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Fibromyalgia syndrome （FMS） is a refractory， chronic non-articular rheumatic disease characterized by widespread pain throughout the body， for which there are no satisfactory therapeutic drugs or options. There are rich Chinese medical therapies， and some non-drug therapies， such as acupuncture， Tai Chi， and Ba-Duan-Jin， have shown satisfactory efficacy and safety and definite advantages of simultaneously adjusting mind and body. FMS is taken as a disease responding specifically to traditional Chinese medicine （TCM） by the National Administration of Traditional Chinese Medicine in 2018. In order to clarify the research progress in FMS and the clinical advantages of TCM/integrated Chinese and Western medicine， the China Academy of Chinese Medicine organized a seminar for nearly 20 experts in Chinese and Western medicine， including rheumatology， psychology， acupuncture and moxibustion， and encephalopathy， with the topic of difficulties in clinical diagnosis and treatment of FMS and advantages of TCM and Western medicine. The recommendations were reached on the difficulties in early diagnosis and solutions of FMS， mitigation of common non-specific symptoms， preferential analgesic therapy， TCM pathogenesis and treatment advantages， and direction of treatment with integrated Chinese and Western medicine. FMS is currently facing the triple dilemma of low early correct diagnosis， poor patient participation， and unsatisfactory benefit from pure Western medicine treatment. To solve the above problems， this paper suggests that rheumatologists should serve as the main diagnostic force of this disease， and they should improve patient participation in treatment decision-making， implement exercise therapy， and fully utilize the holistic and multidimensional features of TCM， which is effective in alleviating pain， improving mood， and decreasing adverse events. In addition， it is suggested that FMS treatment should rely on both TCM and Western medicine and adopt multidisciplinary joint treatment， which is expected to improve the standard of diagnosis and treatment of FMS in China.
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Sjögren's syndrome （SS）， a disorder of immune system， is one of the dominant diseases treated by traditional Chinese medicine （TCM）. China Association of Chinese Medicine organized experts in the field of TCM and western medicine rheumatology and pharmacology to discuss the advantages and optimal regimens of TCM for the treatment of SS. The experts generally agreed on the low early diagnosis rate of SS and the lack of targeted therapeutic drugs. In addition， autoimmune abnormality is the key factor in the occurrence of SS and deficiency of both Qi and Yin is the core pathogenesis. SS has unique tongue manifestations， which is expected to allow for the early diagnosis and treatment with integrated traditional Chinese and western medicine. TCM has advantages in treating SS in terms of alleviating clinical symptoms and systemic involvement， individualized treatment， relieving sleep and mood disorders， preventing the occurrence in the early stage， and enhancing the effectiveness and reducing toxicity in the treatment by integrated TCM and western medicine. In general， TCM has advantages in different stages of SS. Internal and external use of TCM， acupuncture， and acupotome are all available options. The optimal regimens should be determined on the basis of pattern identification， stage of disease， and the advantages of TCM. Clinical characteristics and biomarkers of SS should be studied to classify patients， so as to design precision evidence-based TCM regimens for SS. On the basis of unique tongue manifestations of SS， models for early diagnosis and poor prognosis identification of SS should also be established to achieve early prevention and treatment and to improve the prognosis. In the future， we should vigorously carry out high-quality evidence-based medical research on the treatment of SS by TCM and integrated traditional Chinese and western medicine and develop relevant guidelines to optimize and standardize current diagnosis and treatment， thereby laying a basis for clarifying and explaining the advantages of TCM in treating SS.
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BACKGROUND@#The clinical features of enthesitis in patients with psoriatic arthritis (PsA) have been reported in some Western countries, but data in China are very limited. This study aimed to describe the characteristics of enthesitis in Chinese patients with PsA and compared them with those in other cohorts.@*METHODS@#Patients with PsA enrolled in the Chinese Registry of Psoriatic Arthritis (CREPAR) (December 2018 to June 2021) were included. Data including demographics, clinical characteristics, disease activity measures, and treatment were collected at enrollment. Enthesitis was assessed by the Spondyloarthritis Research Consortium of Canada (SPARCC), Maastricht ankylosing spondylitis enthesitis score (MASES), and Leeds enthesitis index (LEI) indices. A multivariable logistic model was used to identify factors related to enthesitis. We also compared our results with those of other cohorts.@*RESULTS@#In total, 1074 PsA patients were included, 308 (28.7%) of whom had enthesitis. The average number of enthesitis was 3.3 ± 2.8 (range: 1.0-18.0). More than half of the patients (165, 53.6%) had one or two tender entheseal sites. Patients with enthesitis had an earlier age of onset for both psoriasis and arthritis, reported a higher proportion of PsA duration over 5 years, and had a higher percentage of axial involvement and greater disease activity. Multivariable logistic regression showed that axial involvement (odds ratio [OR] 2.21, 95% confidence interval [CI], 1.59-3.08; P <0.001), psoriasis area and severity index (PASI) (OR: 1.03, 95% CI: 1.01-1.04; P = 0.002), and disease activity score 28-C reactive protein (DAS28-CRP) (OR: 1.25, 95% CI: 1.01-1.55; P = 0.037) were associated with enthesitis. Compared with the results of other studies, Chinese patients with enthesitis had a younger age, lower body mass index (BMI), a higher rate of positive human leukocyte antigen (HLA)-B27, more frequent dactylitis, and a higher proportion of conventional synthetic disease-modifying antirheumatic drugs' (csDMARDs) use.@*CONCLUSIONS@#Enthesitis is a common condition among Chinese patients with PsA. It is important to evaluate entheses in both peripheral and axial sites.
