Involvement In Idiopathic Inflammatory Myopathies Research Articles

Prognostic significance of natural killer cell depletion in predicting progressive fibrosing interstitial lung disease in idiopathic inflammatory myopathies.

Interstitial lung disease (ILD) is one of the common extramuscular involvement in idiopathic inflammatory myopathies (IIMs) (1). Several patients develop a progressive fibrosing ILD (PF-ILD) despite conventional treatment, resulting in a progressive deterioration in their quality of life (2). Here, we investigated the clinical and immune characteristics of IIM-ILD and risk factors for PF-ILD in IIM, mainly in anti-melanoma differentiation-associated protein 5 (anti-MDA5+) dermatomyositis (DM) and anti-synthetase syndrome (ASS). Here, a prospective cohort of 156 patients with IIM-ILD were included in the longitudinal analysis and divided into the PF-ILD (n=65) and non-PF-ILD (n=91) groups, and their baseline clinical characteristics were compared. Univariate and multivariate Cox analyses were performed to identify the variables significantly associated with pulmonary fibrosis progression in the total cohort, then anti-MDA5+ DM and ASS groups separately. Peripheral blood lymphocyte counts, including T, B, and NK cell counts, were significantly lower in the PF-ILD group than in the non-PF-ILD group. This characteristic is also present in the comparison between patients with anti-MDA5+ DM and ASS. The multivariate Cox regression analysis revealed that age > 43.5 years [HR: 7.653 (95% CI: 2.005-29.204), p = 0.003], absolute NK cell count < 148 cells/μL [HR: 6.277 (95% CI: 1.572-25.067), p = 0.009] and absolute Th cell count < 533.2 cells/μL [HR: 4.703 (95% CI: 1.014-21.821), p = 0.048] were independent predictors of progressive fibrosing during 1-year follow-up for patients with anti-MDA5+ DM, while absolute count of NK cells < 303.3 cells/µL [HR: 19.962 (95% CI: 3.108-128.223), p = 0.002], absolute count of lymphocytes < 1.545×109/L [HR: 9.684 (95% CI: 1.063-88.186), p = 0.044], and ferritin > 259.45 ng/mL [HR: 6 (95% CI: 1.116-32.256), p = 0.037] were independent predictors of PF-ILD for patients with ASS. Patients with anti-MDA5+ DM and ASS have independent risk factors for PF-ILD. Lymphocyte depletion (particularly NK cells) was significantly associated with PF-ILD within 1-year of follow-up for IIM-ILD.

Myositis-associated antibodies predict the severity of lung involvement in adult patients with inflammatory myositis - a cohort study of 70 adult patients with myositis in a single center.

Idiopathic inflammatory myopathies (IIMs) encompass a diverse group of diseases characterized by considerable variability in clinical manifestations, antibody profiles, and responsiveness to immunosuppressive therapies. This study aimed to investigate the association between organ involvement and distinct myositis autoantibodies in individuals with IIM in a single-center cohort. Patients with ICD diagnoses M33.1, M33.2, M33.9, or M609 who (1) had been tested with Euroline blot assay for myositis autoantibodies and (2) met the classification criteria of definite/probable polymyositis (PM) or dermatomyositis (DM), anti-synthetase syndrome (ASS), or inclusion body myositis (IBM) were included. Medical journals were retrospectively examined with respect to clinical disease features. Seventy patients (median age 58 years; 66% females) were included and represented the following diagnosis: PM (n = 23), DM (n = 21), ASS (n = 23), and IBM (n = 3). Most of the patients (87%) presented a muscle biopsy indicative of myositis. The presence of autoantibodies was as follows: myositis-specific antibodies, MSA (n = 53), myositis-associated antibodies, MAA (n = 33), both MSA + MAA (n = 24), MSA only (n = 29), MAA only (n = 9), no MSA, or MAA (n = 8). Anti-Jo-1 was the most common MSA (19%), whereas the most common MAA was anti-Ro/SSA52 (31%). We observed a significant association between antibody patterns and lung disease. In our cohort, 47% of the patients in the whole study group, 86% of patients with anti-SSA52, and 100% with anti-Jo-1 had pulmonary involvement. Patients with both MSA and MAA had a higher incidence of lung disease and decreased CO-diffusion capacity. This was especially prominent in anti-Ro/SSA52-positive patients. Interestingly, none of the patients suffered from lung disease if only antibodies against Mi-2α, Mi-2β, NXP2, HMGCR, and TIF1γ were present or no MSA/MAA were detected. The simultaneous presence of both MAA and MSA indicates an increased risk of lung involvement in patients with inflammatory myopathies. The presence of any MAA, and especially anti-Ro/SSA52, is associated with more severe pulmonary disease. Our data suggest that MAA antibodies might be relevant markers for early detection and treatment of lung involvement in IIM.

