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Objective@#To observe the long-term efficacy and safety of autologous hematopoietic stem cell transplantation (AHSCT) for systemic sclerosis (SSc) patients.@*Methods@#Between May 2007 and June 2009,4 patients with SSc were enrolled in the study. Peripheral blood stem cells were mobilized with cyclopho-sphamide (CTX) followed by granulocyte colony stimulating factor (G-CSF). Conditioning was performed with i.v. cyclophosphamide 50 mg·kg-1·d-1 for 4 days. The results of the modified Rodnan skin score (mRSS), thoracic high-resolution computer tomography and pulmonary function were collected after transplantation.@*Results@#There was an improvement in mRSS, lung function and HRCT in the six months after AHSCT. Within six month to one year after transplantation, one patient had sustained and two patients recurred. After active treatments two patients were improved again. During the follow-up of 8.7 (4.1-9.8) years, three patients were stable and one patient died. Infection and hepatic function injury were the major complications. There was not transplant-related mortality.@*Conclusion@#AHSCT with CTX as a pre-conditioning regimen is safe and effective for SSc. The efficacy for patients with short course, rapid progress and edema is significant. However, long-term efficacy is poor, and long-term maintenance treatment is needed.
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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 observe the long-term efficacy and safety of autologous hematopoietic stem cell transplantation (AHSCT) for systemic sclerosis (SSc) patients.Methods Between May 2007 and June 2009,4 patients with SSc were enrolled in the study.Peripheral blood stem cells were mobilized with cyclophosphamide (CTX) followed by granulocyte colony stimulating factor (G-CSF).Conditioning was performed with i.v.cyclophosphamide 50 mg ·kg-1 ·d-1 for 4 days.The results of the modified Rodnan skin score (mRSS),thoracic high-resolution computer tomography and pulmonary function were collected after transplantation.Results There was an improvement in mRSS,lung function and HRCT in the six months after AHSCT.Within six month to one year after transplantation,one patient had sustained and two patients recurred.After active treatments two patients were improved again.During the follow-up of 8.7 (4.1-9.8) years,three patients were stable and one patient died.Infection and hepatic function injury were the major complications.There was not transplant-related mortality.Conclusion AHSCT with CTX as a pre-conditioning regimen is safe and effective for SSc.The efficacy for patients with short course,rapid progress and edema is significant.However,long-term efficacy is poor,and long-term maintenance treatment is needed.
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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 To observe the efficacy and safety of rituximab in the treatment of systemic sclerosis (SSc).Methods This was a prospective,non-randomized controlled trial.All patients with SSc were hospitalized from January 2011 to August 2015.Fifty-two patients were enrolled,including 15 patients in the intervention group and 37 patients in the control group.Both groups were given cyclophos-phamide and prednisone.The intervention group was also given rituximab 375 mg/m2,once every 4 weeks,a total of 4 treatments.The erythrocyte sedimentation rate (ESR),C-reactive protein (CRP),intedeukin 6 (IL-6),modified Rodnan skin score (mRSS),HRCT,forced vital capacity (FVC％),1％ forced expiratory volume percentage (FEV 1％),percentage of carbon monoxide dispersion percentage (DLCO％) were assessed alternatively before treatment,6 months and 12 months after treatment.The repeated measures analysis of variance and t-test were used for statistical analysis.Results Both groups showed improvement in ESR,CRP,IL-6,mRSS,HRCT,FVC％,FEV1％ and DLCO％ (P＜0.05),but ESR [6 month after treatment (33±9) mm/1 h,12 month after treatment (20±4) mm/1 h],CRP [6 month after treatment (12.7±3.4) mg/L,12 months after treatment (12.7± 3.4) mg/L],IL-6 [6 month after treatment (73±10) pg/ml,12 month after treatment (57±11) pg/ml],mRSS [6 months after treatment (22.2±1.3),12 month after treatment (18.4±3.1)],HRCT score [6 month after treatment (11.9±1.4),12 month after treatment (11.3±1.0)],in patients of the intervention group were decreased more significantly than patients of the control group (P＜0.05),and FVC％ [6 month after treatment (69.0± 3.4)％,12 month after treatment (77.2±4.1)％],FEV1％[6 month after treatment (73.3±3.4)％,12 month after treatment (78.4±1.6)％] and DLCO％ [6 month after treatment (60.6±2.7)％,12 month after treatment (70.7± 3.0)％] were increased more significantly than patients of the control group (P＜0.05).It showed that the efficacy of the intervention group was better than the control group.There was no infusion reactions,infection,hepatitis B virus reactivation and other side effects in the treatment were observed.Conclusion Rituximab can reduce the level of inflammation in patients with SSc and improve skin sclerosis and lung function.Rituximab combined cyclophosphamide is expected to be a new,safe and effective treatment of SSc.
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Objective To observe the influence of technetium [99Tc] methylenedipho-honate (99Tc-MDP) on osteoclastogenesis induced by receptor activator of NF-κB ligand (RANKL) and macrophage-colony stimulating factor (M-CSF)in peripheral blood mononuclear cells in patients with rheumatoid arthritis, and to study the mechanism of 99Tc-MDP in osteoclast differentiation. Methods The monocytes/macrophages were isolated from peripheral blood in patients with rheumatoid arthri-tis, incubated in RPMI-1640 with receptor activator of NF-κB ligand (RANKL, 25 μg/L), macro-phage-colony stimulating factor (M-CSF, 25μg/L ) and different concentrations of 99Tc-MDP (5, 10, 20,and 50 mg/L) for 4,12, and 20 days. Tartrate resistant acid phosphatase staining was used to observe the formation of osteoclasts. Results After 12 or 16 days culture of peripheral blood mononuclear cells, plenty of large nultinuclear cells could be found on the coverslips. 99Tc-MDP markedly inhibited those changes and the inhibitory effects were increased as the concentration of 99Tc-MDP increased (P<0.05). Conclusion 99Tc-MDP probably has some protective effect on rheumatoid arthritis by inhibiting osteoclast formation.