Dyslipidemia in Juvenile Dermatomyositis.

This study investigates the prevalence of dyslipidemia and its association with disease activity in children with Juvenile Dermatomyositis (JDM). A retrospective chart review of 142 JDM patients who had fasting lipid profiles was conducted. Clinical, and laboratory indicators of disease activity at the time of lipid assessment were obtained. JDM patients displayed a high prevalence (72%) of abnormal or borderline fasting lipid profiles, particularly involving HDL and triglycerides. Treatment-naïve patients exhibited the most significant dyslipidemia, with significantly lower median HDL levels compared to those on medication (30 vs. 49 mg/dL, p < 0.0001). HDL levels inversely correlated with various disease activity measures, including disease activity score (DAS) total (r= -0.38, p < 0.001), DAS muscle weakness (r= -0.5, p < 0.001), DAS skin (r= -0.25, p = 0.003), neopterin (r= -0.41, p < 0.001), ESR (r= -0.25, p = 0.006), and vWF Ag (r= -0.21, p = 0.02). In conclusion, JDM patients have a high prevalence of dyslipidemia, especially low HDL and elevated triglycerides. The severity of dyslipidemia (low HDL) correlates with disease activity, with treatment-naïve patients demonstrating the lowest HDL levels. These findings suggest the importance of annual lipid profile monitoring in JDM patients, potentially followed by early interventions such as dietary adjustments and exercise programs.

Incidence and outcomes for children with idiopathic inflammatory myopathy in Western Australia-a long-term population-based study.

AIM: To determine the incidence and health outcomes for juvenile idiopathic inflammatory myopathy (JIIM) in a long-term whole-population study. METHODS: We included patients under 18 years hospitalized in Western Australia (WA) from 1985 and 2015 with incident JIIM as defined by pertinent diagnostic codes for dermatomyositis (JDM) polymyositis (JPM), other JIIM and overlap myositis (JOM). We compared clinical outcomes and modified Charlson comorbidity scores with age and gender matched (2:1 ratio) patients with new onset juvenile idiopathic arthritis (JIA). Trends over time for annual incidence rate per million child-population (AIR) were analyzed by least square regression and survival by Kaplan-Meier curves. RESULTS: We included 40 patients with JIIM (63% female, median age 8.5 years) for an average AIR of 2.52 per million (CI 1.09-5.57). AIR was stable over time leading to a point prevalence of 52.61 (CI 40.57-67.06) in 2015. Most patients (80%) were classified as JDM with an AIR for JDM of 2.02 (CI 1.09-5.58) and AIR for the combined other JIIM at 0.51 (CI 0.24-1.15). There was female preponderance (62.5%) in both JIIM groups, but no evidence of seasonality. Over a median follow-up of 13 years, one- and ten-year survival was 94.1%. Compared to JIA patients, readmission (80.4 vs. 63.7, p = .02) and infection rates (15.2 vs. 9.6, p < .01) per 100 person-years were higher for JIIM, with similar frequency of interstitial lung disease, fractures, and thrombotic events. At last observation, nearly all patients in both JIIM cohorts (97.5 vs. 92.5%) had accrued some form of comorbidity. CONCLUSIONS: The overall incidence of JIIM leading to hospitalization in WA was stable over 30 years. JIIM prognosis remains suboptimal due to early mortality and accrual of long-term comorbidity.

Hypothyroidism and dermato/polymyositis: a two-sample Mendelian randomization study.

Objective: Observational studies have revealed a higher probability of hypothyroidism in patients with dermatomyositis (DM) or polymyositis (PM), but there is no consensus on whether hypothyroidism causally influences DM or PM. In the present study, we assessed the causal association between hypothyroidism and the risk of dermatomyositis or polymyositis using two-sample Mendelian randomization (TSMR). Methods: The genome-wide association data of hypothyroidism and dermatomyositis/polymyositis were obtained from the IEU Open GWAS project. Then, TSMR was used to determine whether hypothyroidism is causally associated with DM or PM. Single-nucleotide polymorphisms (SNPs) significantly associated with hypothyroidism were identified and used as instrumental variables (IVs), and the causal relationship between hypothyroidism and DM/PM was examined using TSMR. MR pleiotropy and Cochran's Q test were used to confirm the heterogeneity and pleiotropy of identified IVs, then four different models, including the inverse variance weighted model (IVW), MR-Egger, weighted median and weighted model were applied in this MR analysis. Results: Sixty-eight SNPs for DM and 68 SNPs for PM were selected as the IVs (P<5×10-8; linkage disequilibrium R2 <0.001) to assess the causal association between hypothyroidism and DM/PM selected from GWASs on hypothyroidism. The results revealed a positive causal effect of hypothyroidism on both DM and PM (DM: OR 2.563, 95% CI [1.348, 4.874], P = 0.00156; PM: OR1.709, 95% CI [1.157, 2.525], P =0.007). Moreover, there was no heterogeneity or pleiotropy in the results. Conclusion: In conclusion, the MR analysis results provided strong evidence to indicate that hypothyroidism might be causally associated with DM and PM. These findings may have important implications for the pathogenesis and possible future therapies of DM/PM.