Advances in the diagnosis of myocarditis in idiopathic inflammatory myopathies: overview of diagnostic tests.

Cardiac involvement in idiopathic inflammatory myopathies (IIM) purports to worse clinical outcomes and therefore early identification is important. Research has focused on blood biomarkers and basic investigations such as electrocardiography and echocardiography, which have the advantage of wide availability and low cost but are limited in their sensitivity. Imaging the myocardium to directly look for inflammation and scarring has therefore been explored, with a number of new methods for doing this gaining wider research and clinical availability. Cardiovascular magnetic resonance (CMR) with contemporary multiparametric mapping techniques and late gadolinium enhancement imaging, is an extremely valuable and increasingly used non-invasive imaging modality for the diagnosis of myocarditis. The recently updated CMR based Lake Louise Criteria for the diagnosis of myocarditis incorporate the newer T1 and T2 mapping techniques which have greatly improved the diagnostic accuracy of IIM myocarditis.18F-FDG-PET/CT is a well-utilised imaging modality in the diagnosis of malignancies in IIM which also has a role for the diagnosis of myocarditis in multiple systemic inflammatory diseases. Endomyocardial biopsy however remains the gold standard technique for the diagnosis of myocarditis and is necessary for the diagnosis of specific cases of myocarditis. This article provides an overview of the important tests and imaging modalities which clinicians should consider when faced with an IIM patient with potential myocarditis.

Pulmonary involvement in idiopathic inflammatory myopathies

Idiopathic inflammatory myopathies are rare systemic diseases with different types of pulmonary manifestations depending on the underlying aetiology; here, interstitial lung diseases (ILD) are the most frequently found patterns depending on the underlying disorder. There is a lack of sufficient prospective studies on this heterogeneous group of patients, particularly in case of ILD being involved. The diagnosis is based upon guideline recommendations for ILD and requires a multidisciplinary discussion within a team with specific expertise in this field. Myositis specific antibodies and myositis associated antibodies form an essential part of the diagnostic tools and may also be associated with a certain phenotype or disease progression. Anti-t-RNA-synthetase antibodies (Anti-ARS) and anti-melanoma differentiation-associated gene 5 antibodies (MDA5) play an important clinical role for treatment the estimation of response and prognosis. The most common ILD patterns are nonspecific interstitial pneumonia (NSIP) and organising pneumonia (OP) or a mixed pattern of both. Treatment is based on systemic steroids and early initiation of other immunosuppressant drugs. Evidence for this is, however, sparse, since most of the studies having investigated treatment modalities are of retrospective nature, even though some new prospective data may be useful for the establishment of treatment pathways in the future.

The role of neutrophil extracellular traps and proinflammatory damage-associated molecular patterns in idiopathic inflammatory myopathies.

Idiopathic inflammatory myopathies (IIMs) are a group of systemic autoimmune diseases characterized by immune-mediated muscle injury. Abnormal neutrophil extracellular traps (NETs) can be used as a biomarker of IIM disease activity, but the mechanism of NET involvement in IIMs needs to be elucidated. Important components of NETs, including high-mobility group box 1, DNA, histones, extracellular matrix, serum amyloid A, and S100A8/A9, act as damage-associated molecular patterns (DAMPs) to promote inflammation in IIMs. NETs can act on different cells to release large amounts of cytokines and activate the inflammasome, which can subsequently aggravate the inflammatory response. Based on the idea that NETs may be proinflammatory DAMPs of IIMs, we describe the role of NETs, DAMPs, and their interaction in the pathogenesis of IIMs and discuss the possible targeted treatment strategies in IIMs.

The diagnostic value of sST2 for myocardial fibrosis in idiopathic inflammatory myopathies in subclinical stage of cardiac involvement.

