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Objective:To analyze the clinical characteristics of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).Methods:A retrospective study was conducted on AAV patients with long-term follow-up in the rheumatology outpatient clinic of Peking Union Medical College Hospital between February 2015 and February 2022. The demographic characteristics, clinical manifestations, concurrent events, treatment, and prognosis of the three clinical subtypes of AAV were collected and analyzed.Results:There were 71, 45, and 31 cases of granulomatous polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatous polyangiitis (EGPA), respectively, among 147 patients. The ANCA positivity rates in the three groups were 91.5%, 95.6%, and 19.4% ( χ2=76.68, P<0.01), respectively. The upper respiratory tract and lungs were the most frequently affected organs in GPA and EGPA, and the kidneys and lower respiratory tract were the main organs involved in MPA. In addition, cardiac and neurological involvement and thrombosis rates were significantly higher in EGPA patients than in GPA and MPA (12.9%, 9.7%, 41.9% and 19.4%, respectively; χ2=8.51、7.13、7.54、0.02, P<0.05) .The median follow-up time for the three groups of patients was 43, 28, and 46 months respectively.Relapse was more common in patients with GPA and EGPA (up to 59.2% and 64.5%; χ2=11.26, P=0.004), with the lungs and ENT being the most common relapse organs (GPA of 61.9% and 40.5%, EGPA of 55.0% and 50.0%), the lungs and kidneys were the most common manifestations in MPA relapse (64.3% and 60.0%, respectively). The main therapeutic agents were glucocortoid (95.9%), cyclophosphamide (71.4%), methotrexate (54.4%), tripterygium wilfordiz (34.0%),mycophenolate mofetil (31.3%), azathioprine (29.3%), leflunomide (19.0%), rituximab (19.0%), and tacrolimus/cyclosporine (8.8%). There were 6 deaths (4.1%) occurred during the follow-up period of this study. Conclusion:The clinical features of AAV are similar to those reported in the literature and relapses are common>he vast majority of patients need to be treated with glucocorticoid combined with immunosuppressive agents.
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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:To detect the microRNAs (miRNAs) and proteins carried by exosomes in the plasma of patients with newly diagnosed Takayasu′s arteritis (TAK) and analyze their possible roles in the pathogenesis of TAK.Methods:Ten patients with newly diagnosed TAK from the Department of Rheumatology and Immunology, Peking Union Medical College Hospital were selected during June-November 2020. Five healthy controls were matched with five patients by age and sex. RNA sequencing and protein mass spectrometry were used to detect miRNAs and proteins, respectively, carried by exosomes in the plasma. Differentially expressed miRNAs (DE-miRNAs) and proteins (DEPs) were screened. Thereafter, hierarchical cluster analysis, function, signal pathway, and protein domain enrichment analysis of DE-miRNAs and DEPs were performed. Finally, miRNAs and proteins related to vasculitis and autoimmunity were identified. The possible roles of the miRNAs and proteins in the pathogenesis of TAK were explored. Enumeration data were compared using Fisher′s exact probability test or Chi-square test, and a P-value<0.05 was considered significant. Results:Compared with the healthy controls, patients with TAK had 29 DE-miRNAs on their plasma exosomes. Among these DE-miRNAs, miR-101-3p, miR-122-5p, miR-143-3p, miR-185-3p, miR-192-5p, miR-194-5p, miR-19a-3p, miR-19b-3p, miR-20b-5p, miR-21-5p, miR-22-3p, miR-335-5p, miR-34a-5p, miR-3613-5p, miR-548ad-5p, miR-590-3p, and miR-7-5p were upregulated; whereas miR-1249-3p, miR-141-3p, miR-199a-5p, miR-199b-5p, miR-200a-3p, miR-200c-3p, miR-204-5p, miR-29c-5p, miR-335-3p, miR-381-3p, miR-4433b-5p, and miR-584-5p were downregulated. Finally, miR-34a-5p, miR-200c-3p, miR-143-3p, miR-22-3p, and miR-21-5p were identified. Among the 357 DEPs screened, 236 DEPs were upregulated, whereas 121 DEPs were downregulated. Finally, kallikrein B1 (KLKB1), kininogen 1 (KNG1), desmoplakin (DSP) were identified.Conclusion:MiR-34a-5p, miR-200c-3p, miR-143-3p, miR-22-3p, miR-21-5p, KLKB1, KNG1, and DSP carried by exosomes in plasma might participate in the pathogenesis of TAK by regulating vascular physiology, inflammation, autoimmunity, and other processes. They may be biomarkers and therapeutic targets of TAK.