Prevalence and Clinical Implications of Autoantibodies in Juvenile Dermatomyositis: A Single-Center Experience From India.

OBJECTIVE: ​​This study aimed to characterize the profile of myositis-specific and myositis-associated autoantibodies (MSAs/MAAs) in an Indian cohort of juvenile dermatomyositis (JDM) patients and correlate them with clinical features and outcomes. METHODS: Forty-three children diagnosed with JDM were enrolled for this observational study. Clinical details (presentation, course, and outcome) were noted in a predesigned proforma. Serum samples were tested for 16 MSAs/MAAs by line immunoassay. MSAs/MAAs were correlated with clinical features and outcome (defined as a complete clinical response [≥6 months' disease inactivity on medication] or complete remission [≥6 months' inactivity off all drugs]). RESULTS: Thirty-five subjects (81.4%) had at least 1 MSA/MAA detected. The most common antibodies were anti-NXP2 (n = 13, 30.2%), anti-TIF1γ (n = 10, 23.2%), and anti-MDA-5 (n = 8, 18.6%). No patient had anti-Ku, anti-Pm Scl-100, anti-PL-12, anti-EJ, anti-OJ, or anti-Ro52. Thirty-two patients (74.4%) attained a complete clinical response over a median follow-up duration of 14 months, among which 6 (13.9%) achieved complete remission over a median follow-up duration of 30 months. Anti-TIF1γ was associated with younger age at onset (≤3 years) (odds ratio [OR], 6.25; 95% confidence interval [CI], 1.15-34.12; p = 0.034) and disease flares after attaining complete response (OR, 10.18; 95% CI, 1.64-70.93; p = 0.013). Patients with anti-NXP2 had higher odds of severe muscular weakness (OR, 3.73; 95% CI, 0.95-14.59; p = 0.058) and truncal weakness (OR, 3.89; 95% CI, 0.97-15.64; p = 0.056). One child with anti-MDA-5 positivity had interstitial lung disease. We found no association between the MSA/MAA profile and the achievement of complete clinical response or remission. CONCLUSIONS: MSAs/MAAs were identified in 81% of children with JDM in our study, which is higher than most other studies. The most frequently observed antibodies displayed a pattern consistent with other studies. Anti-TIF1γ was associated with a younger age at onset and disease flares even after attaining a complete clinical response. Anti-NXP2 had higher odds of severe muscular weakness. These observations suggest consistency in certain phenotypic associations observed across geographic boundaries.

Atherosclerotic cardiovascular disease following a diagnosis of idiopathic inflammatory myopathy: analysis from a retrospective cohort in the TriNetX registry.

