Clinical analysis of the initial symptoms among 164 synovitis, acne, pustulosis, hyperostosis, osteitis syndrome patients / 中华风湿病学杂志

Objective To explore the initial symptoms of synovitis,acne,pustulosis,hyperostosis,osteitis (SAPHO) syndrome,and to analyze the clinical and laboratory characteristics of these patients.Methods We retrospectively analyzed the initial symptoms of 164 patients diagnosed with SAPHO syndrome presented at Peking Union Medical College Hospital from 2004 to 2015,and their clinical,laboratory,and radiological data were collected.The t test,Mann-Whitney U test and chi-square test were used to compare the clinical differences between the SAPHO patients with different initial symptoms.Results Among the 164 patients recruited,84(51.2％) had skin lesions before osteoarticular symptoms,whereas 29(17.7％) after and 42(25.6％) simultaneously.Nine (5.5％) patients had no skin lesions.The time interval between onset of skin and osteoarticular lesions was less than 2 years in 133 (81.1％) patients,but up to 35 years at most.Interestingly,a significantly higher age at onset was observed in patients with osteoarticular symptoms prior to skin lesions than those after [(41 ±10) years vs (36±11) years,t=-2.174,P=0.032].Moreover,positive HLA-B27 was more frequently detected in patients having osteoarticular symptoms as the initial presentations (10.3％ vs 0,P=0.016).Although treated more aggressively before baseline,patients presented with osteoarticular symptoms prior to skin lesions had significantly higher level of hs-CRP at baseline compared with those after [5.42 (1.88,12.70) mg/L vs 11.60 (3.76,22.08) mg/L,Z=-2.096,P=0.036].Conclusion Skin lesions tend to appear prior to osteoarticular symptoms in most SAPHO syndrome patients.The percentage of patients who developed skin lesions after osteoarticular symptoms increase with age at onset.

Distribution of age at onset and its influence on clinical characteristics of 164 patients with synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome / 中华风湿病学杂志

Objective To investigate the distribution of age at onset and its influence on clinical characteristics in synovitis,acne,pustulosis,hyperostosis,and osteitis (SAPHO) syndrome.Methods We recruited 164 patients with SAPHO syndrome who presented to Peking Union Medical College Hospital from Jan 2004 to Mar 2015.All the patients were assessed for medical history,laboratory tests and imaging presentations.The distribution of age at onset was analyzed using Shapiro-Wilknormality test and Kolmogorov-Smimov test for mixed normal distribution.The influence of age at onset on clinical features was analyzed using Mann-Whitney U test and x2 test.Results A double-peak mixed normal distribution of age at onset of skin lesions was found in female patients with SAPHO syndrome,with means and standard deviations of (30±6) years (early-onset) and (51 ±7) years (late-onset) for each mixed normal distribution.The cut-off point was determined to be 42 years old.Nonetheless,a typical single-peak normal distribution of age at onset of skin lesions was observed in male patients.A significantly higher frequency of thoracic region pain [14/36 (38.9％) vs 6/70 (8.6％),x2=14.28,P＜0.01,spinal lesions revealed by bone scintigraphy [23/35 (65.7％) vs 23/66(34.8％),x2=8.79,P=0.003],and peripheral skeletal lesions revealed by bone scintigraphy [17/35 (48.6％) vs 17/66(25.8％),x2=5.33,P=0.021] were found in late-onset female patients compared with early-onset ones.Moreover,female patients with late onset had significantly higher hs-CRP level [(12±12) mg/L vs (9±11) mg/L;U=911.5,P=-0.042)],pain VAS (4.8±1.8 vs 4.0±2.1;U=948,P=0.036),and BASFI (3.0±2.2 vs 1.8±2.0;U=822.5,P=0.003) at baseline than those with early onset.Conclusion Female patients with SAPHO syndrome have a double-peak distribution of age at onset of skin lesions.Female patients with early and late onset of skin lesions exhibit distinct clinical characteristics.