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Humans , Arthritis, Psoriatic/drug therapy , East Asian People , Enthesopathy/complications , Registries , Severity of Illness Index , Spondylarthritis/epidemiologyABSTRACT
Objective:To investigate the clinical characteristics of patients with rheumatic diseases and abnormal liver function, as well as determine the proportion and severity of liver function abnormalities.Methods:Cross-sectional study. Data were collected from patients registered in the Chinese Rheumatism Date Center from 2011 to 2021. The rheumatic diseases analyzed in this study were rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Sjogren syndrome (SS), ankylosing spondylitis (AS), and gout. Patient data, including demographic characteristics [ such as age, sex, body mass index,(BMI), and smoking history], liver function test results [including alanine aminotransferase (ALT), aspartate aminotransferase, alkaline phosphatase(ALP), and total bilirubin], and use of anti-rheumatic immune drugs and liver-protective drugs, were collected and compared between groups with normal and abnormal liver functions. In addition, the proportions of abnormal liver function were compared between sex and age groups.Results:A total of 116 308 patients were included in this study, including 49 659 with RA, 17 597 with SLE, 9 039 with SS, 11 321 with AS, and 28 692 with gout. The lowest proportion of liver function abnormalities was observed in patients with RA[11.02% (5 470/49 659)], followed by those with SS[17.97% (1 624/9 039)] and AS [18.22% (2 063/11 321) ], whereas patients with SLE [21.14% (3 720/17 597) ] and gout [28.73% (8 242/28 692)] exhibited the highest proportion of these abnormalities. Elevated ALT, mostly classified as grade 1, was the most commonly noted liver function abnormality, whereas elevated ALP was the least common. Some patients who took liver-protective drugs had normal liver function, with the lowest percentage observed in patients with gout [7.45% (36/483) ] and ranging from 21.7% to 30.34% in patients with RA, SLE, SS, and AS. The proportion of liver function abnormalities was higher in males than in females for all disease types [RA: 13.8%(1 368/9 906) vs. 10.3%(4 102/39 753); SLE: 33.6% (479/1 424) vs. 20.0% (3 241/16 173); SS: 25.4%(111/437) vs. 17.6%(1 513/8 602); AS: 20.1%(1 629/8 119) vs. 13.6% (434/3 202); and gout: 29.3% (8 033/27 394) vs. 16.1% (209/1 298)]. In RA, SLE, and AS, the proportions of liver function abnormalities were similar across all age groups. In SS, the proportion of liver function abnormalities increased with age [<40 years: 14.9%(294/1 979); 40-59 years: 18.1%(858/4 741); ≥60 years: 20.4%(472/2 319)], whereas a reversal of this trend was observed in gout [<40 years: 34.9%(4 294/12 320); 40-59 years: 25.5%(2 905/11 398);≥60 years: 21.0%(1 042/4 971)].Conclusions:The proportions of combined liver function abnormalities in patients with rheumatologic diseases were high, and the utilization rates of liver-protective drugs were low. It is necessary to pay more attention to monitoring patients′ liver function, timely administer liver-protective drugs, and optimize liver-protective regimens during the treatment of rheumatic diseases.