OBJECTIVE: Idiopathic inflammatory myopathies (IIM) confer an increased risk of morbidity from atherosclerotic cardiovascular disease (ASCVD). While ASCVD risk has been studied in other countries, these results may not be applicable to patients with dermatomyositis (DM) and polymyositis (PM) in the United States. This retrospective analysis of a cohort of patients identified by ICD code from TriNetX investigated the incidence of ASCVD after International Classification of Disease (ICD) codes of DM, PM, dermatopolymyositis (DPM) or juvenile dermatomyositis (JDM). METHOD: Patients were identified by entry of two ICD codes separated by at least 6 months, according to their first diagnosis code; ASCVD was defined as first ICD code for myocardial infarction, ischemic stroke, transient ischemic attack, or peripheral arterial disease. Cox proportional hazards regression modeled time from first IIM ICD code to ASCVD event. RESULTS: A total of 35,554 patients were identified with the mean age at first IIM code of 54 and 26.1% were male. The most common comorbidity for all groups except JDM was hyperlipidemia (39.9%) though 79.2% of patients were on no cholesterol lowering medication. ASCVD occurred in 30.4% of patients with PM, 24.3% of patients with DM and 0.9% of patients with JDM. Patients with PM had a median time to event of 9.7 years (95% Confidence interval (CI) 9.1, 10.7) and 14.3 years (95% CI 12.6, 14.8) for DM. This study demonstrates that ASCVD is a comorbidity occurring after a median of 12.5 years (95% CI 11.9, 13.6) in patients with IIM. CONCLUSIONS: ASCVD appears to be a long-term complication for IIM patients occurring in nearly a quarter of US patients without prior ASCVD with at least two ICD codes for IIM, with a median time to event of 12.5 years. There appears to be a practice gap in the recognition and treatment of hyperlipidemia in these patients. Key Points • Hyperlipidemia was a common comorbidity identified in patients with IIM though most patients were not on cholesterol lowering medication. • Development of ASCVD appears to be a long-term complication for patients with IIM in the United States.

Exploring the link between inflammatory myopathies and cancer: A comprehensive retrospective analysis in a Colombian cohort.

BACKGROUND: This study investigates the association between inflammatory myopathies (IM), and their correlation with cancer. There are several potential causes behind the association of cancer and inflammatory myopathies. The positivity of specific antibodies for myositis plays a significant role. Our objective is to describe cancer and inflammatory myopathies in Colombia, focusing on demographics, clinical characteristics, and laboratory data. METHODS: We retrospectively analyzed 112 IM patients diagnosed at Fundación Valle del Lili in Cali, Colombia, the cases met the EULAR/ACR criteria. Data included demographics, clinical signs, laboratory findings, and malignancy. Malignancy associations were explored using logistic regression. The survival analysis was assessed using Kaplan-Meier curves and the Log-Rank test. RESULTS: Dermatomyositis was the most common subtype (45.5%), with a female predominance (66.1%). Cancer diagnosis occurred in 11.6% of cases, predominantly thyroid cancer. The median time from myopathy onset to cancer diagnosis was 11 months, with 75% of cases within the first year. Bivariate analysis indicated associations between cancer and age, Gottron's papules, digital ulcers, and heliotrope rash. However, multivariate analysis identified age as the only significant malignancy risk factor. Survival analysis showed better rates in younger patients. CONCLUSION: This study provides into the link between IM and cancer in the Colombian population. Thyroid cancer predominated, with a slightly higher proportion of female cancer diagnoses. Age emerged as a significant risk factor for malignancy. Understanding this association is crucial for early detection and improving patient outcomes related to IM-associated malignancies.

Clinical characteristics and prognostic factor in juvenile dermatomyositis: data of the Spanish registry.

BACKGROUND: Juvenile Dermatomyositis (JDM) is the most common chronic idiopathic inflammatory myopathy in children. The diagnosis is clinical. Baseline laboratory and complementary studies trace the phenotype of these patients. The objective of this study was to describe epidemiological, clinical and laboratory characteristics at diagnosis of JDM patients included in the Spanish JDM registry, as well as to identify prognostic factors on these patients. METHODS: We retrospectively reviewed clinical features, laboratory tests, and complementary studies at diagnosis of JDM patients included on the Spanish JDM registry. These data were analyzed to assess whether there was a relationship with the development of complications and time to disease inactivity. RESULTS: One hundred and sixteen patients from 17 Spanish paediatric rheumatology centres were included, 76 girls (65%). Median age at diagnosis was 7.3 years (Interquartile range (IQR) 4.5-10.2). All patients had pathognomonic skin lesions at the beginning of the disease. Muscle weakness was present in 86.2%. Median Childhood Muscle Assessment Scale was 34 (IQR 22-47). Twelve patients (34%) had dysphagia and 3,5% dysphonia. Anti-p155 was the most frequently detected myositis specific antibody, followed by anti-MDA5. Twenty-nine patients developed calcinosis and 4 presented with macrophage activation syndrome. 70% reached inactivity in a median time of 8.9 months (IQR 4.5-34.8). 41% relapsed after a median time of 14.4 months (IQR 8.6-22.8) of inactivity. Shorter time to treatment was associated with better prognosis (Hazard ratio (HR) = 0.95 per month of evolution, p = 0.02). Heliotrope rash at diagnosis correlates with higher risk of development complications. CONCLUSIONS: We describe heliotrope rash as a risk factor for developing complications in our cohort of JDM patients, an easy-to-evaluate clinical sign that could help us to identify the group of patients we should monitor closely for this complication.