Myocardial fibrosis occurs in the early subclinical stage of cardiac involvement in idiopathic inflammatory myopathies (IIMs). Soluble suppression of tumorigenicity 2 (sST2) is known to have an immunomodulatory impact during autoimmune disease development. The current study investigated the diagnostic value of sST2 for myocardial fibrosis during early stage of cardiac involvement in IIM. A total of 44 IIM patients with normal heart function and 32 age- and gender-matched healthy controls (HCs) were enrolled. Serum sST2 levels were measured by ELISA and cardiac magnetic resonance (CMR) parameters for myocardial fibrosis [native T1, extracellular volume (ECV), late-gadolinium enhancement (LGE)] and oedema (T2 values) were analysed. IIM patients had significantly higher sST2 levels than HCs [67.5 ng/ml (s.d. 30.4)] vs 14.4 (5.5), P < 0.001] and levels correlated positively with diffuse myocardial fibrosis parameters, native T1 (r = 0.531, P = 0.000), ECV (r = 0.371, P = 0.013) and focal myocardial fibrosis index and LGE (r = 0.339, P = 0.024) by Spearman's correlation analysis. sST2 was an independent predictive factor for diffuse and focal myocardial fibrosis after adjustment for age, gender, BMI and ESR. Risk increased ≈15.4% for diffuse [odds ratio (OR) 1.154 (95% CI 1.021, 1.305), P = 0.022] and 3.8% for focal [OR 1.038 (95% CI 1.006, 1.072), P = 0.020] myocardial fibrosis per unit increase of sST2. Cut-off values for diagnosing diffuse and focal myocardial fibrosis were sST2 ≥51.3 ng/ml [area under the curve (AUC) = 0.942, sensitivity = 85.7%, specificity = 98.9%, P < 0.001] and 53.3 ng/ml (AUC = 0.753, sensitivity = 87.5%, specificity = 58.3%, P < 0.01), respectively. sST2 showed a marked elevation during the subclinical stage of cardiac involvement in IIM and has potential as a biomarker for predicting diffuse and focal myocardial fibrosis in IIM.

Multimodality Screening For (Peri)Myocarditis In Newly Diagnosed Idiopathic Inflammatory Myopathies: A Cross-Sectional Study.

Cardiac involvement in idiopathic inflammatory myopathy (IIM or "myositis") is associated with an approximate 4% mortality, but standardised screening strategies are lacking. We explored a multimodality screening on potentially reversible cardiac involvement -i.e. active (peri)myocarditis -in newly diagnosed IIM. We included adult IIM patients from 2017 to 2020. At time of diagnosis, patients underwent cardiac evaluation including laboratory biomarkers, electrocardiography, echocardiography, and cardiac magnetic resonance imaging (CMR). Based on 2019 consensus criteria for myocarditis, an adjudication committee made diagnoses of definite, probable, possible or no (peri)myocarditis. We explored diagnostic values of sequentially added diagnostic modalities by Constructing Classification and Regression Tree (CART) analysis in patients with definite/probable versus no (peri)myocarditis. We included 34 IIM patients, in whom diagnoses of definite (six, 18%), probable (two, 6%), possible (11, 32%), or no (peri)myocarditis (15, 44%) were adjudicated. CART-analysis showed high-sensitivity cardiac troponin T (cut-off value < 2.3 times the upper limit of normal (xULN)) ruled out (peri)myocarditis with a sensitivity of 88%, while high-sensitivity troponin I (cut-off value > 2.9 xULN for females and > 1.8 xULN for males) ruled in (peri)myocarditis with a specificity of 100%. Applying high-sensitivity cardiac troponins with these cut-off values in a diagnostic algorithm without and with a CMR to the total population of 34 patients demonstrated a diagnostic accuracy for a clear diagnosis of probable/definite or no (peri)myocarditis of 59% and 68%, respectively. A diagnostic algorithm for detection of (peri)myocarditis in adult IIM may consist of sequential testing with high-sensitivity cardiac troponins and CMR.