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Objective:We sought to investigate the clinical characteristics and risk factors of antiphospholipid syndrome (APS) complicated by autoimmune hemolytic anemia (AIHA).Methods:Retrospective anaysis.Three hundred fifteen consecutive patients with APS were enrolled at the Department of Rheumatology of Peking Union Medical College Hospital between May 2017 to May 2021, and their clinical manifestations[including initial symptoms, time interval between APS onset and diagnosis, systemic lupus erythematosus(SLE), thrombotic events, obstetric morbidity, and extra-criteria manifestations] and laboratory test results[including blood routine, antiphospholipid antibodies(aPLs), blood lipid profile, homocysteine, anti-nuclear antibody profile, immunoglobulin levels, and complement levels] were collected. Then, univariate and multivariate logistic regression analyses were performed. Clinical features and risk factors were analyzed using univariable and multivariable logistic regression analysis.Results:Among 315 APS patients, 37 cases (11.7%) were complicated by AIHA, and AIHA was the first manifestation or co-occurrence. The median time interval between APS onset and diagnosis was 12 months. The proportion of SLE in APS patients combined with AIHA was higher than that in APS patients without AIHA[62.2%(23/37) vs. 19.4%(54/278), P<0.001]. There was no significant difference in the proportions of thrombosis and pregnancy morbidity between the two groups. In terms of extra-criteria manifestations, APS patients with AIHA had a significantly ( P<0.05) greater risk of thrombocytopenia ( OR=6.19, 95% CI 2.81-13.65) and higher proportions of hypocomplementemia, a positive lupus anticoagulant (LA) result, double aPLs positivity[i.e., any two of the following antibodies were positive: LA, anticardilolipin antibody(aCL), and anti-β2 glycoprotein Ⅰ(β2GPⅠ)], and triple aPLs positivity (i.e., LA, aCL, and anti-β2GPⅠ antibodies were all positive). Multivariate logistic regression analysis showed that SLE ( OR=3.46,95% CI 1.60-7.48), thrombocytopenia ( OR=2.56,95% CI 1.15-5.67), and hypocomplementemia ( OR=4.29,95% CI 2.03-9.04) were independent risk factors for the complication of APS. In the primary APS subgroup, multivariate logistic regression analysis showed that livedo reticularis ( OR=10.51,95%CI 1.06-103.78), thrombocytopenia ( OR=3.77, 95% CI 1.23-11.57), and hypocomplementemia ( OR=5.92,95% CI 1.95-17.95) were independent risk factors for the complication of APS. Conclusions:AIHA is not rare in APS patients; moreover, it occurs more frequently in APS secondary to SLE and is more likely to present with a variety of extra-criteria manifestations. Patients with AIHA should be promptly tested for antiphospholipid antibody profiles and alerted to the possibility of thrombotic events.
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Objective:To investigate the clinical features of patients with coexisting connective tissue disease (CTD) and sarcoisosis and to avoid misdiagnosis and mistreatment.Methods:To analyze the clinical manifestations, laboratory data, imaging and pathological features of patients with CTD combined with sarcoidosis in Peking Union Medical College Hospital from January 1985 to December 2021.Results:There were 17 patients with CTD(including 10 SS, 2 DM, 2 PBC, 1 SLE, 1 RA and 1 UCTD), combined with sarcoidosis, with a mean age of (55±10) years old and the ratio of male-to-female was 1:16. Eight patients were diagnosed as CTD before sarcoidosis, while 3 patients after sarcoidosis. The other 6 patients were diagnosed with the two diseases almost simultaneously. Lymphadenopathy(12/17), pulmonary nodules (8/17), subcutaneous nodules (4/17), rash (4/17) and blurred vision (1/17) were the main manifestations of patients with the onset of nodular disease. Nine patients were treated based on the presentation of sardoisis and 5 patients for CTD; 3 patients were treated for both diseases at the same time. All 17 patients discharged with improvement after treatment.Conclusion:When sarcoidosis do coexists with CTD, occult CTD might occur. It is important to investigate specific manifestations including pathological features of sarcoidosis and differentiate it from CTD.