Serum 25-hydroxyvitamin D levels and dermatomyositis: A 2-sample mendelian randomization study.

BACKGROUND: Previous studies have reported low serum 25-hydroxyvitamin D [25(OH)D] levels in dermatomyositis (DM) patients, but the exact causal relationship between them remains elusive. Our aim is to confirm the causal relationship between 25(OH)D and DM risk through a Mendelian randomization study. METHODS: Retrieve genome-wide association study (GWAS) data on 25(OH)D (n = 441 291) and DM (n cases = 201, n controls = 172 834) from the GWAS database (https://gwas.mrcieu.ac.uk/). Select single-nucleotide polymorphisms (SNPs) strongly correlated with 25(OH)D as instrumental variables (IVs). The primary analytical approach involves the use of the inverse-variance weighted method (IVW), supplemented by MR-Egger regression and weighted median methods to enhance the reliability of the results. Heterogeneity and sensitivity analyses were conducted using Cochran's Q and leave-one-out approaches, respectively. RESULTS: The IVW analysis confirmed a positive causal relationship between genetic variation in 25(OH)D levels and DM (OR = 2.36, 95% CI = 1.01-5.52, p = .048). Although not statistically significant (all p > .05), the other methods also suggested a protective effect of 25(OH)D on DM. Based on MR-Egger intercepts and Cochran's Q analysis, the selected SNPs showed no horizontal pleiotropy and heterogeneity. Sensitivity analysis demonstrated the robustness of the results against individual SNPs. CONCLUSION: We provide the first evidence of a causal relationship between 25(OH)D levels and DM. Our findings support the importance of measuring serum 25(OH)D levels and considering vitamin D supplementation in clinical practice for patients with DM.

The prevalence, epidemiological characteristics and mortality trends of inflammatory myopathies patients in Oman: the Prevision study.

OBJECTIVES: This research aims to investigate the prevalence, epidemiological characteristics, mortality rates, survival rates and the rate of malignancy in patients diagnosed with inflammatory myopathies (IIM) in Oman. METHODS: This is a longitudinal study, that covered a span of 16 years at eight rheumatology centres in Oman. The study included all adults and paediatric patients diagnosed with different types of idiopathic inflammatory myopathies (IIM) and who fulfil either the Bohan classification criteria or the 2017 EULAR/ACR classification criteria. RESULTS: The study included a total of 116 patient with an average age of 38.78 (±17.61 SD) years. The most prevalent form of myositis was found to be dermatomyositis (DM) 48 (41.38%), followed by polymyositis (PM) 36 (31.03%) and juvenile myositis (JDM) 18(15.52%). However, inclusion body myositis and necrotising myopathy were relatively rare conditions. The prevalence rates for DM, PM and JDM were determined as 2.2, 2.2, and 1.14 per 100,000 population respectively. Cardiac complications were observed in 14.66% of cases. Among the individuals studied, a history of malignancy was present in around 1.72% of cases. ANA antibodies were present in 71.55% of the cases, anti-Jo 1 and anti-RNP/SM antibodies were detected in 8.62%, and Anti-Ro antibodies in 24.14%. The overall mortality rate was found to be 6.90% with a rate of 11.1% among JDM cases. The five-year survival rates for PM, DM and JDM were found to be 94.4%, 91.7% and 89.0% respectively. These rates decline over a 10-year period to 67%, 69% and 83.3% respectively. CONCLUSIONS: The study highlights the prevalence, mortality, and survival rates of IIM in Oman. Patients with JDM had a higher mortality rate. This underscores the significance of using novel healthcare strategies to improve clinical outcomes and meet special requirements for this group of patients.

Evaluation of the frequency and risk factors of spontaneous intramuscular hemorrhage associated with dermatomyositis.

