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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.
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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.
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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.
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Sjögren's syndrome (SS) is a common chronic systemic autoimmune disease;however,its pathogenic mechanisms remain unclear. Proper mouse models of SS are essential for experiments. This article summarizes the recent advances in spontaneous mouse models of SS,genetically engineered mouse models of SS,and experimentally induced mouse models of SS.
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Animales , Humanos , Ratones , Modelos Animales de Enfermedad , Síndrome de SjögrenRESUMEN
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.