Higher frequency of brain abnormalities in neuromyelitis optica spectrum disorder patients without primary Sjogren's syndrome

Neuromyelitis optica spectrum disorder often co-exists with primary Sjogreffs syndrome. We compared the clinical features of 16 neuro- myelitis optica spectrum disorder patients with （n = 6） or without primary Sjogreffs syndrome （n = 10）. All patients underwent extensive clinical, laboratory, and MRI evaluations. There were no statistical differences in demographics or first neurological involvement at onset between neuromyelitis optica spectrum disorder patients with and without primary Sjogren＇s syndrome. The laboratory findings of cerebrospinal fluid oligoclonal banding, serum C-reactive protein, antinudear autoantibody, anti-Sjogren＇s-syndrome-related antigen A an- tibodies, anti-Sjogren＇s-syndrome-related antigen B antibodies, and anti-Sm antibodies were significantly higher in patients with primary Sjogren＇s syndrome than those without. Anti-aquaporin 4 antibodies were detectable in 67% （4/6） of patients with primary Sjogren＇s syndrome and in 60% （6/10） of patients without primary Sj6gren＇s syndrome. More brain abnormalities were observed in patients without primary Sj6gren＇s syndrome than in those with primary Sj6gren＇s syndrome. Segments lesions （〉 3 centrum） were noted in 50% （5/10） of patients without primary Sj6gren＇s syndrome and in 67% （4/6） of patients with primary Sjogren＇s syndrome. These findings indicate that the clinical characteristics of neuromyelitis optica spectrum disorder patients with and without primary Sjogren＇s syndrome are similar. However, neu- romyelitis optica spectrum disorder patients without primary Sjogreffs syndrome have a high frequency of brain abnormalities.

Comparison of corneal biomechanics in Sjogren's syndrome and non-Sjogren's syndrome dry eyes by Scheimpflug based device

AIM：To compare the corneal biomechanics of Sj？gren＇s syndrome（SS） and non-SS dry eyes with Corneal Visualization Scheimpflug Technology（CorV is ST）.METHODS：Corneal biomechanics and tear film parameters, namely the Schirmer I test value, tear film break-up time（TBUT） and corneal staining score（CSS） were detected in 34 eyes of 34 dry eye patients with SS（SSDE group） and 34 dry eye subjects without SS（NSSDE group） using CorV is ST. The differences of the above parameters between the two groups were examined, and the relationship between corneal biomechanics and tear film parameters were observed. RESULTS：The differences in age, sex, intraocular pressure（IOP） and central corneal thickness（CCT） were not significant between the two groups（P〉0.05）. The tear film parameters had significant differences between the SSDE group and NSSDE group（all P〈0.05）. Patients in the SSDE group had significantly lower A1-time and HC-time, but higher DA（P=0.01, 0.02, and 0.02, respectively） compared with the NSSDE group. In the SSDE group, DA was negatively correlated with TBUT（rho=-0.38, P=0.03）; HC-time was negatively correlated with CSS（rho=-0.43, P=0.02）. In the NSSDE group, HC-time was again negatively correlated with CSS（rho=-0.39, P=0.02）.CONCLUSION：There are differences in corneal biomechanical properties between SSDE and NSSDE. The cornea of SSDE tends to show less ＂stiffness＂, as seen by a significantly shorter A1-time and HC-time, but larger DA, compared with the cornea of NSSDE. Biomechanical parameters can be influenced by different tear film parameters in both groups.

Sjogren＇s syndrome with extremely elevated plasma alpha-fetoprotein that responded to immunosuppressant treatment

We report a case with extremely elevated tumor marker alpha-fetoprotein （AFP） and jaundice that were caused by primary Sj6gren＇s syndrome but not hepatocellular carcinoma. High plasma AFP alone is not pathognomonic for the diagnosis of hepatocellular carcinoma, and can be caused by autoimmune liver damage.

