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Objective:To investigate the clinical characteristics of dermatomyositis (DM) patients with positive anti-melanoma differentiation-associated protein 5 (MDA5) antibody-complicated with pneumomediastinum.Methods:Clinical data of patients with anti-MDA5 antibody-positive DM with or without pneumomediastinum from March 2017 to December 2019 in Ningbo First Hospital were collected and analyzed. The international literature were reviewed and compared. T-test or Mann-Whitney U test was used for measurement data, chi-square test or Fisher exact probability was used for count data. Logistic regression analysis was used to analyze the risk factors for anti-MDA5 antibody-positive DM with pneumomediastinum. Results:Twelve DM patients with -positive anti-MDA5 antibody without pneumomediastinum, and 1 DM patient with positive anti-MDA5 antibody-complicated with pneumomediastinum. Pooling with literature review, 16 DM patients with positive anti-MDA5 antibody-complicated with pneumomediastinum were compared. It was found that the serum ferritin (SF) level [991.6(548.5, 2875.1) ng/ml vs 355 (143.5, 395) ng/ml, Z=-2.506, P=0.012] and the rate of rapid progressive pulmonary interstitial disease (RPILD) in the pneumo-mediastinum group [76.5% vs 16.7%, χ2=10.076, P=0.002] were significantly higher than those in the non-pneumome-diastinum group. Further Logi-stic regression analysis did not show male gender [ OR=0.192, 95% CI(0.009, 4.125), P=0.291]; SF [ OR=1.002, 95% CI(0.998, 1.006), P=0.279]; RPILD[ OR=0.084, 95% CI(0.003, 2.178), P=0.136]; CADM[ OR=0.258, 95% CI(0.009, 7.419), P=0.429] was risk factor for pneumomediastinum. Conclusion:DM patients with positive anti-MDA5 antibody and high seral SF level and rapid progression of pulmonary interstitial disease are more likely to complicate with pneumomediastinum.
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Objective To evaluate the efficacy and safety of hydroxychloroquine sulfate (HCQ) on pregnancy outcomes in patients with systemic lupus erythematosus (SLE). Methods One hundred and sixty-six pregnant patients with SLE from Janurary 2010 to December 2015 were studied retrospectively . Fifty-two patients were excluded due to new-onset during pregnancy, active disease or termination of pregnancy as a result of continuous intaking of immunosuppressant. The remaining 114 SLE patients in stable condition before pregnancy were divided into the following two groups: prednisone combined with HCQ and prednisone alone. The effects of HCQ on disease activity and pregnancy outcome were analyzed. Differences between groups were analyzed by chi-square test. Results A total of 90 patients (78.9%) had successful pregnancy. Among 71 patients treated with prednisone combined with HCQ, 60 patients (84.5%) had no disease flare and 62 cases (87.3%) had successful pregnancy. Among 43 patients treated with prednisone alone, 28 patients (65.1%) had no disease flare and 28 cases (65.1%) had successful pregnancy. No abnormality of neither visual field nor fundus was observed among patients treated with HCQ. No congenital abnormalities were found among new born infants. Conclusion HCQ intake during pregnancy in SLE patients can reduce disease flare and improve the pregnancy outcome, indicating that HCQ is safe for SLE patients during pregnancy.
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Objective To evaluate the clinical significance of serum level of vascular endothelial growth factor (VEGF),angiopoietin (Ang)-1 and Ang-2 in patients with rheumatoid arthritis (RA).Methods Serum levels of VEGF,Ang-1 and Ang-2 were measured with enzyme linked immunosorbent assay (ELISA).Twenty-one healthy subjects,24 osteoarthritis patients and 82 rheumatoid arthritis patients were included.We defined active and inactive group according to RA disease active score,while early active RA and late active RA group were defined on the basis of disease course.There were 28 early active patients,32 late active patients and 22 inactive patients with rheumatoid arthritis.At the same time 29 RA patients were examined with ultrasound.Synovial hypertrophy (US joint count SH,US index SH),synovial fluid(US joint count SF,US index SF),resistance index and power Doppler signal (US joint PD,TSS) were scored.The correlation was analyzed.We also detected the serum levels of VEGF,Ang-1,Ang-2,ESR,CRP and DAS28 in 25patients with active RA after 3 month regular treatment.We used one-way ANOVA to compare the differences between groups,and Wilcoxon test to compare the differences between before and after treatment.We analyzed the correlation with linear correlation or Spearman rank test.Results The serum level of VEGF [(1285 ±272) pg/ml],Ang-1 [(0.55±0.25)ng/ml] in patients with rheumatoid arthritis were higher than osteoarthritis patients [(934±80) pg/ml,(0.32±0.16) ng/ml] and normal controls [(565±115) pg/ml,(0.24±0.21) ng/ml],and the serum level of Ang-2 [(1.36±0.40) ng/ml] was higher than normal controls [(0.52±0.32) ng/ml].The serum level of VEGF [(1355±194) pg/ml] in