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Objective:To evaluate the differences in clinical characteristics between different genders of Chinese patients with systemic sclerosis(SSc).Methods:The data of SSc patients registered in Chinese Rheumatism Data Center between August 2008 and June 2020 were retrospectively analyzed.Results:A total of 1 844 patients with SSc were enrolled in the study. The ratio of males to females was 289 to 1 555. The onset age was (48.6±13.7) years in males and (45.5±13.1) years in females( P<0.001). Male patients represented shorter disease duration [2.0(0.0, 4.0)years vs.3.0(1.0, 7.0) years, P<0.001],higher proportion of diffuse cutaneous SSc (dcSSc) [63.0% (182/289)vs.44.2%(688/1 555), P<0.001]. Although more man patients experienced smoking [47.4%(137/289) vs. 1.7%(27/1 555), P<0.001] and exposure to harmful environments [7.6%(22/289) vs. 2.1%(33/1 555), P<0.001], there was no statistically significant difference in interstitial lung disease between male and female patients [69.3%(181/261) vs. 74.5%(1 085/1 457), P=0.084].Otherwise, Raynaud′s phenomenon [87.7% (1 364/1 555) vs.75.4%(218/289), P<0.001], arthritis [11.1%(173/1 555) vs.6.9%(20/289), P=0.032], gastroesophageal reflux disease [22.0%(342/ 1 555) vs.13.1%(38/289), P=0.001], and leucopoenia [10.7(161/1 511)% vs. 6.1%(17/279), P=0.019] were more common in female patients, but finger ulcer was less common [22.5%(350/1 555) vs. 30.4%(88/289), P=0.004]. Antinuclear antibody(ANA) positivity rate [85.6%(1 310/1 531) vs. 78.6%(221/281), P=0.003], anti-RNP antibody positivity rate [23.1%(342/1 479) vs.14.0%(38/271), P=0.001], anti-SSA antibody positivity rate [28.2%(419/1 487) vs.13.9%(38/274), P<0.001] were higher in female patients. Physician′s global assessment(PGA) scores [1.4 (1.0, 2.0) vs. 1.0 (0.3, 1.6), P<0.001] and modified Rodnan Skin Score(mRSS) [18.0 (9.5, 28.0) vs. 14.0 (5.0, 28.0), P=0.003] were higher in males. Conclusion:Even though male SSc patients account for a small proportion, more extensive skin involvement, finger ulcers and higher PGA are manifested in males. Physicians need pay attention to these clinical disparities between different genders in SSc.
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Objective:To analyze the risk factors of patients with ankylosing spondylitis (AS) combined with premature coronary atherosclerotic heart disease (PCAD).Methods:A total of 74 patients with AS and coronary atherosclerotic heart disease (CAD) in Peking Union Medical College Hospital from January 1983 to July 2021 were enrolled. According to the age of onset of coronary heart disease, the 74 patients were divided into PCAD group and NPCAD (non-premature coronary heart disease) group. T test and Chi square test were used to analyze the data of the two groups, the risk factors for AS-PCAD were analyzed by multivariate Logistic regression. Results:① There were 37 cases in the PCAD group and 37 cases in the NPCAD group. In the PCAD group, there were 28 men and 9 women; wherease all were men in the NPCAD group. The difference was statistically significant ( χ2=10.25, P=0.001). ② Compared with the NPCAD group, the age of AS-PCAD group was younger [(23±10) years vs (29±12) years, t=-2.28, P=0.026], and the course from AS to CAD was shorter [(25±10) years vs (34±13) years, t=-3.00, P=0.004], hemoglobin (Hb) level was lower [(122±23) g/L vs(132±18) g/L, t=2.10, P=0.039], rate of anemia was higher [38.5%(14/37) vs 16.2%(6/37), χ2=4.39, P=0.037]. Proportion of increased C-reactive protein (CRP) was higher [65.5%(19/29) vs 35.5%(11/31), χ2=5.41, P=0.019]. ③ Juvenile onset AS (JoAS)[ OR(95% CI)=3.45(1.31, 9.10), P=0.012] and high levels of CRP [ OR (95% CI)=3.68 (1.44, 9.40), P=0.006] might berisk factors of AS-PCAD by multiple logisctic regression analysis. Conclusion:Patients with AS have a higher probability of PCAD, especially in those patients with JoAS, persistent inflammation and anemia. It is necessary to be alert to the risk of PCAD and early screening.