Dermatomyositis (DM) is an idiopathic inflammatory myositis (IIM) characterized by skin manifestations and muscle involvement. Spontaneous intramuscular hemorrhage (SIH) is a fatal complication that is very rare in the course of DM, but not well known to rheumatologists. Our aim was to determine the frequency and possible risk factors of DM-related SIH. A retrospective analysis was conducted on a cohort of DM patients who were observed in the rheumatology department of the university hospital between 1998 and January 2024. The clinical, laboratory, radiological data of the patients and the treatments they received during the follow-up were analyzed. To determine possible risk factors for the development of SIH in the course of DM, our patients with DM were analyzed together with other rare SIH cases in the literature. The study included 42 of our DM patients. 32 of the patients (76.2%) were female. The median age of the patients was 53 (24-82) years, the median age of DM diagnosis of the patients was 47 (18-75) years, and the median duration of DM of the patients was 36 (2-276) months. 7.1% of patients had dysphagia, and 16.7% had intertitial lung disease (ILD). 5 (11.9%) patients were diagnosed with malignancy. The incidence rate of SIH development in our DM cohort was 0.238/100 patient years (95% CI 0.006-1.256). We tried to identify independent risk factors for SIH development by comparing our 41 DM patients without SIH with the data of patients with 23 DM-related SIH collected from the literature by adding our 1 patient (24 pts). Male sex (OR 4.97, 95% CI 1.66-14.92, p = 0.003), ILD presence (OR 9.71, 95% CI 2.99-31.47, p < 0.001), anti-MDA5 positivity (OR 16.0, 95% CI 1.60-159.3, p = 0.006), anti-Ro52 positivity (OR 11.6, 95% CI 2.93-46.34, p < 0.001), heparin use (OR 4.42, 95% CI 2.68-7.24, p < 0.001), intravenous immunoglobulin (IVIG) use (OR 11.7, 95% CI 2.26-60.54, p < 0.001), and steroid dose (OR 1.03, 95% CI 1.00-1.05, p = 0.005) were identified as risk factors for the development of SIH in the univariate analysis. The death rate due to hemorrhage was 50%. No single risk factor was found to be associated with death. As a result, SIH may occasionally arise in patients with DM. Rheumatologists should be aware that patients with dysphagia and/or ILD, who are on heparin, getting high doses of steroids, and test positive for anti-MDA5 and/or anti-Ro52 antibodies may develop SIH in the early stages of DM.

Dynamic change in red cell distribution width as a predictor for short-time mortality in dermatomyositis-associated rapid progressive interstitial lung disease.

AIM: We aimed to explore a new and readily available practical marker for rapidly progressive interstitial lung disease (RP-ILD) and poor short-term outcomes in patients with idiopathic inflammatory myopathies (IIM). METHODS: A total of 1822 consecutive patients with IIM between 2009 and 2021 were evaluated retrospectively. All proven cases of naïve ILD with complete medical records were included. Red cell distribution width (RDW) values at the initial stage, 3 months and last follow-up were collected. The clinical characteristics and outcomes of the patients were recorded. RESULTS: We identified 532 patients with IIM with an average follow-up of 4 years. ILD prevalence was higher in patients of elevated RDW (p<0.001). The patients with ILD and elevated RDW had lower levels of PaO2/FiO2, FVC% and DLco% and a higher prevalence of RP-ILD than those with normal RDW (p<0.001). Prognostic analysis revealed that RDW was an independent risk factor for prognosis in patients with IIM-ILD (HR=2.9, p=0.03). Patients with dermatomyositis (DM) with RP-ILD with a change in RDW within 3 months (∆RDW-3) greater than 0 were more likely to die within 3 months. Moreover, the prevalence of ∆RDW-3>0 was higher in patients with RP-ILD and positive for anti-melanoma differentiation-associated gene 5 antibody who died within 3 months (87.5%) compared with those alive at 3 months (24.6%) (p<0.001). CONCLUSION: These findings suggest that repeated RDW assays could assist physicians in identifying patients with DM-ILD who were at a high risk of RP-ILD and death.

Prevalence of hearing loss, tinnitus, vertigo and sudden deafness among patients with polymyositis and dermatomyositis.