The diagnostic value of serum YKL-40 for myocardial involvement in idiopathic inflammatory myopathy

ObjectiveTo investigate the diagnostic value of serum chitinase-3-like-1 protein (YKL-40) levels for myocardial involvement in idiopathic inflammatory myopathies (IIM). MethodsA total of 74 patients with definite IIM who visited Jiangsu Province People's Hospital between May 2018 and January 2022 were enrolled in this retrospective study. Baseline clinical evaluation, laboratory index, electrocardiogram (ECG), echocardiography (ECHO) and cardiac magnetic resonance (CMR) parameters were collected. Serum YKL-40 of all participants was determined by ELISA. Receiver-operating characteristic (ROC) curve was used to assess the diagnostic value of YKL-40 in assessing myocardial involvement in IIM patients. Results1. YKL-40 concentration was significantly higher in IIM patients with myocardial injury than without myocardial injury.2. Multivariate logistic regression analysis demonstrated that serum YKL-40 was an independent risk factor for myocardial involvement in IIM.3. YKL-40 > 66.4 ng/ml (AUC = 0.85, 95 % CI 0.75–0.95) predicted myocardial injury in IIM with a sensitivity of 0.75 and specificity of 0.95. ConclusionSerum YKL-40 could serve as a potential biomarker to predict myocardial injury in IIM patients.

Using multi-parametric quantitative MRI to screen for cardiac involvement in patients with idiopathic inflammatory myopathy

Idiopathic inflammatory myopathies (IIM) is a group of heterogeneous autoimmune systemic diseases, which not only involve skeletal muscle but also myocardium. Cardiac involvement in IIM, which eventually develops into heart failure, is difficult to identify by conventional examinations at early stage. The aim of this study was to investigate if multi-parametric cardiac magnetic resonance (CMR) imaging can screen for early cardiac involvement in IIM, compared with clinical score (Myositis Disease Activity Assessment Tool, MDAAT). Forty-nine patients of IIM, and 25 healthy control subjects with comparable age-range and sex-ratio were enrolled in this study. All subjects underwent CMR examination, and multi-slice short-axis and 4-chamber cine MRI were acquired to evaluate biventricular global circumferential strain (GCS) and global longitudinal strain (GLS). Native T1 and T2 mapping were performed, and post-contrast T1 mapping and LGE were acquired after administration of contrast. A CMR score was developed from native T1 mean and T2 mean for the identification of cardiac involvement in the IIM cohort. Using contingency tables MDAAT and CMR were compared and statistically analyzed using McNemar test. McNemar’s test revealed no significant difference between CMR score and MDAAT (p = 0.454). CMR score had potential to screen for early cardiac involvement in IIM patients, compared to MDAAT.

Associations between anti-mitochondrial antibodies and cardiac involvement in idiopathic inflammatory myopathy patients : Asystematic review and meta-analysis.

The objectives of this study are to analyze the association between anti-mitochondrial antibody (AMA) and cardiac involvement in idiopathic inflammatory myopathy (IIM) and to evaluate the diagnostic value of AMA for cardiac involvement in IIM patients. We conducted acomprehensive search in PubMed, Web of Science, EMBASE, and the Cochrane Library to identify English-language studies published before November19, 2021. Stata12.0 software (Stata Corp., College Station, TX, USA) was used for the statistical analyses. We used the sensitivity, specificity, positive likelihood ratio (PLR), negative likelihood ratio (NLR), and summary receiver operating characteristic (SROC) curve to evaluate the diagnostic value of AMA for cardiac involvement in IIM patients. Statistical heterogeneity of studies was assessed using the I2 statistic with 95% confidence intervals (95% CIs). Seven studies were included in the final analyses, with atotal of 2308IIM patients (including 171 AMA-positive and 2137 AMA-negative patients). The pooled sensitivity of AMA for cardiac involvement in IIM patients was 0.29 (95% CI: 0.19-0.43) and specificity was 0.92 (95% CI: 0.88-0.96). The pooled PLR was 3.9 (95% CI: 2.82-5.38), NLR was 0.76 (95% CI: 0.66-0.88), and the diagnostic odds ratio (DOR) was 5 (95% CI: 3-7). The area under the SROC curve was 0.76 (95% CI: 0.72-0.79). The overall diagnostic value of AMA may not be very high for cardiac involvement in IIM patients.

Defining cardiac involvement in idiopathic inflammatory myopathies: a systematic review.