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Objective:To investigate the clinical and laboratory characteristics of systemic sclerosis (SSc) patients at different age of onset.Methods:Data of SSc patients with onset age ≥18 years old who were registered in the Peking Union Medical College Hospital and Chinese Rheumatism Data Center from August 2008 to June 2020 were included. Patients were divided into 3 groups by the age of onset according to the age segmentation of the World Health Organization. Counting variables were presented as frequency (percentage). Quantitative results were presented as mean±standard deviation, or median, inter quartile range. Differences between groups were analyzed by analysis of variance, the Mann-Whitney test or the chi-square test, depen-ding on the distribution of the variables.Results:Six hundred and eighty-two SSc patients were included. Accor-ding to the age of onset, they were divided into three groups: youth group (18-44 years old), middle-aged group (45-59 years old) and elderly group (over 60 years old). There were 361 patients in the youth group,245 patients in the middle age group and 76 patients in the elderly group. The mean age of onset was (43.8±12.1) years. The variables with significant different among the groups were as the following: left ventricular diastolic dysfunction [14.0%(14/100), 38.8%(39/98), 65.4%(17/26); χ2=30.756, P<0.001]; cardiac arrhythmias [1.9% (7/361), 3.7% (9/361), 7.9% (6/76), χ2=7.38, P=0.024), Raynaud's phenomenon [94.7% (342/361), 89.4%(219/245), 89.5%(68/76), χ2=6.73, P=0.035], loss of finger pad substance [36.9%(133/360), 25.4% (62/244), 18.4% (14/76), χ2=15.184, P=0.001]; digital ulcer [31.0% (112/361), 23.0% (56/244), 15.8% (12/76), χ2=9.86, P=0.007]; arthritis [16.3%(59/361), 13.5%(33/245), 5.3%(4/76), χ2=6.49, P=0.039], digital contracture [11.6%(42/361), 5.7%(14/245), 9.2%(7/76), χ2=6.10, P=0.047]; positive anti-RNP antibody [32.3% (116/359), 20.7% (50/241), 17.3% (13/75), χ2=14.06, P=0.001]; and positive anti-centromere antibody [8.9% (32/351), 18.4%(45/239), 23.7%(18/76), χ2=17.78, P<0.001] were significantly different between the young age group and elder group. Conclusion:The predominant age of disease onset of SSc is middle and young age. Elder onset SSc patients are more likely to have left ventricular diastolic dysfunction, and young onset patients are more likely to have microvascular lesions, which needs more attentions by clinicians.
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Objective:To describe a series of systemic sclerosis (SSc) patients with the combination of scleroderma renal crisis (SRC) and pulmonary arterial hypertension (PAH).Methods:The medical records of 472 SSc patients in Peking Union Medical College Hospital between January 2012 and October 2020 were reviewed and a retrospective analysis of the characteristics of patients with SRC and PAH among SSc patients was conducted.Results:Thirteen patients suffered from SRC and PAH in the SSc patients, 1 case was limited cutaneous SSc, and 12 cases were diffuse cutaneous SSc. Five patients had renal crisis before pulmonary arterial hypertension, 4 patients had pulmonary arterial hypertension before the occurrence of renal crisis, and the remaining 4 patients were found at the same time. Among them, 11 patients had Raynaud's phenomenon, 7 had gastrointestinal bleeding, 6 had pulmonary edema and 3 had telangiectasias. Twelve cases were positive for anti-nuclear antibodies and 4 cases were positive for anti-Scl-70 antibodies. N-terminal pro-brain natriuretic peptide (NT-proBNP)>1 400 ng/L in 11 patients. Two patients had thrombotic microangiopathy (TMA). Among the 13 patients, 3 patients died during hospitalization, 2 patients were lost to follow-up, and 2 patients died within 5 years of follow-up. Six patients survived, and 1 of the 4 patients with regular dialysis were discharged from dialysis.Conclusion:In patients with scleroderma, SRC can occur earlier, later than, or at the same time with SSc-PAH. Patients may have a higher incidence of gastrointestinal bleeding and higher level of NT-proBNP. PDE5i or ERAs may be beneficial.
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Interstitial lung disease (ILD) is a frequent complication of patients with connective tissue disease (CTD) and significantly affects morbidity and mortality. Disease course may vary from stable or mildly progressive to more severe, with rapid loss of lung function. At present, there are great challenges and poor prognosis in the diagnosis and treatment of CTD-ILD. Based on the evidence and guidelines from China and other countries, experts from the Chinese Rheumatology Association developed standardization of diagnosis and treatment of CTD-ILD. The aim is to strengthen the early identification of, standardize the diagnosis and treatment of CTD-ILD, and delay the progress of the disease.
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To investigate the distribution and clinical significance of nuclear dense fine speckled (DFS) pattern in various diseases. A total of 95 289 patients who received DFS tests at Peking Union Medical College Hospital from January 2019 to December 2020 were included in this study. The results of indirect immunofluorescence assay (IIF) for detection of antinuclear antibody (ANA) were evaluated. The positive rates of ANA and DFS were 39.60% (37 733/95 289) and 1.19% (1 139/95 289) respectively. The positive rate of DFS in ANA-positive patients was 3.02% (1 139/37 733). DFS and ANA positivity were significantly different among different age groups rather than gender. The positivity rate of DFS reached the peak (55.57%, 633/1 139) in young patients between 21-40 years, while positive ANA with negative DFS was mainly observed in patients between 41-60 years (37.26%, 13 636/36 594). Additionally, single ANA-positivity were mainly detected in rheumatology department (59.23%, 18 402/31 066), whereas positive DFS was more common in obstetrics and gynecology department (3.08%, 49/1 593). There were 82.88% (944/1 139) patients with positive DFS diagnosed with non-autoimmune disease (non-AID), and 19.49%(222/1 139) with dermatosis. Positive DFS with higher titer (≥1∶320) was detected more frequently in autoimmune disease (AID) patients (5.13%, 10/195) than in non-AID patients (1.69%, 16/944) ( P<0.05). The DFS pattern is rare in ANA positive patients, which is mainly observed in women between 21-49 years. High titer of DFS is prevalent in AID patients, but positive DFS is detected more in non-AID patients, especially those with dermatosis.