Cluster analysis of clinical phenotypes and its implication in 164 patients with Synovitis-Acne-Pustulosis-Hyperostosis-Osteitis syndrome / 中华风湿病学杂志

Objective To use cluster analysis to explore the clinical phenotypes of Synovitis-Acne-Pustulosis-Hyperostosis-Osteitis (SAPHO) syndrome.Methods One hundred and sixty-four patients fulfilled the Kahn and Khan's criteria for SAPHO syndrome were recruited in Peking Union Medical College Hospital from 2004 to 2015.For all patients,demographics,clinical,laboratory and imaging data were collected.Cluster analysis was performed using an iterative partitioning K-means method including 11 variables that was most characteristic in patients with SAPHO syndrome.The optimal number of clusters was determined by the elbow method and Silhouettes coefficient in combination with clinical significance.Results An optimal of two phenotypes with distinct clinical features were identified.Cluster 1 was characterized by axial skeletal involvement with older age at onset [(38±11) years] and lower prevalence of severe acne (11.2％);Cluster 2 had no axial involvement with younger age at onset [(33±8) years;U=1 800,P=0.010] and higher prevalence of severe acne (26.8％;x2=4.567,P=0.033).Cluster l patients had been treated more aggressively by baseline compared with Cluster 2 patients;and were more frequently prescribed TNF-α inhibitors (32.8％ vs 2.4％;x2=1 672.5,P＜0.01) and bisphosphonates (39.7％ vs 19.5％;x2=1962,P=0.032).Nonetheless,the disease activity indices were significantly higher at baseline in Cluster 1 than Cluster 2 patients [Bath ankylosing spondylitis disease activity index (BASDAI) (3.5±1.8) vs (2.8±2.0);U=1 800,P=0.010] [Bath ankylosing spondylitis functional Index(BASDFI) (2.4±2.3) vs (1.5±1.7);U=1 791,P=0.009).Moreover,Cluster 1 patients had significantly increased inflammatory markers at baseline compared with Cluster 2 patients [erythrocyte sedi-ment-ation rate(ESR) (34.9±2.9) mm/1 h vs (19.0±14.6) mm/1 h;U=1 204.5,P＜0.01] [high-sensitivity C-reactive protein (hs-CRP) (16±19) mg/L vs (8±11) mg/L;U=1 628,P=0.01].Conclusion Char-acterized by the presence or absence of axial skeletal involvement,two disease subtypes exist in SAPHO syndrome,which exhibit distinct features in age at onset,the prevalence of severe acne,and disease severity.

Clinical Characteristics of 65 Male Patients with Sj(o)gren's Syndrome / 医学研究杂志

Objective To analyze the different clinical features and system lesions of female and male primary Sj(o)gren's syndrome (pSS) patients and to attract more attention to male pSS patients' pathogenic features.Methods Clinical information of pSS patients was gathered and physicochemical tests were conducted.The clinical features of female and male patients were summarized and analyzed.Results 935 pSS cases were collected,among which 65 cases were male.The male-female ratio was 0.7∶ 10.The man's median duration of diagnosis was 10 months,with a median duration of 36 months,compared with women's time (P ＜ 0.05).There were significant statistical differences between both the dry mouth and dry eyes occurrence rate of female and male patients (P ＜ 0.05).In ophthalmic Schirmer tests,440 female patients and 8 male presented abnormal results (P ＜ 0.05).For labial gland biopsy,the positive rate of male patients was much higher than that of the female (P ＜ 0.05).The incidence of lymphadenectasis of male patients was 16.9％,which was higher than that of female patients (5.2％),presenting statistical significance (P ＜ 0.05).As regards pSS system lesions,54.48％ of the female presenting presented system lesions,while this rate for male patients was only 36.92％;the rate of multiple system lesions for female and male patients was 10.8％ and 3％ (P ＜ 0.05).The incidence of lymphadenectasis of male patients was higher than that of female patients (P ＜ 0.05).As regards pSS system lesions,the incidence of multiple system involvement was low(P ＜ 0.05).Conclusion The incidence of male pSS is significantly lower than that of female.Dry mouth dryness is lower than that of female,and there is no difference in digestive,respiratory,urinary and nervous system involvement.