Recent advances in cardiac MRI (CMR) and other diagnostic techniques have made it easier to identify subclinical cardiac inflammation and dysfunction in the idiopathic inflammatory myopathies (IIM). Herein, we systematically review the literature regarding cardiac involvement in IIM. We searched Medline and EMBASE from 1990 to 2020 using keywords related to IIM and cardiac disease. We included English language studies in adults with any immune-mediated, inflammatory muscle pathology. We identified 10 425 potentially relevant abstracts, of which 29 were included. Most frequently these included patients with PM or DM without symptomatic myocarditis. Five categories of cardiac investigation were used in these patients: cardiac enzyme testing, ECG, transthoracic echocardiography, CMR and nuclear medicine testing. Patients with clinical myocarditis had universally abnormal cardiac troponin levels and ECG. Elevated cardiac troponin T was more common than cardiac troponin I and may correlate with disease activity, whereas cardiac troponin I was more specific for cardiac involvement. Non-specific ECG changes were common. The major finding on transthoracic echocardiography was abnormal ejection fraction. Gross systolic dysfunction was unusual, but subclinical systolic dysfunction was reported in several studies. Abnormal diastolic function was common and may be associated with disease duration. Late gadolinium enhancement (reflecting regional necrosis or scarring) and abnormal myocardial mapping parameters (reflecting myocardial inflammation, fibrosis and oedema) were frequently identified on CMR, suggesting significant subclinical myocardial pathology (despite typically normal ejection fraction). Abnormal cardiac investigations are commonly found in asymptomatic IIM patients, which has potential prognostic and treatment implications.

Myocardial involvement in idiopathic inflammatory myopathies: a multi-center cross-sectional study in the CRDC-MYO Registry.

This study aimed to investigate the associated factors of myocardial involvements (MIs) in patients with idiopathic inflammatory myopathies (IIMs). In this multi-center cross-sectional study, 1946 patients with IIMs were enrolled from Chinese Rheumatism Data Center-Myositis Registry (CRDC-MYO). A total of 108 (5.5%) patients were identified with MIs, including congestive heart failure (n = 67, 62.0%), and severe arrhythmias (n = 61, 56.5%). The other 1838 IIM patients without IMs were set as the control group. Clinical features were collected including age, gender, comorbidities, clinical symptoms, clinical signs of both IIMs and MIs, lab findings including myositis-specific antibodies (MSAs) and myositis-associated antibodies (MAAs), echocardiogram, and radiological exams. Multivariate logistic analysis was used to explore independent associated factors of MIs in patients with IIMs. Several independent associated factors were identified in multi-variate logistic regression, including positivity for anti-mitochondrial antibody-subtype 2 (AMA-M2) (OR 5.194, 95% CI 2.509-10.753, P < 0.001), elevation of creatine kinase (CK) (OR 2.611, 95% CI 1.312-5.198, P = 0.006), elevation of C-reactive protein (CRP) (OR 2.150, 95% CI 1.211-3.818, P = 0.001), and pulmonary hypertension (OR 4.165, 95% CI 1.765-9.882, P = 0.009). AMA-M2 and pulmonary hypertension were the most consistent associated factors in the polymyositis subgroup and the dermatomyositis/clinically amyopathic dermatomyositis subgroup. MIs are rare but serious complication of IIMs could lead to congestive heart failure and severe arrhythmias. IIM patients with AMA-M2 positivity, elevation of CK and CRP, and pulmonary hypertension are more likely to develop MI complications. Key Points • This study investigated the independent associated factors for clinically significant myocardial involvements among idiopathic inflammatory myopathies in a large-scale, nation-wide multi-center cross-sectional study.

Muscle pathological features and extra-muscle involvement in idiopathic inflammatory myopathies with anti-mitochondrial antibody

ObjectiveAnti-mitochondrial antibodies (AMAs) can be detected in some idiopathic inflammatory myopathy (IIM) patients. We aimed to investigate the clinical features of IIM patients with AMAs. MethodsWe retrospectively analysed 1,167 consecutive patients with IIM for AMA-associated myositis and compared them to age- and gender-matched AMA-negative IIM patients. ResultsTwenty-nine patients (2.5%) were identified with AMA-positive myositis; eight of them had primary biliary cholangitis (PBC). There were no significant differences in skin rash, dysphagia, interstitial lung disease, and muscle strength between AMA-positive patients and AMA-negative patients. Of 23 cases, 12 (52.2%) showed immune-mediated necrotizing myopathy (IMNM)-like pathological features. amongst AMA-positive patients, 11 of 16 patients with isolated anti-AMAs were classified as IMNM which was significantly higher than that of patients with coexistent anti-AMAs and myositis-specific antibodies (p = 0.026). Moreover, subclinical cardiac involvement was significantly more common in AMA-positive patients than in AMA-negative patients (21/29 VS 33/116, p<0.001). In addition, patients without PBC had a significantly higher incidence of abnormal echocardiography findings than that of patients with PBC (p = 0.009). Patients without heart abnormalities took significantly less time to achieve disease remission and prednisone tapering to <10 mg than patients with heart abnormalities (p = 0.000 and p = 0.001, respectively). ConclusionsIMNM was a major histopathological finding in IIM patients with isolated AMAs. AMAs were significantly associated with subclinical cardiac involvement in IIM. PBC seemed to be a protective factor for abnormal echocardiography findings in AMA-positive patients. Patients without heart involvement took less time to achieve disease remission and prednisone tapering off.