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A 50-year-old man was admitted to the Department of Rheumatology at Peking Union Medical College Hospital with rash for 6 months, and fever and hematuria for 5 months. The main clinical manifestations included fever, fatigue, purpura, hematuria and thrombocytopenia. He was positive for antinuclear antibody (ANA), anti-neutrophil cytoplasmic antibodies (ANCA) and rheumatoid factor (RF), and had low complement levels. Initial blood culture, echocardiography and chest CT showed no signs of infection. Diagnosis of connective tissue disease was made initially. His disease improved under treatment with glucocorticoids and immunosuppressive agents, but relapsed when glucocorticoids were tapered. After admission, the diagnosis was reconsidered, and infective endocarditis was finally diagnosed with repeated positive blood cultures and vegetations detected by transesophageal echocardiography. Amoxicillin and clavulanate potassium were initiated, and surgery was performed. His symptoms finally recovered gradually.
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Objective:To investigate the relationship between psoriasis severity and clinical features in psoriatic arthritis (PsA).Methods:Patients were recruited from the Chinese REgistry of Psoriatic ARthritis (CREPAR) between December 2018 and June 2021, and data were collected including the baseline demographic characteristics, various clinical manifestations (including arthritis, nail disease, comorbidities), laboratory tests[including erythrocyte sedimentation rate(ESR), C-reactive protein (CRP)], health assessment questionnaire (HAQ). Body surface area (BSA) and psoriasis area and severity index (PASI) were selected for the tools of assessment of cutaneous psoriasis. Patients were divided to two groups, including the severe psoriasis group (BSA>10%) and the non-severe psoriasis group (BSA≤10%). Disease assessment included ankylosing spondylitis disease activity score (ASDAS), disease activity score 28 (DAS28) and disease activity in psoriatic arthritis (DAPSA).Results:1 074 eligible patients with PsA were recruited, and 106 (9.9%) had severe psoriasis. Compared with non-severe psoriasis group, the severe psoriasis group had more peripheral joint involvement (including patients with ever or current peripheral arthritis, 94.3% vs. 85.6%), more polyarticular joint involvement (including patients with current peripheral arthritis, 74.0% vs. 58.2%), more axial joint involvement (51.4% vs. 39.9%), more nail disease (72.6% vs. 61.4%), more frequency of smoking (20.2% vs. 18.7%), and higher proportion of hypertension (23.4% vs. 14.4%). In addition, the severe psoriasis group had higher level of ESR [33(10, 70) mm/1h vs. 20(9, 38) mm/1h] and CRP [18.6(5.0, 60.8) mg/L vs. 7.0(2.4, 18.1) mg/L], higher values of DAS28-ESR (4.5±1.7 vs. 3.7±1.5), DAS28-CRP (4.2±1.5 vs. 3.4±1.4), ASDAS-ESR (3.5±1.4 vs. 2.6±1.2), and ASDAS-CRP(3.4±1.6 vs. 2.5±1.2), higher scores of HAQ [0.6(0.1, 1.0) vs. 0.3(0.0, 0.8)].Conclusion:Patients with PsA with severe psoriasis bore a heavier disease burden. Therefore, clinicians were supposed to pay more attention to them. In addition to skin lesions, they should also focus on examination of other clinical manifestations, such as joints and nails.