Clinical Profile and Significance of Mucocutaneous Lesions of Primary Sjogren＇s Syndrome： A Large Cross-sectional Study with 874 Patients

Background： Mucocutaneous lesions are common features of primary Sjogren＇s syndrome （pSS）, but only a few studies have focused on them. To demonstrate the profile ofmucocutaneous lesions ofpSS and further explore their potential clinical significance, we perfermed a cross-sectional study on 874 patients. Methods： Demographic data, clinical manifestations, and laboratory results of 874 pSS patients were collected. Patients were divided into two groups according to the presence of mucocutaneous lesions. Differences in primary symptoms and systemic impairments between the two groups were analyzed. Results of laboratory tests were also compared alter excluding those who had taken corticosteroid from both groups. One-year follow-up was done, and occurrences of various new complications were compared. Results： Among the 874 pSS patients, 181 patients had mucocutaneous lesions, accounting for 20.7%. Multiple mucocutaneous manifestations were displayed, and the top four most common types of lesions were purpuric eruptions （39.8%）, urticaria （23.8%）, Raynaud＇s phenomenon （14.9%）, and angular stomatitis （9.9%）. Incidences of pulmonary interstitial fibrosis, pulmonary bullae, leukopenia, and anemia were significantly higher among patients with mucocutaneous lesions （P 〈 0.05）. Increase in lgG and decrease in C4 among patients with mucocutaneous lesions displayed statistical significance after excluding patients from both groups who had taken corticosteroid （P 〈 0.05）. Alter one-year follow-up, patients with mucocutaneous lesions presented a slightly higher incidence of new complications compared to those without. Conclusions： Mucocutaneous manifestations ofpSS patients were common and diverse. Patients with mucocutaneous manifestations had more systemic damages, higher level of IgG, and lower level of serum C4, suggesting a higher activity of the primary disease.

Effect of Chinese Herbal Medicine for Nourishing Yin,Supplementing Qi,and Activating Blood on the Th1/Th2 Immune Balance in Peripheral Blood in Patients with Primary Sjogren's Syndrome

Objective： To observe the effect of Chinese herbal medicine for nourishing yin, supplementing qi, and activating blood on the expression of interferen- γ （IFN-γ,）/interleukin-4 （IL-4） in peripheral blood and disease activity in primary Sjogren＇s syndrome （pSS） patients, and to study the relationship between the immune balance of Th1/Th2 and the disease activity. Methods： A total of 66 pSS patients were randomized with tossing coins method into two groups： the integrative therapy group （34 cases） and the control group （32 cases）; and 28 healthy subjects were taken as the normal group. The integrative therapy group was treated by Chinese herbal medicines for nourishing yin, supplementing qi, and activating blood combined with hydroxychloroquine sulfate tablets and the control group was treated with hydroxychloroquine sulfate tablets. The treatment course was 3 months for both groups. The levels of serum immunoglobulin G （IgG）, erythrocyte sedimentation rate （ESR）, IFN-γand IL-4 in peripheral blood were measured before and after treatment. Results： Compared with the normal group, the levels of IgG, ESR, IFN- γ and IL-4 were significantly increased in pSS patients （P〈0.05）. Remarkably, after 3 months of treatment, these levels were dramatically decreased in both the integrative therapy group and the control group, although still higher than the normal group. The levels of IgG, ESR, IFN- and IL-4 in the integrative therapy group were lower than the control group and the same group before treatment （P〈0.05）. The ratio of IFN-γ/IL-4 also significantly decreased after treatment. Moreover, the level of IFN- γ, and the ratio of IFN- γ/IL-4 in the integrative theraphy group were significantly lower than the control group （P〈0.05）. For all patients the ratio of IFN-γ/IL-4 before and after treatment was positive correlated with the levels of IgG and ESR. Conclusion： Chinese herbal medicine for nourishing yin, supplementing qi, and activating blood can alleviate the disease activity of pSS by regulating the immune balance of Th1/Th2.

Therapeutic Effect of Chinese Herbal Medicine for Strengthening Qi,Nourishing Yin,and Removing Stasis on Serum Osteopontin and Quality of Life of Patients with Primary Sjogren's Syndrome