Anti-MDA5 juvenile idiopathic inflammatory myopathy with second-degree heart block but no skin or lung involvement: a case report

BackgroundPatients with idiopathic inflammatory myopathy and autoantibodies directed against melanoma differentiation-associated protein 5 (MDA5) characteristically have interstitial lung disease, severe cutaneous involvement, arthritis, and relatively mild myositis. Cardiac involvement in idiopathic inflammatory myopathy can occur and has been associated with anti-signal recognition particle and anti-polymyositis-scleroderma autoantibodies, but not with anti-MDA5 autoantibodies.Case presentationA 14-year-old male presented with weakness, second-degree heart block, arthritis, and hematologic cytopenias. Imaging and biopsies confirmed the diagnosis of juvenile idiopathic inflammatory myopathy, and he had high titer anti-MDA5 autoantibodies. There were no cutaneous or pulmonary abnormalities. While on prednisone and methotrexate, the patient’s heart block improved from second- to first-degree and the cytopenias resolved. Persistent myositis prompted the addition of intravenous immunoglobulin. Seven months into the disease course, the arthritis and myositis are in remission and the patient is no longer taking corticosteroids.ConclusionsWe report a novel case of a patient with juvenile idiopathic myositis who lacked the typical cutaneous and pulmonary findings associated with anti-MDA5 positivity, but who had cardiac conduction defects. This report broadens the clinical spectrum of anti-MDA5-associated inflammatory myopathy.

T1 mapping-derived signature of myocardial involvement in idiopathic inflammatory myopathy compared to acute viral myocarditis: a texture-based analysis

Abstract Funding Acknowledgements Type of funding sources: None. Background. Recent studies revealed the ability of MRI T1 mapping to characterize myocardial involvement in both idiopathic inflammatory myopathy (IIM) and acute viral myocarditis (AVM), as compared to healthy controls. However, neither myocardial T1 nor T2 maps were able to discriminate between IIM and AVM patients, when considering conventional myocardial mean values and derived indices such as lambda and extracellular volume. Purpose. To investigate the ability of T1 mapping-derived texture analysis to differentiate IIM from AVM. Methods. Forty patients, 20 with IIM (51 ± 17 years, 9 men) and 20 with AVM (34 ± 13 years, 16 men) underwent 1.5T MRI T1 mapping using a modified Look-Locker inversion-recovery sequence before and 15 minutes after injection of a gadolinium contrast agent. After manual delineation of endocardial and epicardial borders and co-registration of all inversion time images, native and post-contrast T1 maps were estimated. Myocardial texture analysis was performed on native T1 maps. Textural features such as: autocorrelation, contrast, dissimilarity, energy and sum entropy were used to build a least squares-based linear regression model. Finally, receiver operating characteristic (ROC) analysis was used to investigate the ability of such texture features score to classify IIM vs. AVM patients, compared to the performance of mean myocardial T1. A Wilcoxon rank-sum test was also used to test difference significance between groups. Results. Both native and post-contrast mean myocardial T1 values were comparable between IIM (native: 1022 ± 43 ms; post-contrast: 319 ± 44 ms) and AVM (1056 ± 59 ms, p = 0.07; 318 ± 35 ms, p = 0.90, respectively) groups. Results of ROC analyses are provided in the Table, indicating that a better discrimination between IIM and AVM patients was obtained when using texture features, with higher AUC and accuracy than mean T1 values (Figure). Conclusion. Texture analysis derived from MRI T1 maps without contrast agent injection was able to discriminate between IIM and AVM with higher accuracy, sensitivity and specificity than conventional T1 indices. Such analysis could provide a useful myocardial signature to help diagnose and manage cardiac alterations associated with IIM in patients presenting with myocarditis and primarily suspected of AVM. Table Area under curve (AUC) Accuracy Sensitivity Specificity Native T1 0.67 0.70 0.65 0.75 Post-contrast T1 0.49 0.60 0.25 0.95 Texture features score 0.85 0.82 0.90 0.75 ROC analyses for classification between IIM and AVM patients Abstract Figure

Cardiovascular Magnetic Resonance Mapping and Strain Assessment for the Diagnosis of Cardiac Involvement in Idiopathic Inflammatory Myopathy Patients With Preserved Left Ventricular Ejection Fraction.