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The American College of Rheumatology (ACR) and the American Association of Hip and Knee Surgeons (AAHKS) convened a writing group to develop a consensus report on the management of anti-rheumatic medication in patients with rheumatic diseases undergoing elective total hip or total knee arthroplasty in June 2022. In particular, the consensus provides significant updates on target synthetic disease modifying anti-rheumatic drugs and perioperative medication management in patients with systemic lupus erythematosus, as well as the addition of newly approved antirheumatic medications for administration. This article will interpret the consensus and provide a reference for the perioperative management of antirheumatic medications for hip and knee arthroplasty in patients with rheumatic diseases in China.
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Objective:To explore the efficacy and safety of adalimumab (ADA) in Chinese Takayasu arteritis (TAK) patients.Methods:This study prospectively included ten refractory active TAK patients. The clinical, laboratory and imaging data of these patients before and after ADA treatment were collected and compared. Statistical analysis was carried out with the statistical product and service solutions (SPSS) program V.24.0. We performed univariate analysis by Wilcoxon-Mann-Whitney test for numerical data according to normality. The P value <0.05 was interpreted as statistically significant. Results:Ten TAK patients recruited in this study were all female, with a median age of 27.5 [25.0, 36.0] years. Fever or fatigue (6/10, 60%), neck pain (6/10, 60%), and limb claudication (4/10, 40%) were the most common clinical manifestations. The most common pattern of vascular involvement was Numano type V (7/10, 70%). The serum erythrocyte sedimentation rate (ESR) [22.0(4.5, 37.5) mm/1 h vs 7.0(4.5, 31.0) mm/1 h, Z=-2.04, P=0.042] and IL-6 level [12.3(3.6, 57.7) ng/L vs 2.8(2.0, 24.0) ng/L, Z=-2.19, P=0.028] reduced significantly after ADA treatment. The thickness of the common carotid artery intima decreased from 2.7(2.2, 4.0) mm to 2.3(1.6, 3.4) mm after ADA treatment ( Z=-1.99, P=0.046). The lumen diameter of the common carotid artery increased from 0.31(0.20, 0.42) cm to 0.37(0.29, 0.43) cm ( Z=-2.02, P=0.043). The glucocorticoid dosage was reduced from [11.2(6.9, 15.0) mg to 10.0(5.0, 13.1) mg, Z=-1.89, P=0.059]. In the 10 patients, 6 patients (60%) reached complete response, 2 patients (20%) reached the partial response, and the total effective rate was 80%(8/10). Pneumonia in one TAK patient was recorded. Conclusion:The results of this study suggest, that ADA can be used for refractory active TAK patients, and is safe.
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Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a group of systemic small vasculitis characterized by ANCA positive in serum. Three diseases are included in this group of diseases: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). In China, standardized diagnosis and treatment of AAV is still lacking. Based on the evidence and guidelines from China and abroad, the Chinese Rheumatology Association formulated the standardization of diagnosis and treatment of ANCA associated vasculitis. The purpose is to standardize the diagnosis of AAV and disease activity assessment, and recommend the treatment strategies.