Objective：To observe the therapeutic effect of Chinese herbal medicine for strengthening qi, nourishing yin,and removing stasis on serum osteopontin（OPN） and quality of life（QOL） in patients with primary Sjogren＇s syndrome（pSS） and to study the correlation between OPN level and the disease.Methods：Sixtyeight pSS patients were randomly assigned to two groups：the treatment group（35 cases） treated by Chinese herbal medicine for strengthening qi,nourishing yin,and removing stasis combined with hydroxychloroquine sulfate tablet（HCQ） and the control group（33 cases） treated by HCQ only.Both were treated for 3 months. Before and after treatment,immunoglobulin G（IgG） level,erythrocyte sedimentation rate（ESR）,and OPN level were measured.The QOL in patients was evaluated using the Short Form-36 Health Survey（SF-36） before and after treatment.Thirty healthy females were taken as the normal control.Results：Before treatment,levels of IgG,ESR,and OPN in patients were higher than those in the normal control.After 3 months of treatment, those in both treatment groups decreased but were lower in the treatment group than those in the control group （P0.05）.The scores of 8 dimensions of SF-36 were lower in the pSS patients than those in the normal control （P0.05） and higher in the treatment group than in the control group after treatment（P0.05）.Furthermore, there was a positive correlation between the levels of OPN and the levels of IgG and ESR,and a negative correlation between OPN levels and the overall score of SF-36（P0.05）.Conclusions：Chinese herbal medicine for strengthening qi,nourishing yin,and removing stasis could alleviate pSS disease and improve the QOL.In addition,the OPN level might be used as an evaluating index for pSS disease.

Effect of Chinese Herbal Medicines for Nourishing Yin,Supplementing Qi,and Activating Blood on Reproductive Endocrine Activity and Immune Functions in Patients with Primary Sjogren's Syndrome

ABSTRACT Objective： To investigate the effect of Chinese herbal medicines for nourishing yin, supplementing qi, and activating blood on the reproductive endocrine-immune network and its mechanisms in patients with primary Sjogren＇s syndrome （pSS）. Methods： Seventy pSS patients were randomly assigned to two groups using a randomized digital table： the integrative therapy group （36 cases） and the control group （34 cases）. Thirty healthy subjects were taken as a normal group. The control group was treated with hydroxychloroquine sulfate tablets alone, and the integrative therapy group was treated by Chinese herbal medicines for nourishing yin, supplementing qi, and activating blood combined with hydroxychloroquine sulfate tablets. The treatment course was 6 months for both groups. Before and after treatment, serum estradiol （E2）, testosterone （T）, luteinizing hormone （LH）, prolactin （PRL） by radioimmunoassay and immunoglobulin （IgG） by immunodiffusion, erythrocyte sedimentation rate （ESR） by Westergren, interferon-γ （IFN-γ） and interleukin-4 （IL-4） by enzyme linked immunosorbent assay were determined. Results： E2 and T levels in all patients were lower than those of normal subjects before treatment （P〈0.05） and were increased significantly after 6-month treatment （P〈0.05）. ESR, FSH, LH, IgG, IFN - γ, IL - 4 and ratios of E2/T, and IFN --γ/IL in the patients were higher than those of normal subjects before the treatments （P〈0.05）, and were reduced significantly after the treatments （P〈0.05）. The T and IFN -γ levels and E2/T ratio in the patients treated with integrative therapy were reduced significantly compared with the control group （P〈0.05）. However, the PRL levels before and after treatment were not significantly changed in the two groups （P〉0.05）. The ratios of E2/T and IFN -γ/IL-4, and levels of IgG and ESR were positively correlated before and after treatment （P〈0.05）. Conclusions： The ratios of E2/T and IFN - γ/ IL-4 might be used as indicators of pSS activity. Chinese herbal medicines for nourishing yin, supplementing qi, and activating blood combined with Western medicine could improve the therapeutic effect by regulating the reproductive endocrine-immune network in pSS patients.

Expression of Phytoestrogens in pGL2/AQPI Promoter Reporter System

In this study, we amplified aqnaporin I（AQP1） promoter sequence with polymerase chain reaction（PCR）, then AQP1 promoter fragment and pGL2 basic vector were linked to create an artificial pGL2/AQP1 promoter re- porter system. A certain concentration of 17β-estradiol（E2） activated pGL2/AQPlp, which demonstrated the pGL2/AQPlp transcriptional system effective. The pGL2/AQP1 promoter reporter system was applied to evaluate the activate effect on AQP1 of different kinds of phytoestrogens. Dual hiciferase reporter gene activity assay showed that a certain concentration phytoestrogens including daidzein and genistein can increase AQP1 promoter transcription activity. In addition, E2, daidzein and genistein can make AQP1 protein endogenous expression level increase and promote the function of water scretion. The result can guide the clinical application to treat the Sjogren＇s syndrome and provide a necessary molecular tool for the subsequent drug screening.