The aim of the study was to evaluate the role of cardiac magnetic resonance (CMR) mapping and strain analysis in the identification of cardiac involvement in idiopathic inflammatory myopathy (IIM) patients with preserved left ventricular ejection fraction. In all, 38 IIM patients who underwent CMR examination at our institution were retrospectively included. Twenty-three age-matched healthy individuals served as controls. Mapping parameters including native T1, extracellular volume (ECV), and T2 mapping and strain parameters including global radial strain, global circumferential strain, and global longitudinal strain were measured semiautomatically using a dedicated processing software. All the mapping and strain values were compared between patients and controls. Late gadolinium enhancement was only present in IIM patients (n=17, 44.7%). IIM patients showed higher native T1 (1346 vs. 1269 ms, P<0.001), ECV (31.1% vs. 27.4%, P<0.01), and higher T2 (44.4 vs. 39.2 ms, P<0.001) values compared with controls. The global radial strain (36.7% vs. 46.9%, P<0.001), global circumferential strain (-21.2% vs. -24.1%, P<0.01), and global longitudinal strain (-13.6% vs. -15.6%, P<0.05) values were significantly reduced compared with controls. Native T1, ECV, T2 values, and global strain values may hold promise for the detection of subclinical myocardial involvement in IIM patients with preserved left ventricular ejection fraction.

The diagnostic value of GDF-15 for myocardial involvement in idiopathic inflammatory myopathy.

Cardiac involvement is a serious complication of idiopathic inflammatory myopathy (IIM). GDF-15 can predict the risk and the prognosis of cardiovascular disease, but its value is unclear in IIM. To investigate the diagnostic value of GDF-15 for myocardial involvement in IIM. A total of 77 IIM patients from May 2018 to August 2020 were included in this retrospective study. Of these, 43 patients underwent cardiac magnetic resonance (CMR) examination. There were 33 SLE patients and 16 healthy people were used as the control group. The concentration of GDF-15 of these groups was measured by ELISA. There were significant differences in GDF-15 levels in patients with IIM, SLE and healthy controls (H = 45.291, P<0.001). GDF-15 levels were statistically significant different between IIM patients with the myocardial injury [1484.88(809.07 2835.50) pg/ml] and without myocardial injury [593.26(418.61 784.59) pg/ml, P=0.001]. After adjusted for age, renal function, the risk of myocardial injury in IIM patients increased an average of 0.3% by per increased unit of GDF-15 (odds ratio=1.003, 95% CI: 1.000, 1.007). The level of GDF-15 was positively correlated with extra-cellular volume (ECV) (rs = 0.348, P=0.028). GDF-15 ≥ 929.505 pg/ml (area under the curve=0.856, 95% CI: 0.744, 0.968) predicted myocardial injury in IIM with a sensitivity of 0.75 and specificity of 0.90. GDF-15 could serve as a potential biomarker to predict myocardial injury in IIM patients.

Necrotizing Myopathy Resulting In Myocarditis And Sudden Death

AbstractNecrotizing myopathy (NM), a type of idiopathic inflammatory myopathy (IIM), is an autoimmune disorder in which immune cells target skeletal muscle, resulting in muscle breakdown and weakness. Cardiac involvement in this disorder is rare. We highlight a case of autoantibody-negative NM complicated by myocarditis and sudden cardiac death.Case Report: A 45-year-old male with a history of hypertension, previous hepatitis B, and obesity presented with 6 months of progressive proximal lower extremity weakness. Workup revealed elevated creatinine phosphokinase, ESR and CRP, elevated troponin, and electromyography notable for a myopathic process. Age appropriate malignancy screening, infectious workup, and myositis panel were negative. The patient underwent left bicep biopsy which revealed muscle fiber necrosis and regeneration, consistent with the diagnosis of seronegative NM. Patient was discharged on mycophenolate and intravenous immunoglobulin with improvements in peripheral strength. Unfortunately, while recovering, he was readmitted with palpitations and dyspnea. Electrocardiogram showed a new trifascicular block and telemetry revealed frequent runs of non-sustained ventricular tachycardia (NSVT). A Cardiac MRI revealed evidence of myocarditis (Image 1). Endomyocardial biopsy showed fibrosis with entrapped myocytes (Image 2). He was treated with pulse dose intravenous glucocorticoids and mycophenolate was increased. EP study (EPS) was performed which did not reveal inducible VT; therefore, implantable cardiac defibrillator (ICD) was not implanted. Three months later the patient had sudden unexplained death at home.Discussion: Cardiac involvement in IIM—especially seronegative NM—is rare and not well described. Our patient had worsening cardiac involvement despite improvements in his skeletal myopathy. Despite a recent negative EPS, the patient had sudden unexplained death. Perhaps, a lower threshold for ICD implantation should be considered in this population. This case raises awareness for cardiac involvement in seronegative NM and raises the question of whether these patients are at increased risk of sudden cardiac death.