early active patients was higher than late active patients [(1096±477) pg/ml] and inactive patients [(862±91) pg/ml].The serum level of Ang-1 in early active patients,late active patients and inactive patients with rheumatoid arthritis had no statistically significant differences.The serum level of Ang-2 in inactive patients [(2.0±2.0) ng/ml] was significantly higher than late active patients [0.9±0.8) ng/ml].The serum level of VEGF was positively correlated with US joint SH,US index SH,US joint PD,and TSS.The serum level of Ang-1 was positively correlated with US joint SH,US joint PD,and TSS.The serum level of VEGF and Ang-1 were negatively correlated with RI.The serum level of Ang-2 was not correlated with US joint SH,US index SH,US joint SF,US index SF,US joint PD,TSS and RI.In the active RA patients,the serum level of VEGF,Ang-1 and Ang-2 was positively correlated with each other.In the inactive RA patients,the serum level of VEGF,Ang-1 and Ang-2 was not correlated with each other.The serum level of VEGF and Ang-1 before treatment was slightly higher than that of after treatment,but the difference was not statistically significant.The serum level of Ang-2 after treatment was significantly higher than that before treatment.ESR,CRP and DAS28 of after treatment were lower than those before treatment.Conclusion The serum VEGF and Ang-1 level could be used as useful index to reflect RA synovial thickening and angiogenesis The serum level of Ang-2 could be used as one of the efficacy indices.They may influence each other,and they may be the key factors that mediate the onset and development of RA angiogenesis and synovial inflammation.
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Objective To investigate the clinical efficacy and safety of low-dose rituximab (RTX) for patients in primary Sj(..o)gren's syndrome (pSS) with thrombocytopenia.Methods Four pSS patients,2 with refractory thrombocytopenia and 2 with glucocorticoid-dependent thrombocytopenia,were treated with rituximab at 100 mg,intravenous,weekly for a total of two cycles,together with prednisone 1-2 mg · kg-1 ·d-1,and the counts of platelets and B-cells were evaluated.Results Efficacy of treatment was observed in all patients.The counts of platelets,at (3-39) x 109/L baseline,incleased in 1-2 weeks,and went up to ( 107-241 ) × 109/L in 3-8 weeks.Sustained remission had been achieved for 27-52 weeks.The doses of prednisone were tappered to 3.75-7.50 mg/day in 12 weeks.One patient who relapsed at the 27th week (platelet count 47 x 109/L),was retreated with 100 mg of RTX and still had good efficacy.The counts of B-cells reduced to (0.007-0.010) × 109/L,but they did not achieved the depletion.There were no severe adverse events during RTX therapy.Conclusions Our study has shown good efficacy and tolerability of lowdose RTX for pSS with thrombocytopenia.Low-dose RTX allows for reduction in corticosteroid doses and B-cells,while large-scale randomized double-blind controlled trials are needed to confirm the results.
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Objective To investigate the clinical characteristics of bronchopulmonary amyloidosis secondmy to primary Sj(0)gren's syndrome (pSS).Methods One patient in our series and 42 patients in the literatures were analyzed.The clinical manifestations,imaging features,diagnosis,treatments and prognosis of these patients were described respectively.Results Among the 43 cases,42 patients were female (98％).The median age was 57 (range 29-79) years.The diagnosis of bronchopulmonary amyloidosis was made subsequently to that of pSS with a median delay of 8.9 (range 0-30) years.Thirty-eight cases (88％) were localized amyloidosis.Most cases were related to AL amyloidosis (21/28,75％ ).The main clinical manifestations included cough (18/38,47％),short of breath (13/38,34％ ),sputum (9/38,24％) and hemoptysis (5/38,13％ ).Nine patients (9/38,24％ ) had no clinical symptoms.The most common patterns of radiological manifestations included multiple nodules (40/43,93％),multiple cysts or bullae (16/43,37％),interstitial lung disease (16/43,37％ ),irregular luminal narrowing and airway wall thickening (8/43,19％ ).The pulmonary function test was done in 17 patients,which revealed moderate to severe reduced diffusion capacity for carbon monoxide (8/17,47％ ).The diagnosis of amyloidosis was made based on pathological findings in all cases.Pathologic examination showed diffuse deposits of amorphous,eosinophilic,Congo-red positive staining material.The treatments were symptomatic.The prognosis of most patients was good.The median follow-up time was 26.5 (range 2-96) months,only 2 patients died during the follow-up.Conclusion The bronchopulmonary amyloidosis secondary to pSS is localized amyloidosis in most cases.Clinical manifestations depend on the location and extent of airway lesions.The appearance of multiple lung nodules with calcified or cysts in chest images should be considered as secondary amyloidosis.No specific therapy is available for these cases,however,this condition in majority of patients progresses slowly.