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 features of dyslipidemia in patients with primary biliary cholangitis / 中华全科医师杂志

Objective@#To analyze the clinical features of dyslipidemia in patients with primary biliary cholangitis (PBC).@*Methods@#The clinical and laboratory data of 136 PBC patients in Peking Union Medical College Hospital from 2010 to 2016 were retrospectively analyzed.The liver function was compared between patients with normal and abnormal blood lipids.@*Results@#Among 136 PBC patients, 100(74%)had abnormal serum lipids. The incidence of increased cholesterol, low-density lipoprotein and triglyceride was 61%(59/96), 58%(48/83) and 47%(46/97), respectively; while that of reduced HDL-C was 26%(21/82). The incidences of pruritus [26%(26/100) vs. 8%(3/36), χ2=4.93, P=0.032], serum total bilirubin (TBIL) [17.3(12.2, 28.2) μmol/L vs. 14.5 (9.4, 21.1) μmol/L, Z=2.25, P=0.024], direct bilirubin (DBIL)[5. 5 (3.4, 12.4) μmol/L vs. 4.4(2.9, 7.1) μmol/L, Z=2.00, P=0.045], and glutamyl aminotransferase (GGT)[193.0(64.3, 454.8)U/L vs. 105.5(53.5, 179.5)U/L, Z=2.02, P=0.043], alkaline phosphatase(ALP)[183(86, 351)U/L vs. 135(85, 188) U/L, Z=1.98, P=0.048] in PBC patients with dyslipidemia were significantly higher than thosein patients with normal serum lipids.Pearson regression analysis showed that in PBC patients with dyslipidemia, the ALT was positively corrected with TG and TC(r=0.248 and 0.272, P=0.015 and 0.008); ALB was positively correlated with LDL-C(r=0.335, P=0.002); DBIL was positively corrected with HDL-C(r=0.252, P=0.022); TC was positively correlated with ALP and GGT(r=0.313 and 0.346, P=0.002 and 0.001); GGT was positively correlated with LDL-C(r=0.251, P=0.022).@*Conslusion@#Increased TC and LDL-C were more common in PBC patients. PBC patients with dyslipidemia have more severe liver damage than the patients with normal serum lipids.

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.

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.

Analysis of the relationship between health-related quality of life (primary biliary cholangitis-40) and clinical presentations of patients with primary biliary cholangitis / 中华风湿病学杂志

Objective To investigate the health related quality of life score [primary biliary cholangitis (PBC)-40] in patients with PBC,and the relationship between PBC-40 and clinical presentations.Methods The PBC-40 score and clinical presentations in PBC patients (n=65) were adapted in this study.Patients were divided into the untreated group and the treated group,and the treated group was further divided into ursodesoxycholic acid (UDCA) response group and UDCA non-response group.PBC-40 scores of different groups were analyzed by t-test and the relationship between PBC-40 and clinical presentations were analyzed with Pearson's test.Results Dimensions of PBC-40 scores of this group of patients were as follows:symptoms were (15.8±4.1) points,itch was (4.9±2.8) points,atigue was (23.8±8.9) points,cognitive dysfunction score was (11.4±4.7) points,social activity score was (17.0±7.5) points,and the emotion score was (6.5±3.1) points.The untreated group had higher emotion scores than the treated group (t=2.024,P=0.045).Compared with the UDCA response group,UDCA non-response group had higher scores in cognitive,social and emotion dimension (t =2.126,2.309,2.062,respectively,P=0.039,0.025,0.045,respectively).Itch score was significantly correlated with total bilirubin (TBil),direct bilirubin (DBil),alkaline phosphatase (ALP) and total bile acid (TBA) (r=0.349,0.345,0.324,0.427,respectively,P＜0.01),while the social scores were correlated with TBil,DBil,aspartate aminotransferase (AST) and TBA (r=0.361,0.383,0.316,0.331,P＜0.01) and emotion scores were associated with ALT,TBil,GGT,ALP,AST and TBA (r=0.332,0.430,0.265,0.326,0.297,0.353,P＜0.05).ConclusionPBC-40 can be used as a health-related quality of life assessment for PBC patients inChinese population.Itch,social and emotion dimensions are correlated with clinical activity indicators.Hyperbilirubin,ALP and TBA can predict low health quality of life in PBC patients.Conclusion PBC-40 can be used as a health-related quality of life assessment for PBC patients in Chinese population.Itch,social and emotion dimensions are correlated with clinical activity indicators.Hyperbilirubin,ALP and TBA can predict low health related quality of life in PBC patients.

A survey on perception of disaster management among medical students / 基础医学与临床

Objective To investigate current situation of disaster medicine courses among medical schools in China and perception of disaster management among medical students.Methods The survey was conducted among medical students from 14 medical schools in China.A questionnaire which included self-evaluation of ability to practice disaster management, status of disaster medicine courses and self-interest in learning disaster management was used.Results The survey showed that only a few of medical schools offered disaster medicine courses and the quality of current courses was not satisfying.Medical students were generally lack of systematic perception of disaster management, but they were interested in studying related knowledge.Conclusions It is necessary to design disaster medicine courses of high quality and promote them among medical schools in China.It can not only meet the requirements of medical students, but also effectively improve the response capacity of medical students and doctors when facing catastrophes.

Concerns about disease management and psychological stress in SAPHO patients during the COVID-19 epidemic

ObjectivesThe coronavirus disease 2019 (COVID-19) epidemic brings potentially impact on the care of patients with rheumatic diseases, including SAPHO syndrome. We aimed to investigate the disease status, concerns about management, and psychological stress in SAPHO patients during the COVID-19 epidemic. MethodA structured questionnaire was distributed online to patients with SAPHO syndrome enrolled in a Chinese cohort study on March 3rd, 2020. Patients were ask about the current treatments, disease status, and concerns about disease management during the epidemic. Psychologic stress (scored from 0 to 10 points) and psychological problems were reported by the patients. ResultsA total of 157 patients (mean age 38.4 {+/-} 12.3 years, 66.9% females) were included in the study. None of the patients were diagnosed with COVID-19. Sixty-five (41.4%) patients worried about their disease conditions during the epidemic with concerns including medication shortage (73.8%), delay of consultation (46.2%), and disease aggravation (61.5%). Sixty-seven (42.7%) patients had medication withdrawal or dose reduction due to lack of drugs, irregular daily schedule or subjective reasons. The most common psychological problems reported was little interest or pleasure in doing things (66.2%). Patients with progressive disease condition were more distressed and disturbed by the epidemic. Patients with nail involvement felt more worried about their disease conditions than patients without (59.6% vs 31.0%, p =0. 001). ConclusionsThe COVID-19 epidemic imposes a negative impact on the disease management and psychological stress in SAPHO patients. Patients access to specialty care and medication well as mental stress is of great concern.