. Using a Chinese version of the SF-36 questionnaire [12], both pSS and secondary SS patients from Hong

Kong were found to have poorer QOL in the physical functioning, role physical, and general health domains [15]. Interestingly, Þbromyalgia, which is commonly described as a co-morbidity in pSS patients in the West, is uncommonly seen in Chinese.

Salivary ßow and saliva pH and buffering capacity were found to be reduced in patients with SS. In addition, xerostomia had a negative impact on oral health-related QOL, which correlated with the number of missing, Þlled, and decayed teeth [16]. Dental caries is a major problem in patients with SS in China, probably because of the lack of a comprehensive national dental service and habitual smoking among patients. Anecdotal evidence suggests that half of all these patients have severe dental caries, with many of them losing all of their teeth. Interestingly, patients with secondary SS had poorer dry mouth measures than pSS. Treatment with muscarinic receptor agonist was well tolerated with improvement in oral health-related QOL but not salivary ßow [17].

References

1.Zeng QY, Chen R, Darmawan J, et al. Rheumatic diseases in China. Arthritis Res Ther. 2008;10:R17. Epub 2008 Jan 31.

2.Xiang YJ, Dai SM. Prevalence of rheumatic diseases and disability in China. Rheumatol Int. 2009;29(5):481Ð90.

3.Cohen MG, Li EK, Ng PY, Chan KL. Extraarticular manifestations are uncommon in southern Chinese with rheumatoid arthritis. Brit J Rheumatol. 1993;32:209Ð11.

4.Vitali C, Bombardieri S, Jonsson R, et al. ClassiÞcation criteria for SjšgrenÕs syndrome: a revised version of the European criteria proposed by the AmericanÐEuropean Consensus Group. Ann Rheum Dis. 2002;61:554Ð8.

5.Zhao Y, Kang J, Zheng WJ, et al. Evaluation of International ClassiÞcation Criteria (2002) for primary SjšgrenÕs syndrome in Chinese patients. Chin Med Sci J. 2005;20:190Ð3.

6.AP Huo, KC Lin, CT Chou. Predictive and prog-

nostic value of antibodies and rheumatoid factor in primary SjšgrenÕs syndrome. Int J Rheum Dis. 2010;13(1):39Ð47.

7.Yan SM, Zhao Y, Zeng XF, Zhang FC, Dong Y. Lung involvement of primary SjšgrenÕs syndrome. Zhonghua Jie He He Hu Xi Za Zhi. 2008;31:513Ð6.

8.Shi JH, Liu HR, Xu WB, et al. Pulmonary manifestations of SjšgrenÕs syndrome. Respiration 2009;78:377Ð86.

9.Ye YC, Zeng Y, Zhu WL, et al. Cardiac manifestations in SjšgrenÕs syndrome. Zhonghua Xin Xue Buan Bing Za Zhi. 2008;36:327Ð31.

10.Mok MY, Lau CS. Protein losing enteropathy and primary SjšgrenÕs syndrome. Clin Exp Rheumatol. 1997;15:705.

11.Nagashima T, Hoshino M, Shimoji S, et al. Proteinlosing gastroenteropahy associated with primary SjšgrenÕs syndrome: a characteristic oriental variant. Rheumatol Int. 2009;29:817Ð20.

12.Lam CLK, Gandek B, Ren SZ, Chan MS. Tests of scaling assumptions and construct validity of the Chinese (HK) version of the SF-36 health survey. J Clin Epidemiol. 1998;51:1139Ð47.

13.Lam CLK, Launder IJ, Lam TP, Gandek B. Population based norming of the Chinese (HK) version of the SF-36 health survey. HK Pract. 1999;21:460Ð70.

14.Wong MCM, Lo ECM, McMillan AS. Validation of a Chinese version of the oral health impact proÞle (OHIP). Comm Dent Oral Epidemiol. 2002;30: 423Ð30.

15.McMillan AS, Leung KCM, Leung WK, Wong MCM, Lau CS, Mok TMY. Impact of SjšgrenÕs syndrome on oral health related quality of life in southern Chinese. J Oral Rehab. 2004;31:653Ð9.

16.Leung KCM, McMillan AS, Leung WK, Wong MCM, Lau CS, Mok TMY. Oral condition and saliva ßow in southern Chinese with SjšgrenÕs syndrome. Int Dent J. 2004;54:159Ð65.

17.Leung KCM, McMillan AS, Wong MCM, Leung WK, Mok MY, Lau CS. The efÞcacy of cevimeline hydrochloride in the treatment of xerostomia in SjšgrenÕs syndrome in southern Chinese patients: a randomized double-blind, placebo crossover study. Clin Rheumatol. 2008;27:429Ð36.

Primary and secondary SjšgrenÕs syndrome (SS) are common diseases in Israel, but their precise prevalence has not been investigated. The knowledge about the disease and its manifestation is well established by physicians of all specialties. The local SS patientsÕ association is also very active by promoting local patientsÕ conferences and editing a local quarterly newsletter. SS has been investigated in depth by Israeli physicians, thus leading to better understanding of the clinical and pathophysiological aspects of this complex disease. Israeli rheumatologists have been involved in the European study group,

M. Tishler ( )

Department of Medicine ÒBÓ, Asaf Harofe Medical Center, ZeriÞn, Israel 70300

e-mail: tishlerm@netvision.net.il

which has determined the European diagnostic criteria for SS that were used for more than a decade until replaced later on by the AmericanÐ European Consensus Group classiÞcation criteria [1, 2]. A study assessing the clinical and laboratory data of 60 SS patients, done by Tishler et al. revealed that despite the different genetic background the clinical features and immunological speciÞcities were the same as those of many European centers [3].

Some important clinical Þndings have been reported by this group concerning allergy, sleep disorders, and Þbromyalgia in SS patient. We have detected that moderate-to-severe sleep disturbances have been reported by 49 out of 65 SS patient (75%) and that Þbromyalgia was also common in this group (55%) and was associated with the sleep disturbances that were detected [4].