Relationship between disease activity and Th17/regulatory T cells level in peripheral blood of patients with synovitis acne pustulosis hyperostosis osteitis syndrome / 中华风湿病学杂志

Objective To investigate whether the proportions of Th17/Treg balance were impaired in the peripheral blood of patients in different phases of synovitis acne pustulosis hyperostosis osteitis (SAPHO) syndrome.Methods We studied 22 cases diagnosed as SAPHO syndrome and 11 healthy controls.According to the scores of VAS pain,BASDAI and BASFA,the 22 patients were divided into active group and stable group.By means of flow-cytometry,the frequencies of total and different subsets of Th17 and Treg cells in peripheral blood mononuclear cell of different groups of SAPHO syndrome and healthy controls were studied.The values of Th17/Treg balance were analyzed.The relationship was analyzed with Kruskal-Wallis test,Mann-Whitney test and Pearman's test.Results The mean percentage of Th17 cells was markedly higher in the active group [(2.74±0.25)％] than in the stable group [(1.16±0.09)％] (U=0.000,P＜0.01) and healthy controls [(1.13±0.11)％] (U=0.000,P＜0.01).No differences were found among active group [(2.10±0.20)％],stable group [(2.51±0.20)％] and control group [(2.44±0.22)％] (x2=2.16,P=0.339 4).The ratio of Th17 cells to Treg cells was markedly higher in active group [(1.48±0.25)％] than in the other two groups [(0.47±0.03)％] (U=0.000,P＜0.01).We also found the positive correlation of the ratios of Th17/Treg cells with the values of VAS in SAPHO syndrome patients (r=0.752 7,P＜0.01).Conclusion The results demonstrate that the development of SAPHO syndrome is closely related to the imbalance of systemic Th17/Treg cells,Increased ratio of Th17/Treg cells may be the main factor that cause disease recurrence,and then,lead to the manifestations of high levels of inflammation and joint pain.

Association between disease activity and osteocalcin, β-isomerized C-terminal telopeptides in SAPHO syndrome / 中华风湿病学杂志

Objective To investigate the relationship between disease activity and osteocalcin,β-isomerized C-terminal telopeptides (β-CTX) in SAPHO syndrome.Methods We studied 58 cases who were diagnosed as SAPHO syndrome.The following indices used to assess the activity of SAPHO were measured:Bath ankylosing spondylitis Disease activity index (BASDAI),Bath ankylosing spondylitis functional index(BASFI),visual analogue scale (VAS) pain and erythrocyte sedimentation rate (ESR),C-reactive protein (CRP),osteocalcin and β-CTX.The SAPHO patients were divided from two groups according to the disease activity.The relationships were analyzed with t-test and Pearson's test.Results The β-CTX of the active group(0.43±0.20) ng/ml was elevated than the stable group (0.23 ±0.09) ng/mL (t=-4.620,P=0.007).And there was no significant difference in the osteocalcin between the active group (2.7±1.0) μg/L and the stable group(2.4±1.0) μg/L (t=-1.169,P=0.820).There was no significant correlation between the serum osteocalcin and ESR or hypersensitive CRP (hs-CRP) (rESR=0.228,PESR=0.088;rhsCRP=-0.085,PhsCRP=0.528).And β-CTX level was related to the VAS (r=0.496,P＜0.01),BASDAI (r=0.401,P=0.002) and BASFI (r=0.295,P=0.025).While no significant relationship between the osteocalcin and pain index was observed in our study.Conclusion The positive correlation between serum β-CTX and disease activity indicates significant bone absorption during the bone destroy process in SAPHO syndrome.

Effects of Ginkgo biloba extract in improving episodic memory of patients with mild cognitive impairment: a randomized controlled trial / 中西医结合学报

Mild cognitive impairment is a transitional stage between normal aging and dementia. It is important in terms of recognizing memory loss in older people as well as identifying a group of individuals at high risk of developing dementia and who may benefit from preventive strategies. Ginkgo biloba extract has been shown to possess polyvalent properties, such as anti-oxidation, anti-apoptosis and anti-inflammation. Ginkgo biloba extract appears to have a neuroprotective effect against neurodegenerative diseases.

Discussion on modif ied Shenxian decoction treating connective tissue disease (CTD) combined with pulmonary interstitial f ibrosis (PIF) / 中华中医药杂志

Pulmonary interstitial fibrosis (PIF) is a common complication of connective tissue disease (CTD). The patients with PIF usually manifest with intensive dry cough, progressive dyspnea aggravated by activities, and even weight loss and very fatigued. In this paper, we have applied the traditional methods of "invigorating qi for ascending" and "strengthening earth to generate metal" with modif ied Shenxian decoction for treating CTD combined with PIF to support healthy energy, ascend thoracic qi, and ascend both qi of lung and spleen. We have observed Shenxian decoction could enhance body immune system, benefit the repair of damaged lung tissues, improve microcirculations, and treat both branch and root of body, which has been giving patients prominent effects.