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Objective:To evaluate the efficacy and safety of rituximab(RTX) as remission-mainten-ance therapy in antineutrophil cytoplasmic antibody(ANCA) associated vasculitis(AAV).Methods:Patients with AAV, including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), treated with rituximab (RTX) in Peking Union Medical College Hospital during September 2005 to June 2021 were included into this study. Clinical data, relapse rate, time of first relapse and adverse events were collected and analyzed. The cumulative relapse rate was calculated by Kaplan-Meier, t test, and Man-Whithey U test and chi-square were used to compare differences between two groups. Results:① Thirty-nine AAV patients were enrolled, including 36 GPA and 3 MPA. During the 20(3, 104) months follow-up, 59.0%(23/39) patients had suffered relapses. The time for first relapse was 11(3, 42) months after remission. ② There were no difference in the relapse rate [60.0%(18/30) vs 55.6%(5/9), χ2=0.06, P=1.000), the time of first relapse [15(3, 42) vs 10(9, 30), Z=0.45, P=0.678], CD19 + B [23.5 (5, 148) cell/μl vs 3(2, 15) cell/μl, Z=0.57, P=0.605] and serum IgG [7.09(5.13, 13.90) g/L vs 9.72(5.32, 12.0) g/L, Z=0.36, P=0.770] between standard dose and low-dose groups. The rate of major relapse-free was significantly less in patients treated with standard dose than patients with reduced dose of RTX {87.1%[95% CI(73.4%, 100.8R%)] vs 64.3%[95% CI(23.1%, 105.4%)], χ2=7.59, P=0.006}. ③ There were no difference in relapse rate [50.0%(3/6) vs 60.6%(20/33), χ2=0.24, P=0.674], time of first relapse [23(6, 25) vs 11(3, 42), Z=0.05, P=0.982], CD19 + B[35(15, 50) cell/μl vs 10(0, 148) cell/μl, Z=0.95, P=0.382] and serum IgG[6.70(5.91, 7.49) g/L vs 7.69(3.78, 13.90) g/L, Z=0.48, P=0.700] between the fixed interval dosage and the on-demand dosage groups. There was no difference in the rate of major relapse-free between the two groups (100% vs 77.8%, χ2=1.79, P=0.181). ④ The incidence of infusion reaction was 5.1%(2/39) and infection was 20.5%(8/39). Serum IgG level was 4.37(3.78, 13.4) g/L at infection. There was no difference in safety between the standard and low-dose groups or between fixed interval and on-demand dosage groups ( P>0.05). Conclusion:There is no significant difference in relapse rate bet-ween the standard RTX dose and low-dose RTX induction therapy group, but the major relapse rate is sign-ificantly reduced in the standard dose RTX therapy. The relapse rate of fixed intervals dosage group is similar to that of on-demand dosage group. The safety profile of the standard dose and low-dose induction therapy groups or fixed intervals and on-demand dosage groups is similiar.
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One 51 years old man was admitted to the rheumatology department with a history of prominent eyes, headache and blurred vision for half year. The main manifestations included retrobulbar inflammatory pseudotumor and retroperitoneal fibrosis. He was initially diagnosed as granulomatosis with polyangiitis. Prednisone and cyclophosphamide were administrated and effective. New mass of dura mater and osteosclerosis presented during follow up. Finally Erdheim Chester disease(ECD) was diagnosed by biopsy and pathological examination. Vemurafenib, a v-raf murine sarcoma viral oncogenes homolog B1 (BRAF) inhibitor, 480 mg was given twice a day. The patient′s condition is stable and still in follow-up. Although ECD is a rare histiocytosis, clinicians should pay attention to its manifestations and differential diagnoses.
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With the recognition of antineutrophil cytoplasmic antibodies (ANCA)-related vasculitis and widespread vaccination against viral hepatitis B, the prevalence of polyarteritis nodosa (PAN) varied considerably. In our study, patients diagnosed as polyarteritis nodasa (PAN)based on the 1990 American College of Rheumatology(ACR) criteria were reclassified using 2007 European Medicines Agency(EMA) algorithm modified by 2012 Chapel Hill Consensus Conference(CHCC) definitions, aiming to evaluate the new classification criteria for the diagnosis of PAN. A total of 113 PAN patients admitted to Peking Union Medical College Hospital from January 2002 to December 2018 were retrospectively analyzed, who were classified into three subtypes including 9 patients with cutaneous, 80 with classic and 24 Hepatitis B virus (HBV) associated PAN. All patients were reclassified according to 2007 EMA algorithm using CHCC 2012 definitions. As a result, 7 patients were diagnosed as microscopic polyangiitis(MPA) and 19 patients with unclassified vasculitis based on the new classification criteria. The diagnostic rate of PAN was gradually declined as the classification criteria of vasculitides was update. However, there are quite a few PAN patients in China, whom rheumatologists should pay attention to the early diagnosis and treatment.
