Objectives We aimed to review whether polymyositis (PM) and dermatomyositis (DM) patients have an increased cardiovascular (CV) risk, including ischaemic heart disease (IHD), cerebrovascular accidents (CVA), and venous thromboembolism (VTE). Methods We searched PubMed, Embase, and the Cochrane database for relevant studies from inception to February 2021. Results Twenty-two studies comprising 25,433 patients were included. With PM/DM versus general populations, the risk was significantly increased for CV events (relative risk (RR)=2.37, 95%CI:1.86-3.02). The RR of CV events for males with PM/DM was higher than for females (RR = 1.43; 95%CI:1.17-1.74). PM/DM patients followed for one to five years had a significantly higher CV risk than those followed for five to ten years (RR = 3.51, 95%CI:1.95-6.32). The risk was increased for North Americans (RR = 4.28, 95%CI:2.57-7.11), Europeans (RR = 2.29, 95%CI:1.58-3.31), and Asians (RR = 2.03, 95%CI:1.41-2.90). Our meta-analysis found that the elevated CV event risk was related to PM (RR = 2.35, 95%CI:1.51-3.66) and DM (RR = 2.55, 95%CI:1.66-3.93). Subgroup analyses showed that the risk was significantly increased for IHD (RR = 1.76, 95%CI:1.40-2.21), CVA morbidity (RR = 1.31, 95%CI:1.03-1.67), and ischaemic stroke (IS) (RR = 1.47, 95%CI:1.26-1.73), with no statistically significant increased risk of haemorrhagic stroke mortality (RR = 1.43, 95%CI:0.92-2.21). The CV event risk was increased for VTE (RR = 4.60, 95%CI:3.17-6.66), deep venous thrombosis (DVT) (RR = 5.53, 95%CI:3.25-9.39), and pulmonary embolism (PE) (RR = 5.26, 95%CI:2.62-10.55). Conclusion This meta-analysis found that PM/DM patients had a ∼2.37 times increased CV risk, particularly males diagnosed in the previous five years. PM/DM may be an independent risk factor for developing IHD, IS, DVT, and PE.
Objectives To evaluate the efficacy and safety of intravenous immunoglobulin (IVIG) in the treatment of dermatomyositis (DM) and polymyositis (PM). Methods A comprehensive systematic review was conducted in accordance with the guidelines of PRISMA (Preferred Reporting Items for Systematic Reviews And Meta-analyses). PubMed, Embase, and China National Knowledge Infrastructure (CNKI) were searched to find articles published between July 1919 and May 2021 concerning IVIG therapy in PM/DM. We analyzed continuum data through mean difference and the estimated pooled improvement rate through Log transformation. We calculated all the effect measures with a 95% confidence interval. The I²statistic was calculated to assess statistical heterogeneity across the studies. I²values of 25%, 50% and 75% were defined as low, moderate and high, respectively. All analyses were conducted using R Studio, Version 3.6.3. Results Seventeen papers pertinent to our questions were found: three case-control studies, fourteen non-randomized studies. We evaluated the efficacy of IVIG in DM/PM by the indicators of creatine kinase (CK), Manual Muscle Test (MMT) scores, Medical Research Council (MRC) scale, the Activities of Daily Living (ADL) scale and the pooled improvement rate. In a meta-analysis, we found that IVIG significantly improved the level of CK (SMD -0.69, 95%CI -0.93, -0.46; P<0.0001), MMT (SMD 1.12; 95%CI 0.77, 1.47; P<0.00001), MRC (SMD 1.59; 95%CI 0.86, 2.33; P<0.00001), ADL (SMD 1.07; 95%CI 0.59, 1.56; P<0.0001). The CK levels in DM and PM were also significantly improved after IVIG (SMD = -0.73, 95%CI -1.12, -0.34; P=0.0002; and SMD = -3.29, 95%CI -5.82, -0.76; P < 0.0001, respectively). The meta-analysis of three RCTs showed that there was a statistically significant improvement after IVIG (SMD 0.63; 95%CI 0.22, 1.03; P=0.002). In a random effects model pooled muscle power improvement rate was 77% (95% CI: 66.0–87.0%). Meta-analyses of IVIG as first-line therapy showed a significant improvement of CK level (SMD -0.71; 95%CI -1.12, -0.30; P=0.0007). In three studies, the polled improvement rate of esophageal disorders was 88% (95% CI: 80.0–95.0%). There was no statistically significant difference in the rate of improvement between the number of courses < 2 and ≥ 2 (0.80 vs. 0.80 %, P = 0.9). The corticosteroid-sparing effect of IVIG was also well demonstrated, with the proportion of corticosteroid-sparing success reaching 81.8% (72/88). Adverse reactions included headache, fever, Hypotension and dizzy and so on. Mild cortical stroke, staphylococcal septicaemia, asymptomatic myocardial infarction, cerebral infarction, deep vein thrombosis and subendocardial ischemia as severe adverse events were found in seven cases. Conclusion IVIG seems to be an effective drug for DM\PM, improving muscle strength, CK levels and esophageal involvement, and it is well tolerated by patients.
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