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Objective:To study clinical characteristics and pregnancy outcomes under anti-coagulation therapy of non-criteria obstetric antiphospholipid syndrome.Methods:Patients suspected of obstetric antiphospholipid syndrome(OAPS) were recruited through Chinese Rheumatism Data Center from 2015 to 2019 consecutively. Patients fulfilling 2006 Sydney revised antiphospholipid syndrome criteria were classified as OAPS. Patients fulfilling definition of non-criteria OAPS(NCOAPS) by expert consensus on diagnosis and management of obstetric antiphospholipid syndrome of China were classified as NCOAPS. Clinical characteristics and laboratory results of two groups were compared. Live birth rates and pregnancy outcomes under anti-coagulation therapy were studied.Results:A total of 88 patients were enrolled, including 56 patients (63.6%) as OAPS, 32(36.4%) as NCOAPS. Live births were only reached in 16.1% (9/56) in OAPS patients and 12.5%(4/32) in NCOAPS. Fetal losses after 10 weeks of gestation and pre-eclampsia before 34 weeks were more common in OAPS group compared to NCOAPS group [78.6%(44/56) vs. 18.8%(6/32), P<0.001; 25.0%(14/56) vs. 3.1%(1/32), P=0.020, respectively]. After enrollment, 15 pregnancies were recorded in OAPS, 10 in NCOAPS, all of whom were treated with low-dose aspirin (LDA) combined with low-molecular weight heparin (LMWH). Live birth rates saw dramatic improvements compared to baseline levels in OAPS [16.1% (9/56) vs. 11/15] along with NCOAPS [12.5% (4/32) vs. 7/10]. Conclusion:Though NCOAPS and OAPS patients differ in antiphospholipid antibody spectrum and pattern of pregnancy morbidities, both groups benefit from LDA combined with LWMH treatment, as live birth rates improve. Non-criteria OAPS patients are recommended to receive anti-coagulation therapy during pregnancy.
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Objective@#Portal vein thrombosis (PVT) is a rare and severe clinical manifestation of antiphospholipid syndrome (APS), as well as a predictor of poor prognosis. This study was conducted to explore the clinical features and risk factors of PVT in APS patients.@*Methods@#A total of 123 APS patients diagnosed from 2012 to 2019 were retrospectively enrolled. The diagnosis of PVT was made according to the 2009 American College of Liver Diseases (AASLD) criteria. Clinical and laboratory data were collected. A multivariate (MV) logistic regression model was constructed using a stepwise forward selection procedure among those candidate univariables with P values<0.10.@*Results@#A total of 28 cases with PVT, and 95 control cases without PVT were finally enrolled.The 28 APS-PVT patients included 5 males and 23 females with age range from 17 to 63 years. Clinical manifestations included acute thrombosis in 8 patients, chronic thrombosis in 16, and 4 with portal vein spongiform. As to the involved vessels, single portal vein thrombosis was seen in 20 patients, portal combined with superior mesenteric vein (SMV) and splenic vein in one patient, portal plus SMV in 4 and only SMV in 3 patients. Other manifestations were portal hypertension (16/28), esophageal varices (13/28), spleen infarction (7/28) and gastrointestinal bleeding (4/28). Two antiphospholipid antibodies were positive in 13 cases. Triple positive antibodies were seen in 7 cases. Multivariate logistic regression analysis showed that disease duration less than 0.5 years (OR=72.74, 95%CI 7.50-705.45, P<0.001), hypoalbuminemia (OR=356.45, 95%CI 19.19-6 620.14, P<0.001), and elevated erythrocyte sedimentation rate (ESR)/C-reactive protein (CRP) (OR=14.41, 95%CI 1.49-139.20, P<0.001) were independent risk factors for PVT in APS.@*Conclusion@#PVT is usually misdiagnosed due to insidious onset. Short disease duration, hypoalbuminemia and elevated ESR/CRP are risk factors for PVT in APS. Better understanding, early diagnosis and treatment will improve the clinical outcome.
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Objective To explore the predictive factors for determining the therapeutic response and prognosis of severe thrombocytopenia (TP) in patients with primary Sj(o)gren syndrome(pSS).Method Patients with pSS and severe TP (platelet count ≤ 50× 109/L) admitted between 2010 to 2016 at Peking Union Medical College Hospital were classified according to their therapeutic response and analyzed retrospectively.The response parameters and clinical data including bone marrow aspiration results and laboratory findings were collected.Result Thirty patients were finally analyzed,including twenty with appreciable bone marrow aspiration results.Fourteen and 7 patients achieved a complete response (CR) and a partial response (PR) respectively,other 9 patients with no response (NR).The megakaryocyte counts in bone marrow (BM-MK) counts per slide in each group were 13.0 (9.2,23.5) in CR patients,7.0 (7.0,20.0) in PR patients,and 5.0 (1.0,6.0) in NR patients.BM-MK counts in patients with clinical response (CR+PR)were significantly higher than those with NR (P=0.006).A receiver-operation characteristic analysis revealed a cutoff value of BM-MK counts at 6.5 per slide stratifying patients by different responses with a sensitivity of 13/14,a specificity of 6/7,and area under the curve of 0.879.Univariate analysis indicated a better prognosis as BM-MK counts＞6.5 per slide.Conclusion BM-MK count could be a predictive factor of response in patients with pSS and severe TP.Patients with BM-MK counts≤6.5 per slide represent worse platelet improvement..
