Background Anti-melanoma differentiation-associated protein-5 (anti-MDA5) positive patients are characterized by the high mortality rate caused by interstitial lung disease (ILD). We conducted a retrospective study to summarize the clinical features and identify the initial predictors for death in anti-MDA5 positive patients. Methods We designed a retrospective cohort of anti-MDA5 positive patients. The demographic and clinical data recorded on first admission, as well as the outcomes during the first six months follow-up, were collected. Predictors of rapidly progressive ILD (RPILD) and poor outcomes were calculated using logistic regression models and Cox proportional hazard regression models, respectively. Results A total of 90 anti-MDA5 positive patients were included in this study. Eighty-one (90%) patients presented ILD on admission and 35 (38.9%) patients developed RPILD subsequently. During the first six months of follow-up, 22 (24.4%) patients died of respiratory failure at an average time of 6.6 ± 5.9 weeks. Factors including disease duration < 2 months (OR 6.1, 95% CI 1.7–22.4, P = 0.007), serum ferritin ≥ 1500 ng/ml (OR 12.3, 95% CI 3.1–49.6, P < 0.001), CRP ≥ 13 mg/L (OR 4.6, 95% CI 1.3–16.9, P = 0.021) and total GGO score ≥ 4 (OR 6.3, 95% CI 1.8–21.9, P = 0.003), were identified as independent predictors for RPILD. Cox regression model showed that total CT GGO score ≥ 4 (HR 4.8, 95% CI 1.3–17.9, P = 0.020), KL-6 > 1600 U/ml (HR 3.7, 95% CI 1.5–9.1, P = 0.004) and CRP > 5.8 mg/L (HR 3.7, 95% CI 1.0–12.8, P = 0.044) were poor prognostic risk factors, however initial combined treatment (HR 0.3, 95% CI 0.1–0.8, P = 0.019) predicted good prognosis in anti-MDA5 positive patients. Conclusion Anti-MDA5 positive patients demonstrated a high prevalence of ILD on admission, leading to a high short-term mortality rate. Higher total GGO score, higher levels of initial KL-6 and CRP predict poor outcome in anti-MDA5 positive patients. However, initial intensive treatment may improve the prognosis.

Correlations of carotid intima-media thickness (IMT), atherosclerotic plaque stability, serum inflammatory factors and serum matrix metalloproteinase (MMP)-2 and MMP-9 levels with the condition of disease in patients with acute cerebral infarction were analyzed to explore the predictive value of these risk factors. A total of 56 patients diagnosed with acute cerebral infarction in Jingmen First People's Hospital from February 2016 to January 2017 were selected and divided into the plaque stability group (n=25) and plaque instability group (n=31). Our results showed that the level of total cholesterol (TC) in the plaque instability group was significantly higher than that in the plaque stability group (P<0.05). IMT and National Institutes of Health Stroke Scale (NIHSS) score in the plaque instability group were significantly higher than those in the plaque stability group, but eccentricity index (EI) and Barthel index were significantly lower than those in the plaque stability group (P<0.05). The serum C-reactive protein (CRP), tumor necrosis factor-α (TNF-α) and interleukin-6 (IL-6) levels in the plaque instability group were significantly higher than those in the plaque stability group (P<0.05). The levels of serum MMP-2 and MMP-9 in the plaque instability group were significantly higher than those in the plaque stability group (P<0.05). Barthel index was correlated with IMT (r=−0.693, P<0.01), MMP-2 (r=−0.605, P<0.01), CRP (r=−0.765, P<0.01) and EI (r=0.811, P<0.01), respectively. Hemoglobin A1c (HbA1c), TC, systolic blood pressure, coronary heart disease, diabetes mellitus, IMT, EI, CRP, TNF-α, IL-6, MMP-2 and MMP-9 had independent predictive values for acute cerebral infarction (P<0.05). Carotid IMT, stability of the atherosclerotic plaque, serum inflammation, serum MMP-2 and MMP-9 levels have close correlations with acute cerebral infarction. The larger the carotid IMT is, the more unstable the plaque is and the higher the levels of serum inflammatory factors, MMP-2 and MMP-9 are, the greater the risk of acute cerebral infarction will be.