Myocardial involvement and deformation abnormalities in idiopathic inflammatory myopathy assessed by CMR feature tracking

BackgroundCardiac involvement has been described in idiopathic inflammatory myopathies (IIM), including non-specific ECG and echocardiographic findings. Aim of our study was to evaluate myocardial deformation parameters in IIM and to correlate them with late gadolinium enhancement (LGE) findings using cardiac magnetic resonance imaging (CMR).MethodsForty-seven consecutive patients with histologically proven IIM were included into our study. Twenty-five healthy volunteers were used as a control group. All patients and controls underwent CMR examination using a 1.5 T scanner including functional cine and LGE imaging. After a mean follow-up of 234.7 ± 79.5 days a second CMR examination was performed in IIM patients.ResultsIn comparison to healthy volunteers, IIM patients had lower left ventricular mass and left ventricular global radial, circumferential and longitudinal strain. There was no significant difference in left ventricular ejection fraction.Patients with LGE (N = 28) had lower left ventricular ejection fraction (p = 0.016), global right and left ventricular longitudinal strain (p = 0.014 and p = 0.005) and global left ventricular diastolic longitudinal strain rate (p = 0.001) compared to patients without LGE (N = 19).In IIM patients, a significant decrease of left ventricular ejection fraction, left ventricular mass and all measured deformation parameters was observed between baseline and follow-up CMR.ConclusionCardiac involvement in IIM is frequent. Impairment of systolic and diastolic deformation parameters and a worsening over time can be observed. CMR is a useful tool for cardiac diagnostic work-up of these patients.

CMR T1 mapping and strain analysis in idiopathic inflammatory myopathy: evaluation in patients with negative late gadolinium enhancement and preserved ejection fraction.

To investigate whether cardiovascular magnetic resonance (CMR) T1 mapping and strain parameters can detect early histological and functional myocardial changes in idiopathic inflammatory myopathy (IIM) with negative late gadolinium enhancement (LGE) and preserved ejection fraction. Thirty consecutive patients with IIM (41.5 ± 15.4 years, 24 females) who did not have LGE or reduced left ventricular ejection fraction (LVEF) and 30 age- and gender-matched healthy controls (40.6 ± 14.2 years, 20 females) were recruited. Patients with IIM were further classified into two subgroups according to high-sensitivity cardiac troponin I (hs-cTnI) values: elevated hs-cTnI subgroup (n = 10) and normal hs-cTnI subgroup (n = 20). Myocardial native T1 values, extracellular volume (ECV) fractions, and strain parameters were analyzed in patients with IIM and healthy controls. Compared with healthy controls, patients with IIM had significantly prolonged native T1 values and increased ECV in each LV segment (p < 0.05). In further subgroup analysis, LV mid-slice native T1 values had the most power to discriminate between patients with elevated hs-cTnI and healthy controls (area under the curve = 0.92). There was no significant difference of global LV strain or strain rates between IIM patients and controls. Diffuse interstitial fibrosis can be detected by CMR T1 mapping in patients with IIM who do not have LGE or reduced LVEF or elevated hs-cTnI, and it may be a promising method for screening subclinical cardiac involvement in IIM. • Myocardial abnormality in IIM is often subclinical and leads to poor prognosis. • Conventional CMR parameters have limitations in early detection of cardiac function and tissue changes. • CMR T1 mapping techniques and myocardial strain analysis have the potential to provide detailed information on cardiac histology and function.