Little is known about a possible association of autoimmune inner ear disease among patients diagnosed with polymyositis (PM)/dermatomyositis (DM). This study aimed to explore differences in the prevalence of inner ear symptoms among patients with and without PM/DM using a nationwide population-based dataset. Data for this study were retrieved from the Taiwan National Health Insurance Research Database. The study sample included 1622 patients diagnosed with PM/DM and 8109 propensity-score matched comparison patients without PM/DM. We performed multivariate logistic regressions to calculate odds ratios (ORs) and 95% confidence interval (CI) for tinnitus, hearing loss, sudden deafness, and vertigo among patients with PM/DM versus comparison patients. Chi-square tests showed statistically significant differences between patients with PM/DM and comparison patients in the prevalence of tinnitus (16.1% vs. 12.7%, p < 0.001), non-conductive hearing loss (9.2% vs. 6.8%, p < 0.001), and vertigo (14.4% vs. 11.1%, p < 0.001). The adjusted ORs for tinnitus, non-conductive hearing loss, and vertigo, respectively, were 1.332 (95% CI = 1.147-1.547), 1.399 (95% CI = 1.154-1.696), and 1.374 (95% CI = 1.173-1.611) for patients with PM/DM when compared to comparison patients. Our study finds that patients with PM/DM have higher prevalence rates of tinnitus, non-conductive hearing loss, and vertigo than comparison patients.

Nail involvement in connective tissue diseases: an epidemiological, clinical, and dermoscopic study.

BACKGROUND: The assessment of nail changes in connective tissue diseases (CTD) has been rarely explored in previous studies. The use of dermoscopy to study vascular changes in nailfolds is an interesting diagnostic technique. The aim of the study was to describe the epidemiological, clinical, and dermoscopic features of nail lesions in CTD. METHODS: A prospective study was performed at the Dermatology Department of Habib Thameur Hospital (Tunis, Tunisia) in collaboration with the Internal Medicine Department over a period of 15 months, from July 2020 to September 2021, including patients diagnosed with systemic sclerosis (SS), systemic lupus erythematosus (SLE) and dermatomyositis (DM). RESULTS: Our study included 48 patients. Nail involvement was found in 44 cases. Dermoscopic nailfold abnormalities were identified in 37 cases. The most common clinical features were ragged cuticle, nailfold erythema, and onycholysis. Additionally, splinter hemorrhage, longitudinal ridging, lunula abnormalities, melanonychia, trachyonychia, leukonychia, increase in transverse curvature, parrot beak nail, half and half nails, and onychorrhexis were described. Nailfold dermoscopy showed a normal pattern in 10 cases, a nonspecific pattern in nine cases (SLE), and a scleroderma pattern in 29 cases (SS and DM). The scleroderma pattern was further categorized into an early pattern (6), an active pattern (14), and a late pattern (9). Normal pattern was observed solely in patients in remission. The late scleroderma pattern was associated with disease duration and systemic involvement. In SLE, disease activity correlated with onycholysis, nailfold erythema, and pathologic pattern in dermoscopy. However, patients with DM displayed a positive correlation between pulmonary involvement and scleroderma pattern. CONCLUSION: Nail involvement in CTD includes a diverse range of abnormalities. Despite being nonspecific, it can provide crucial clues for establishing a diagnosis. Nailfold dermoscopy serves as a mirror for microangiopathy, enabling the detection of changes at an initial stage, and thus, it becomes a diagnostic and prognostic tool.

Cancer Risk and Mortality in Hospitalized Patients With Idiopathic Inflammatory Myopathies in Western Australia.

OBJECTIVE: To compare cancer incidence, type, and survival between patients with idiopathic inflammatory myopathies (IIMs) in Western Australia (WA) and the general population. METHODS: Administrative health data for hospitalized patients with incident IIM (n = 803, 56.5% female, median age 62.0 yrs), classified by a validated algorithm as polymyositis (PM; 36.2%), dermatomyositis (DM; 27.4%), inclusion body myositis (IBM; 17.1%), overlap myositis (OM; 10.7%), and other IIM (8.6%), were linked to WA cancer and death registries for the period of 1980 to 2014. Cancer incidence rates (CIRs) before and after IIM diagnosis as well as cancer mortality were compared with age-, sex-, and calendar year-matched controls (n = 3225, 54.9% female, median age 64 yrs) by rate ratios (RRs) and Kaplan-Meier survival estimates. RESULTS: The prediagnosis CIR was similar for patients with IIM and controls (6.57 vs 5.95; RR 1.11, 95% CI 0.88-1.39) and for patients evolving to DM (n = 220) or other IIM subtypes (6.59 vs 6.56; RR 1.01, 95% CI 0.38-3.69). During follow-up, CIR was higher for all DM (4.05, 95% CI 3.04-5.29), with increased CIR for lung cancer vs controls (1.05 vs 0.33; RR 3.18, 95% CI 1.71-5.47). Cancer post diagnosis shortened life span by 59 months for patients with IIM (103 vs 162 months, P < 0.01), but reduced survival rates were observed only in patients with DM and IBM. CONCLUSION: Cancer risk was not increased prior to IIM, but CIR for lung cancer was increased following DM diagnosis. As cancer reduced survival only in patients with DM and IBM, these data support a strategy of limited cancer screening in IIM.