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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:To investigate the clinical characteristics and risk factors for osteonecrosis (ON) in patients with systemic lupus erythematosus (SLE).Methods:This is a case-control study. A total of 118 patients diagnosed with SLE complicated with ON (study group) were retrospectively analyzed between 2014 and 2019. Gender, age, and course matched 118 SLE patients without ON were selected as controls. Clinical manifestations, laboratory findings, medical history, and treatments were recorded and analyzed.Results:Among 118 patients, the male to female ratio was 20 to 98 with a median age of 27 years and course of disease 1-168 months. Compared with the control group, the study group presented a longer cumulative duration of glucocorticoid therapy [36.5 (0-168) months vs. 19.0(0-168) months on average, P<0.05], a higher incidence of osteoporosis (29.7% vs. 4.2%, P<0.001), a higher frequency of immune-suppressive therapy (83.9% vs. 64.4%, P=0.035), more organs involveed [median 2 (0-5) vs. 1 (0-4)], and a higher SLE disease activity index (SLEDAI) (14.22±7.40 vs. 11.63±6.11, P<0.05) in univariate logistic regression. The control group had a higher rate of positive Coombs test (39.8% vs. 7.6%, P<0.05). No statistical difference on methylprednisolone (MP) pulse therapy ( P>0.05) was observed. Multivariate logistic regression suggested that SLEDAI ( OR= 1.070, 95% CI 1.026-1.116, P<0.005), osteoporosis ( OR=10.668, 95% CI 3.911-29.103, P<0.001) and a positive Coombs test( OR=0.492, 95% CI 0.266-0.910, P<0.05) were related to the development of ON in SLE patients. Conclusion:A higher disease activity and the presence of osteoporosis are associated with an increased risk of ON in patients with SLE, and positive Coombs test seems a protective factor of ON.
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Objective:To analyze the relevant risk factors for restenosis after percutaneous transluminal angioplasty of patients with Takayasu′s arteritis.Methods:Clinical data of 43 patients undergoing percutaneous angioplasty due to Takayasu arteritis were retrospectively analyzed. Univariate and multivariate Logistic regression analysis was used to explore the relevant risk factors for restenosis after percutaneous transluminal angioplasty.Results:There were 9 males and 34 females. The mean age was 23 (18-33) years old, 59 times of PTA were performed, including 44 in renal artery, 9 in aorta, 2 in iliac and 2 in carotid artery, 1 in brachiocephalic trunk and 1 in left subclavian artery. The mean follow up time was (64±42) months. The rate of restenosis was 47.5%(28/59)and the mean time of restenosis was (23±27) months. The restenosis rate of aorta and iliac artery was 9.1%, that of renal artery was 52.3% and that of supra aortic artery was 100% . The rate of restenosis was higher in patients with symptoms of headache, syncope and low back pain, the elevated ESR and CRP increased the risk of restenosis (all P<0.05). Multivariate Logistic analysis showed that preoperative elevation of ESR and CRP were risk factors for restenosis after percutaneous angioplasty for Takayasu arteritis. Conclusions:PTA was safe and effective in Takayasu arteritis involving aorta-iliac and renal artery, the elevated ESR and CRP was related to high risk of restenosis.
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Objective:To analyze the clinical and prognostic characteristics in patients with eosinophilic granulomatosis with polyangitis (EGPA).Methods:The clinical data of 146 EGPA patients hospitalized in Peking Union Medical College Hospital from 2000 to 2019 were analyzed retrospectively, including clinical manifestations, laboratory results, treatment, complications and outcome at discharge. Birmingham Vasculitis activity score-V3 (BVAS-V3) was used to evaluate disease activity.Results:The ratio of male to female was 1.8∶1 with average age (41.7±16.1) year-old. The median time from disease onset to diagnosis was 18(6, 60) months (0.5~450). The most common clinical manifestations were lung [121(82.9%)] and nose/paranasal sinuses [119(81.5%)] involvement. The positive rate of anti-neutrophil cytoplasmic antibody (ANCA) was 24.7%, mainly peripheral (P)-ANCA/myeloperoxidase (MPO)-ANCA. Compared with ANCA-negative patients, the ANCA-positive patients had a higher incidence of renal involvement and nervous system involvement (66.7% vs. 20.9%, 80.6% vs. 51.8%, P<0.001), fever and optic neuropathy (66.7% vs. 40.9%,8.3% vs. 0, P<0.05), more active disease [median BVAS-V3 25(18,30)vs. 19(14,24), P=0.001] and more elevated erythrocyte sedimentation rate [40.5(20.5,82.8)mm/1h vs. 25.0(13.3,50.8)mm/1h, P=0.006] and C-reactive protein [37.1(11.8,72.9)mg/L vs.13.5(3.4,66.1)mg/L, P=0.036]. More ANCA-negative patients had pleural effusion (20.9% vs. 5.6%, P<0.04) compared with ANCA-negative patients. Pulmonary infection was the most common complication. A total of 12 EGPA patients (8.2%)achieved remission and 6 patients (4.1%)died or discharged themselves from the hospital. Conclusion:EGPA is a rare small vessel vasculitis. The clinical manifestations and outcomes are heterogenous. The mortality rate of EGPA is high.