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Objective To analyze the clinical characteristics of antiphospholipid syndrome (APS) patients with chronic thromboembolic pulmonary hypertension (CTEPH).Methods A total of 22 APS patients with CTEPH were enrolled in our study,who were admitted in Peking Union Medical College Hospital from January 2012 to August 2018.Diagnoses were confirmed by computed tomographic pulmonary angiography (CTPA),or pulmonary angiography.Demographic characteristics,clinical manifestations,laboratory tests,therapy,World Health Organization (WHO) functional class were retrospectively collected.Results There were 15 females and 7 males with a median age of 29-year-old.Chest pain (6 cases),dyspnea on exertion (22 cases),cough (6 cases) and hemoptysis (9 cases) were the most common clinical manifestations.Lupus anticoagulant (LA),anticardiolipin (ACL) antibodies and anti-beta 2 glycoprotein Ⅰ (anti-β2 GP Ⅰ) antibodies were all positive in 12 patients,two of three antibodies positive in 5 patients,only one positive in 5 patients.The WHO functional classes were Ⅱ-Ⅳ before treatment.Anticoagulants were administrated in all patients.After multidisciplinary evaluation,9 patients underwent pulmonary thromboendarterectomy (PTE),who all had a good outcome.Symptoms in eleven over thirteen patients with only anticoagulants improved.Three patients developed cardiac deterioration while other 3 patients died of right heart failure during follow-up.Conclusion Pulmonary embolism is one of the most common thrombotic events in APS patients.It is important to recognize symptoms and signs related to pulmonary embolism and start anticoagulation as soon as possible.Standard anticoagulation improves symptoms but can't reverse the process of pulmonary hypertension.Some patients may benefit from PTE after anticoagulation and multidisciplinary evaluation.
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Objective To summarize the neurological manifestations in patients with primary Sj?gren′s syndrome (pSS). Methods A total of 68 patients were diagnosed as pSS in neurology department of Peking Union Medical College Hospital from March 2014 to February 2018, among whom sixteen cases were excluded due to modified final diagnoses of primary neurological diseases. Therefore 52 pSS patients with neurological involvement were enrolled and retrospectively analyzed. They were divided into two groups as extensive group in which both central and peripheral nervous system were involved, non?extensive group in which either central or peripheral nervous system was involved. Results Neurological manifestations were presented as primary symptoms in 98.1%(51/52) patients, while 35 had neurological involvement as their only extraglandular manifestations. Thirteen cases were in extensive group. The other 39 in non?extensive group including 22 cases with only peripheral nervous system involved and 17 cases with only single central nervous system involved. Compared to non?extensive group, the proportion of woman patients [13/13 vs.71.8% (28/39),P＝0.047], serum IgG level [17.73(11.11,22.41)g/L vs. 11.49(9.58,13.40)g/L, P＝0.017] and positive rates of oligoclonal band (OB) in cerebral spinal fluid (CSF) [7/13 vs. 22.6%(7/31), P＝0.042)] were significantly higher in extensive involvement group. Conclusions Neurological manifestations in pSS patients could be extensive, both central and peripheral nervous system might be associated. Female patients, high serum IgG level and positive OB in CSF are risk factors of extensive neurological involvement, suggesting that the immune system may be generally over?stimulated.
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A 45-year-old woman was admitted to the Department of Rheumatology and Immunology, Peking Union Medical College Hospital, due to weakness of the upper limbs, fever, and blurred vision. She was clinically diagnosed as systemic lupus erythematosus overlapped primary biliary cirrhosis, with renal, retinal, hematological and musculoskeletal involvement, combined with severe pulmonary infection and respiratory failure. Treated with glucocorticoids, ursodeoxycholic acid, antibiotics and respiratory support, the patient got better. A couple of days later, her fever recurred and platelets count dropped to 30×109/L, hemoglobin to 78 g/L, fibrinogen to<1.5 g/L, ferritin to 1 640 ng/ml, natural killer (NK) cell count to 8/μl, the activity of NK cells 2% (reference value 9.5%-23.5%), considering the occurrence of hemophagocytic lymphohistiocytosis (HLH). Cytomegalovirus pp65 antigenemia test: 13 positive cells/2×105 WBC. Considered the possibility of HLH caused by cytomegalovirus infection and treated by 250 mg ganciclovir intravenous drip twice a day for a full course. The temperature of the patient was gradually reduced to 36.5℃, the count of platelets were increased to 229×109/L, the hemoglobin was increased to 94 g/L, and the fibrinogen was increased to 3.26 g/L. When there were unexplained critical signs of the primary disease during systemic lupus erythematosus treatment, severe complications such as infection, HLH, thrombotic thrombocytopenic purpura should be taken into account.