A bibliometric analysis of the research status and trends in studies on polymyositis and dermatomyositis with interstitial lung disease from 2000 to 2022 using Web of Science.

BACKGROUND: The main subtypes of idiopathic inflammatory myopathies (IIMs)-polymyositis (PM) and dermatomyositis (DM)-are often presented as interstitial lung disease (ILD) in clinical practice; therefore, many researchers have combined the three studies into PM/DM with ILD. METHODS: Using bibliometrics, the research status, progress, and hotspots of PM/DM with ILD between 2000 and 2022 were studied. Literature data on PM/DM with ILD were retrieved from the Web of Science (WoS) database for the research period. Visualization software, including VOSviewer, Pajek, CiteSpace, and Scimago Graphica were used for bibliometric analysis. RESULTS: A total of 1555 relevant articles were obtained, and the overall research in this field showed an increasing trend. Regarding contributing countries and venues, Japan published the most articles while Rheumatology was the most prolific journal. Regarding authors, the most published article was by Wang Guochun from Changchun University of Technology in China. Keyword analysis and cocited literature cluster analysis showed that diagnosis, classification, autoantibodies, antibodies, prognosis, complications, and treatment of PM/DM with ILD have been hot topics in this field recently. Moreover, our study shows that anti-mda5 antibody, mortality, gene 5 antibody, IIMs, double-blind, and prognostic factors, among others, may be new hot topics. CONCLUSION: This study found that research on PM/DM with ILD has increased over time, and scholars are paying more attention to this field. The development of new drugs for the management, treatment, and prevention of PM/DM with ILD is the primary task of researchers and a direction for future research in this field.

Dermatomyositis in Association With SARS-CoV-2 Infection or COVID-19 Vaccine.

OBJECTIVE: New-onset and relapsed dermatomyositis (DM) has been reported following SARS-CoV-2 infection or COVID-19 vaccination. This study aims to show the characteristics of a DM cohort after COVID-19 infection and vaccination. METHODS: A retrospective review was performed on patients treated for DM between March 1, 2020, and October 31, 2022. Charts were evaluated for the presence of new-onset DM or relapse of preexisting DM following either SARS-CoV-2 infection or COVID-19 vaccination. Data on symptom onset, timing of vaccination, type of vaccination, and disease characteristics were collected. RESULTS: Ninety-eight patients treated for DM at our institution in the Division of Rheumatology were included. In total, 12 of 98 patients (12.2%) experienced DM symptoms (either incident or relapse) following either infection or vaccination. Of the 12 patients who developed incident disease or relapse, 7 (58.3%) developed postinfection symptoms, and 8 (66.7%) developed symptoms after vaccination (3 patients had symptoms following both infection and vaccination). The mean onset of symptoms following COVID-19 infection was 3.2 days (median 0.5 days), and mean onset following COVID-19 vaccination was 5.75 days (median 3.5 days). Nine of 12 patients (75%) had a positive myositis-specific antibody, and the remaining 3 (25%) had myositis-associated antibodies. There was no predominant vaccine associated with the development of postvaccination DM symptoms. CONCLUSION: This retrospective review revealed a strong temporal relationship between DM symptoms and COVID-19 infection or vaccination in 12.2% of all patients with DM evaluated in our clinic during the pandemic. Additional studies are required to understand the possible pathophysiology behind this association.

Calcinosis in Juvenile Dermatomyositis-Epidemiology, Pathogenesis, Clinical Features, and Treatment: A Systematic Review.