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Objective:The aim of this study was to compare the efficacy and safety of iguratimod (IGU) or leflnomide (LEF) in combination with methotrexate (MTX) in patients with active rheumatoid arthritis (RA).Methods:This was a multicenter, randomized, double-blinded, double dummy and controlled clinical trial. Patients with moderate or high active RA were randomized in a 1∶1 ratio to receive IGU+MTX (Group A) or LEF+MTX (Group B) treatment. The efficacy and safety were assessed at week 12, 24 and 52, respectively. The primary endpoint was the American Colleague of Rheumatology 20 (ACR20) response rates at the 52th week. Pearson chi square test and two-way Analysis of Variance (ANOVA) were used to compare the improve- ment of ACR20 and DAS28 at 52 weeks. Pearson chi square test or Fisher exact probability test were used to compare the ACR 20 and ACR70 rate between the two groups after treatment. The measurement data of the two groups were compared by independent sample t-test or nonparametric test. Results:A total of 240 RA patients were enrolled in the present study. As a result, 84.1% and 81.0% of patients achieved ACR20 criteria at the 52th week in Group A and Group B, respectively ( χ2=0.35, P=0.56). And the ACR50/70 response rates, disease activity score 28 (DAS28), simplified disease activity index (SDAI) and the absolute decrease of DAS28 from baseline were not statistically different between the two groups at week 12, 24 and 52. The rates of adverse events were lower in Group A than those in Group B (60.0% vs 79.0%, P<0.01). The elevations of glutamic pyruvic transaminase/glutamic oxalacetic transaminase levels, concomitant use of hepatinica and white blood cell decrease were more common in Group B ( P<0.05). Conclusion:IGU in combination with MTX is an efficacious and safe treatment regimen, which is comparable in efficacy in control active RA but superior in safety to LEF combined with MTX.
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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 investigate the clinical characteristics of polyarteritis nodosa (PAN) patients with renal involvement.@*Methods@#PAN patients admitted to the department of rheumatology, department of pediatrics, department of nephrology, general internal medicine department and department of vascular surgery at Peking Union Medical College Hospital from June 2012 to August 2018 were enrolled in this study and were divided into two groups according to renal involvement or not. The clinical characteristics were analyzed.@*Results@#A total of 94 PAN patients were finally enrolled and 57 (60.64%) presented kidney manifestation. The mean age of onset was (37.76±17.40) years old and the interval from onset to diagnosis was 10 (0 to 240) months. Forty patients were misdiagnosed once or more times. In patients with renal involvement, 9 cases suffered from renal ischemia or infarction, 31 with microscopic haematuria, 26 with proteinuria, renal artery or its branch involved in 17 cases, renal vein thrombosis in 1 case, 4 cases with pyeloureterectasis, one case with renal fascia thickening, 33 cases with impaired renal function (serum creatinine>84 μmol/L) including creatinine>140 μmol/L in 10 patients. Renal artery branch stenosis was the most common presentation [9 cases (52.94%)] of renal vascular involvement, other abnormalities including nodular dilatation [4 cases (23.53%)], occlusion [3 cases (17.65%)]. There were significant differences (P<0.05) in the PAN patients with and without renal involvement in the following: age of onset [(33.72±16.13) years vs. (43.97±17.66) years, t2=2.901, P=0.005], weight loss(≥4kg since PAN onset) [25(43.86%) vs. 7(18.92%), χ2=6.216, P=0.013], elevation of diastolic blood pressure [22(38.60%) vs. 7(18.92%), χ2=4.072, P=0.044], acromegaly gangrene [18(31.58%) vs. 21(56.76%), χ2=5.859, P=0.015], and gastrointestinal artery involvement [20(35.09%) vs. 6(1.22%), χ2=3.993, P=0.046]. Laboratory parameters and the application of glucocorticoid and cyclophosphamide therapies were similar in two groups (all P>0.05).@*Conclusion@#Young PAN patients are more likely to be associated with renal involvement, especially gastrointestinal arteries.