Background: Anti-melanoma differentiation-associated protein-5 (anti-MDA5) positive patients are characterized by the high mortality rate caused by interstitial lung disease (ILD). We conducted a retrospective study to summarize the clinical features and identify the initial predictors for death in anti-MDA5 positive patients.Methods: We designed a retrospective cohort of anti-MDA5 positive patients. The demographic and clinical data recorded on first admission, as well as the outcomes during the first six months follow-up, were collected. Predictors of rapidly progressive ILD (RPILD) and poor outcomes were calculated using logistic regression models and Cox proportional hazard regression models, respectively.Results: A total of 90 anti-MDA5 positive patients were included in this study. Eighty-one (90%) patients presented ILD on admission and 35 (38.9%) patients developed RPILD subsequently. During the first six months of follow-up, 22 (24.4%) patients died of respiratory failure at an average time of 6.6 ± 5.9 weeks. Factors including disease duration < 2 months (OR 6.1, 95% CI 1.7-22.4, P = 0.007), serum ferritin ≥ 1500 ng/ml (OR 12.3, 95% CI 3.1-49.6, P <0.001), CRP≥13mg/L (OR 4.6, 95% CI 1.3-16.9, P = 0.021) and total GGO score≥4 (OR 6.3, 95% CI 1.8-21.9, P = 0.003), were identified as independent predictors for RPILD. Cox regression model showed that total CT GGO score ≥ 4 (HR 4.8, 95% CI 1.3-17.9, P = 0.020), KL-6 > 1600 U/ml (HR 3.7, 95% CI 1.5-9.1, P = 0.004) and CRP > 5.8 mg/L (HR 3.7, 95% CI 1.0-12.8, P = 0.044) were poor prognostic risk factors, however initial combined treatment (HR 0.3, 95% CI 0.1-0.8, P = 0.019) predicted good prognosis in anti-MDA5 positive patients.Conclusion: Anti-MDA5 positive patients demonstrated a high prevalence of ILD on admission, leading to a high short-term mortality rate. Higher total GGO score, higher levels of initial KL-6 and CRP predict poor outcome in anti-MDA5 positive patients. However, initial intensive treatment may improve the prognosis.

Objective Rapidly progressive interstitial lung disease (RP-ILD) in dermatomyositis (DM) patients positive for anti–melanoma differentiation–associated gene 5 (anti–MDA5) autoantibody (MDA5-DM) often have poor prognosis, frequently fatal. As there is a scarcity of data regarding the effect of intravenous immunoglobulin (IVIG) on RP-ILD in MDA5-DM patients (MDA5-RPILD), we conducted this study to determine the efficacy of a IVIG add-on initial treatment. Methods Patients with newly-onset MDA5-RPILD from September 2018 to June 2020 were retrospectively reviewed for 6 months in the First Affiliated Hospital of Zhengzhou University. They were divided into two groups: IVIG and non-IVIG groups. The major measurement of treatment outcome was the difference in the mortality in 3-month and 6-month between two group patients. Other relevant indicators were also recorded, including the incidence of infection, the dosages of GCs, the remission rate and the variables in laboratory data. Results The IVIG group (n = 31) showed significantly lower 6-month mortality rate than the non-IVIG group (n = 17) (22.6% vs 52.9%; p= 0.033). The IVIG group patients had a higher remission rate at 3 months (71.0% vs 41.2%; p= 0.044). Gradual reduction was observed in the first 3 months with regards to the titer of anti–MDA5 autoantibody, the serum level of ferritin, and the GGO scores. Conclusion IVIG adjunct therapy is a very effective first-line treatment for patients with MDA5-RPILD. IVIG may increase the survival and remission rate by lowering ferritin concentration, anti-MDA5 titer and GGO score.

ObjectiveWe aimed to identify different subtypes of dermatomyositis (DM) patients positive with anti-melanoma differentiationassociated gene 5 antibody (DM-MDA5 + ) for customised treatments to improve the outcomes. Methods Among 96 DM-MDA5 + patients, subgroups with similar phenotypes were delineated using hierarchical clustering analysis of the clinico-biological characteristics. Classification and regression trees were used to build a classification model and survival analysis was used to evaluate the prognoses of subgroups. Results Threesubgroups were identified among 96 DM-MDA5 + patients, and patients in different subgroups had highly heterogenic manifestations and outcomes. Cluster 1 patients were referred to as mild group of rheumatologic patterns with good prognosis. Cluster 2 patients were referred to as young typical DM group with good prognosis. Cluster 3 patients were referred to as elderly rapidly progressive interstitial lung disease (RPILD) group with poor prognosis. A predictive model to classify patients was established, and three critical factors were found, including age, serum ferritin and myalgia. Conclusion DM-MDA5 + patients have a poor short-term prognosis. Three clinical phenotypes with different prognoses were identified in DM-MDA5 + patients.