PURPOSE OF REVIEW: We performed a systematic review of the literature on the epidemiology, pathogenesis, clinical and laboratory characterization, and treatment of calcinosis in patients with juvenile dermatomyositis (JDM). A qualitative systematic review was conducted from January 1975 to April 2023 according to the PRISMA protocol using three electronic databases: PubMed, Web of Science, and Scopus. Studies were analyzed based on the following eligibility criteria: at least one combination of the terms described in the search strategy appeared in the title, written in English, Portuguese, or Spanish, and addressed the epidemiology, pathogenesis, diagnosis, and treatment of calcinosis in juvenile dermatomyositis. Systematic or scoping reviews, letters, clinical images, book chapters, abstracts, inflammatory myopathy in other connective tissue diseases, idiopathic inflammatory myopathies in adults, and purely qualitative studies were excluded. RECENT FINDINGS: Seventy-five studies were included. According to the literature, calcinosis is common in women, around five years old, with three years of disease in association with osteoarticular, cutaneous, pulmonary manifestations, and fever. The pathogenesis is still unknown, but the participation of interleukin 1 and 6, tumor necrosis factor alpha, and innate immunity dysregulation seem to be involved. Common autoantibodies are anti-NXP-2, anti-MDA-5, and anti-Mi-2, and their treatment remains controversial. Prospective, randomized, controlled studies are needed to evaluate treatment protocols and map the natural history of this serious complication. Calcinosis seems to be more common in White female children with muscle weakness, fever, arthritis, severe pulmonary, and skin involvement with anti-NXP-2, anti-MDA-5, and anti-Mi-2 autoantibodies. The multitargets and aggressive treatment is recommended.

Obesity is an independent risk factor for cancer development following diagnosis of dermatomyositis.

Patients with dermatomyositis (DM) are at an increased risk of cancer development, especially around the time of diagnosis of DM. Obesity is also a risk factor in the general population for cancer development. This study aimed to assess the association between cancer in DM patients with and without obesity as defined by ICD code and BMI data. In this analysis of patients with DM, logistic regression modeling of the odds of cancer outcome was performed for patients with DM and obesity compared to those without obesity, adjusted for age and sex. A total of 12,722 patients with DM were identified, of whom 6,055 had available BMI data. DM patients who were coded obese at any point had significantly higher odds 1.98 (95 % Confidence interval (CI) 1.70, 2.30) of a subsequent cancer diagnosis. This association was also found in the subgroup analysis with available BMI where patients with obesity (BMI ≥30 kg/m2) had an increased odds of cancer 1.23 (1.02, 1.49) when compared to patients with BMI <30 kg/m2 with DM. In time to event analysis any obesity code was associated with a 16 % increased hazard of cancer (adjusted hazard ratio 1.16 [95 % CI 1.02, 1.31]). Overall, the most frequent type of cancer was breast cancer, however patients with DM and obesity had higher frequencies of lymphoma, colorectal, melanoma, uterine, renal cancers compared to patients with DM without obesity.

Gender differences in patients with anti-MDA5-positive dermatomyositis: a cohort study of 251 cases.

OBJECTIVE: To investigate the impact of sex differences on the clinical characteristics and prognosis of patients with anti-melanoma differentiation-associated gene 5-positive dermatomyositis (MDA5+ DM). METHODS: We retrospectively analyzed a cohort of 251 patients with MDA5+ DM, including 71 in the male group and 180 in the female group. A multivariate logistic regression model was built to analyze independent risk factors for RPILD in each group. An ROC curve was drawn to evaluate the predictive value of independent risk factors. Kaplan‒Meier analysis was used to compare the cumulative survival rates, while the log-rank test was used to test for significant differences between the two groups. RESULTS: Patients in the male group had a significantly higher prevalence of heliotrope rash, V sign, severe interstitial lung disease (ILD), and rapidly progressive interstitial lung disease (RPILD) than those in the female group. Anti-Ro52 positivity, high CRP level and short disease were identified as independent risk factors for RPILD in both male and female groups by multivariate logistic regression analysis. The mortality rates of males and females were 33.8% and 22.0%, respectively, and the survival time of patients in the male group was shorter than that in the female group. CONCLUSION: Male patients with MDA5+ DM exhibit an increased risk of RPILD, elevated mortality rates and reduced overall survival time compared to their female counterparts, and anti-Ro52 positivity may be an unfavorable prognostic factor for these patients. Key Points • The prevalence of solar rash, V sign, severe interstitial lung disease (ILD) and rapidly progressive interstitial lung disease (RPILD) in anti-MDA5-positive female patients was significantly lower than that in male patients. • Positive Anti-Ro52, high CRP level, and short course of disease were independent risk factors for RPILD in both men and women. • Female patients exhibited a lower mortality rate than male patients (22.0% vs 33.8%) and demonstrated longer survival time.