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Objective To investigate the clinical characteristics of polyarteritis nodosa (PAN) patients with renal involvement. Methods PAN patients admitted to the department of rheumatology, department of pediatrics, department of nephrology, general internal medicine department and department of vascular surgery at Peking Union Medical College Hospital from June 2012 to August 2018 were enrolled in this study and were divided into two groups according to renal involvement or not. The clinical characteristics were analyzed. Results A total of 94 PAN patients were finally enrolled and 57 (60.64%) presented kidney manifestation. The mean age of onset was (37.76±17.40) years old and the interval from onset to diagnosis was 10 (0 to 240) months. Forty patients were misdiagnosed once or more times. In patients with renal involvement, 9 cases suffered from renal ischemia or infarction, 31 with microscopic haematuria, 26 with proteinuria, renal artery or its branch involved in 17 cases, renal vein thrombosis in 1 case, 4 cases with pyeloureterectasis, one case with renal fascia thickening, 33 cases with impaired renal function (serum creatinine>84 μmol/L) including creatinine>140 μmol/L in 10 patients. Renal artery branch stenosis was the most common presentation [9 cases (52.94%)] of renal vascular involvement, other abnormalities including nodular dilatation [4 cases (23.53%)], occlusion [3 cases (17.65%)]. There were significant differences (P<0.05) in the PAN patients with and without renal involvement in the following: age of onset [(33.72±16.13) years vs. (43.97±17.66)years, t2=2.901, P=0.005], weight loss(≥4kg since PAN onset) [25(43.86%) vs. 7(18.92%), χ2=6.216, P=0.013], elevation of diastolic blood pressure [22(38.60%) vs. 7 (18.92%), χ2=4.072, P=0.044], acromegaly gangrene [18(31.58%) vs. 21(56.76%), χ2=5.859, P=0.015], and gastrointestinal artery involvement [20(35.09%) vs. 6(1.22%), χ2=3.993, P=0.046]. Laboratory parameters and the application of glucocorticoid and cyclophosphamide therapies were similar in two groups (all P>0.05). Conclusion Young PAN patients are more likely to be associated with renal involvement, especially gastrointestinal arteries.
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Objective To investigate the clinical characteristics of gastrointestinal involvement in polyarteritis nodosa (PAN),and to improve the understanding of the disease.Methods PAN patients hospitalized in Peking Union Medical College Hospital from March 2002 to September 2016 were enrolled in this study,and were divided into gastrointestinal involvement group and non-gastrointestinal involvement group according to clinical manifestations and imaging findings.Data on clinical features,treatments and outcome were recorded.t test,chi-square test were used for statistical analysis.Results A total of 117 patients with PAN were hospitalized in the past 14 years.The prevalence of gastrointestinal involvement was 38％(44 cases).There was no significant difference in age and sex between the two groups (P＞0.05).Abdominal pain (29 cases,66％) was the most frequent manifestation,then gastrointestinal bleeding (10 cases,23％),splenic infarction (3 cases,7％),gastrointestinal ulcers (2 cases,5％),intestinal obstruction or diarrhea (each 2 cases,5％),and vomiting (1 case,2％).Patients with gastrointestinal involvement had more frequent fatigue (27％ vs 11％;x2=5.156,P=0.023),increased diastolic pressure (55％ vs 34％;x2=4.647,P=0.031),renal (34％ vs 18％;x2=3.998,P=0.046) and cardiac (25％ vs 8％;x2=6.225,P=0.013) involvements.ESR in the gastrointestinal involvement group was significantly higher (75％ vs 56％;x2=4.190,P=0.041).The average follow-up time was 315.8 (20.3,441.3) days,the relapse rate was higher in the gastrointestinal involvement group (23％ vs 8％;x2=4.895,P=0.027).The incidence of death or the irreversible organ injury was higher in the gastrointestinal involvement group (27％ vs 11％,x2=5.156,P=0.023).Conclusion Gastrointestinal invol-vement in poly-arteritis nodosa is common and its condition is severe.The incidence of relapse and death or irreversible organ injury is high.
