Interstitial lung disease in dermatomyositis complicated by right ventricular thrombus secondary to macrophage activation syndrome- a case report

Paul, Namrata; Avalos, Cesar; Estifan, Elias; Swyden, Shelbi

doi:10.21037/acr.2020.03.06

Cited by 4 publications

(2 citation statements)

References 21 publications

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“…Another concern is that macrophage activation syndrome is caused by phagocytosis of hematopoietic components by activated macrophages. Macrophage activation syndrome is more common in RA and SLE; however, it has also been reported in some cases of DM [ 83 , 84 ]. The above reports combined with our results have illustrated that macrophage infiltration is vital in the occurrence and progression of PM/DM.…”

Section: Discussionmentioning

confidence: 99%

Identification of hub biomarkers and immune cell infiltration in polymyositis and dermatomyositis

Chen

Zhan

et al. 2022

Aging

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Objective: Polymyositis (PM) and dermatomyositis (DM) are heterogeneous disorders. However, the etiology of PM/DM development has not been thoroughly clarified. Methods: Gene expression data of PM/DM were obtained from Gene Expression Omnibus. We used robust rank aggregation (RRA) to identify differentially expressed genes (DEGs). Gene Ontology functional enrichment and pathway analyses were used to investigate potential functions of the DEGs. Weighted gene co-expression network analysis (WGCNA) was used to establish a gene co-expression network. CIBERSORT was utilized to analyze the pattern of immune cell infiltration in PM/DM. Protein–protein interaction (PPI) network, Venn, and association analyses between core genes and muscle injury were performed to identify hub genes. Receiver operating characteristic analyses were executed to investigate the value of hub genes in the diagnosis of PM/DM, and the results were verified using the microarray dataset GSE48280. Results: Five datasets were included. The RRA integrated analysis identified 82 significant DEGs. Functional enrichment analysis revealed that immune function and the interferon signaling pathway were enriched in PM/DM. WGCNA outcomes identified MEblue and MEturquoise as key target modules in PM/DM. Immune cell infiltration analysis revealed greater macrophage infiltration and lower regulatory T-cell infiltration in PM/DM patients than in healthy controls. PPI network, Venn, and association analyses of muscle injury identified five putative hub genes: TRIM22 , IFI6 , IFITM1 , IFI35 , and IRF9 . Conclusions: Our bioinformatics analysis identified new genetic biomarkers of the pathogenesis of PM/DM. We demonstrated that immune cell infiltration plays a pivotal part in the occurrence of PM/DM.

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Section: Discussionmentioning

confidence: 99%

Identification of hub biomarkers and immune cell infiltration in polymyositis and dermatomyositis

Chen

Zhan

et al. 2022

Aging

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“…We analyzed eight patients identified from the literature. [6][7][8][9][10][11] Their characteristics are summarized in Tables 2 and 3. Together with our cohort, 12 patients were identified, including one juvenile DM (JDM).…”

Section: Related Studiesmentioning

confidence: 99%

Anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis complicated with macrophage activation syndrome

Ding

2022

Therapeutic Advances in Chronic Disease

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Background: Anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis (DM) has low survival rate, whereas macrophage activation syndrome (MAS) is a severe and life-threatening syndrome associated with autoimmune diseases. Their coexistence is very rare. This study aimed to describe the prevalence, clinical characteristics, and outcomes of anti-MDA5 antibodies-positive DM patients complicated with MAS. Methods: In this retrospective study, we enrolled DM patients with anti-MDA5 antibodies, who were hospitalized between 2016 and 2020 and included patients diagnosed with MAS. Results: We identified four (2%) DM patients with anti-MDA5 antibodies. They were females with interstitial lung disease (ILD). The level of aspartate aminotransferase (AST), lactate dehydrogenase (LDH), and ferritin were significantly higher in the MAS group than those without MAS ( p < 0.05). Patients with MAS were significantly more likely to develop a dysphagia ( p = 0.012). Literature review revealed eight similar cases. Together with the present study, we identified 12 patients complicated with ILD. The median age of disease onset was 52 years with a male to female ratio of 1:6. The median duration between DM onset and MAS diagnosis was 3 months. The mortality of MAS in anti-MDA5 antibody-positive DM was 50%. Patients who died were older than those who survived (56.7 years versus 35.5 years; p = 0.015). Conclusions: MAS was rare in anti-MDA5 antibody-positive DM. The higher the level of AST, LDH, and ferritin, the greater the risk of MAS. They were associated with high mortality rates, particularly in older patients.

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Prevalence and Risk Factors of Thromboembolic Events in Dermatomyositis in China: A 10-Year Retrospective Analysis

Li,

Ding,

Shi

et al. 2024

JIR

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Objective Dermatomyositis (DM) is an autoimmune disease characterized by chronic muscle inflammation and weakness. Patients with DM are at an increased risk of thromboembolic events (TEs). This study aimed to investigate the prevalence of TEs in DM and to identify the independent predictors. Methods A total of 543 patients hospitalized for DM within the past 10 years were analyzed retrospectively and compared with patients with DM with and without TEs for demographic, clinical, and laboratory characteristics. The independent predictors were analyzed using multivariate logistic regression analysis. The diagnostic performance was calculated by a receiver operating curve (ROC). Results Twenty-two (4.1%) patients with DM had TEs, including 12 (54.5%) with venous thromboembolism and 10 (45.5%) with arterial thromboembolism. Multivariate logistic regression analysis demonstrated that glucocorticoid therapy (odds ratio (OR)=0.003, 95% confidence interval (CI) 0.00–0.03, P <0.001) was a protective factor for the patients with DM developing TEs, whereas increased D-Dimer (OR=1.885, 95% CI 1.21–2.95, P =0.006) was a risk factor. The combined ROC analysis of glucocorticoid therapy and D-Dimer indicated high diagnostic values in distinguishing patients with both DM and TEs from patients without TEs, with 86.4% sensitivity, 98.9% specificity, and 0.983 area under the ROC curve (95% CI 0.962–1.000, P <0.001). Conclusion Patients with DM who have never received glucocorticoid therapy and have increased D-Dimer (>1.3 mg/L fibrinogen equivalent units) should be screened for TEs.

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Interstitial lung disease in dermatomyositis complicated by right ventricular thrombus secondary to macrophage activation syndrome- a case report

Cited by 4 publications

References 21 publications

Identification of hub biomarkers and immune cell infiltration in polymyositis and dermatomyositis

Identification of hub biomarkers and immune cell infiltration in polymyositis and dermatomyositis

Anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis complicated with macrophage activation syndrome

Prevalence and Risk Factors of Thromboembolic Events in Dermatomyositis in China: A 10-Year Retrospective Analysis

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