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Objective The aim of this study was to evaluate the long-term efficacy and safety of tocilizumab (TCZ) in Chinese patients with Takayasu's arteritis (TA).Methods We retrospectively studied the active TA patients treated with TCZ from January 2014 to April 2016 in our hospital.The demographic and clinical data,laboratory tests and vascular images were collected.Doppler ultrasonography was used to track vascular changes during TCZ treatment.The data were analyzed by t test and rank sum test.The data were analyzed by Fisher exact test or Chi square test.Results Eighteen patients with a median age of 25.5 (range 18,47) were analyzed.Seventeen patients had taken a median of 3 (range 1,5) immune suppressants before TCZ therapy.Three patients withdrew TCZ after receiving 1 infusion each due to unbearable neck pain.The other 15 patients were treated with TCZ for a median of 13 (range 6,26) months.After TCZ treatment,the median ESR level,hsCRP level,mural thickness of common carotid artery and subclavical artery decreased from 45.5 (range 7-101) mm/1 h (Z=6.731,P＜0.01),37.40 (range 7.6-163.12) mg/L,0.25 (range 0.06-0.59) cm,0.18 (range 0.07-0.47) cm to 7 (range 1-44) mm/l h (Z=6.731,P＜0.01),0.94 (range 0.08-93.21) mg/L (Z=3.212,P=0.016),0.18 (range 0.04-0.53) cm (Z=4.873,P=0.001),and 0.12 (range 0.07-0.18) cm (Z=5.568,P＜ 0.01) respectively,with median glucocorticoid dosage being successfully tapered.One patient discontinued TCZ for unsatisfactory response after 6 TCZ infusions.One episode of urinary infection was recorded and relieved after antibiotic therapy.Neither neutropenia nor abnormal liver enzyme was observed.Conclusion Our study suggests that tocilizumab is a safe and effective agent for long term use among Chinese TA patients,especially for those refractory cases.
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Objective To investigate the value of antineutrophil cytoplasmic antibody (ANCA) in clinical phenotype of eosinophilic granulomatosis with polyangiitis (EGPA).Methods The clinical data of 64 patients with EGPA from Peking Union Medical College Hospital between 2007 to 2016 were retrospectively analyzed,and the patients were followed up.Characteristics of patients with ANCA positive and ANCA negative were compared by independent-samples t test,Mann-Whitney U test and Chi-square test.Results Among 64 patients with EGPA,12(19％) were serum ANCA positive and 52(81％) were negative.The incidence of fever (77％ vs 35％,x2=9.403,P=0.002) and renal involvement,including proteinuria (67％ vs 25％,x2=7.678,P=0.006),hematuria (58％ vs 8％,x2=17.57,P＜0.01),renal inadequacy (33％ vs 4％,x2=9.978,P=0.002),and the BVAS score higher than 15 (92％ vs 60％,x2=4.440,P=0.035) in ANCA positive group were higher than ANCA negative group,while the presence of allergic rhinitis (17％ vs 56％,x2=5.969,P=0.015),mucocutaneous lesion (33％ vs 65％,x2=4.152,P=0.042) and cardiac involvement (8％ vs 44％,x2=3.361,P=0.021) in the ANCA-positive group was lower when compared with ANCA-negative patients.The positive ratio of rheumatoid factor (RF) (100％ vs 42％,x2=7.723,P=0.006),and the level of erythrocyte sedimentation rate (ESR) (50 vs 35.5 mm/1 h,P=0.034) in ANCA-positive group were higher than in ANCA negative group.There was no significant difference in pathological characteristics between the two groups.According to the treatment and prognosis,there were no significant differences between the two groups in the usage and dosage of steroids and immunosuppressant,the remission rate and recurrence rate of the disease,and the death rate due to the primary disease.Conclusion The clinical manifestations of EGPA are complicate.Whether ANCA is positive or not may be related to the clinical phenotypes.More attention should be paid to renal involvement in ANCA positive patients while cardiac involvement in ANCA negative patients.