scholarly journals ECG Changes Through Immunosuppressive Therapy Indicate Cardiac Abnormality in Anti-MDA5 Antibody-Positive Clinically Amyopathic Dermatomyositis

2022 ◽  
Vol 12 ◽  
Author(s):  
Takashi Matsuo ◽  
Tsuneo Sasai ◽  
Ran Nakashima ◽  
Yoshihiro Kuwabara ◽  
Eri Toda Kato ◽  
...  
Keyword(s):  
Immunosuppressive Therapy ◽  
Cardiac Involvement ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Active Phase ◽  
Muscle Enzymes ◽  
T Wave ◽  
Group I ◽  
Remission Phase ◽  
Active Disease

Anti-melanoma differentiation-associated gene 5 (MDA5) antibody, a dermatomyositis (DM)-specific antibody, is strongly associated with interstitial lung disease (ILD). Patients with idiopathic inflammatory myopathy (IIM) who are anti-MDA5 antibody positive [anti-MDA5 (+)] often experience chest symptoms during the active disease phase. These symptoms are primarily explained by respiratory failure; nevertheless, cardiac involvement can also be symptomatic. Thus, the aim of this study was to investigate cardiac involvement in anti-MDA5 (+) DM. A total of 63 patients with IIM who underwent electrocardiography (ECG) and ultrasound cardiography (UCG) during the active disease phase from 2016 to 2021 [anti-MDA5 (+) group, n = 21; anti-MDA5-negative (-) group, n = 42] were enrolled in the study, and their clinical charts were retrospectively reviewed. The ECG and UCG findings were compared between the anti-MDA5 (+) and anti-MDA5 (-) groups. All anti-MDA5 (+) patients had DM with ILD. The anti-MDA5 (+) group showed more frequent skin ulcerations and lower levels of leukocytes, muscle enzymes, and electrolytes (Na, K, Cl, and Ca) than the anti-MDA5 (-) group. According to the ECG findings obtained during the active disease phase, the T wave amplitudes were significantly lower for the anti-MDA5 (+) group than for the anti-MDA5 (-) group (I, II, and V4–6 lead; p < 0.01; aVF and V3, p < 0.05). However, the lower amplitudes were restored during the remission phase. Except for the E wave, A wave and Sep e’, the UCG results showed no significant differences between the groups. Four patients with anti-MDA5 (+) DM had many leads with lower T wave and cardiac abnormalities (heart failure, diastolic dysfunction, myocarditis) on and after admission. Though anti-MDA5 (+) patients clinically improved after immunosuppressive therapy, some of their ECG findings did not fully recover in remission phase. In conclusion, anti-MDA5 (+) DM appears to show cardiac involvement (electrical activity and function) during the active phase. Further studies are necessary to clarify the actual cardiac condition and mechanism of these findings in patients with anti-MDA5 (+) DM.

scholarly journals Betekintés az idiopathiás inflammatorikus myopathiában szenvedő betegek gyógytornájába

2016 ◽  
Vol 157 (39) ◽  
pp. 1557-1562
Author(s):  
Andrea Váncsa
Keyword(s):  
Randomized Controlled Trials ◽  
Inclusion Body ◽  
Aerobic Capacity ◽  
Inclusion Body Myositis ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Muscle Performance ◽  
Controlled Trials ◽  
Randomized Controlled ◽  
Active Disease

Using current recommended treatment, a majority of patients with idiopathic inflammatory myopathy develop muscle impairment and poor health. Beneficial effects of exercise have been reported on muscle performance, aerobic capacity and health in chronic polymyositis and dermatomyositis, as well as in active disease and inclusion body myositis to some extent. Importantly, randomized controlled trials indicate that improved health and decreased clinical disease activity could be mediated through increased aerobic capacity. Recently, reports seeking pathomechanisms of the underlying effects of exercise on skeletal muscle indicate increased aerobic capacity (i.e. increased mitochondrial capacity and capillary density, reduced lactate levels), activation of genes of aerobic phenotype and muscle growth programs and down regulation of genes related to inflammation. Exercise contributes to both systemic and within-muscle adaptations demonstrating that it is fundamental for improving muscle performance and health in patients with idiopathic inflammatory myopathy. There is a need for randomized controlled trials to study the effects of exercise in patients with active disease and inclusion body myositis. Orv. Hetil., 2016, 157(39), 1557–1562.

Polymyositis and dermatomyositis

2010 ◽  
pp. 3692-3698
Author(s):  
John H. Stone
Keyword(s):  
Cytotoxic T Cells ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Hla Class I ◽  
Muscle Enzymes ◽  
Calcinosis Cutis ◽  
Muscle Involvement ◽  
Cutaneous Manifestations ◽  
Associated Malignancy ◽  
Mechanic’S Hands

Polymyositis and dermatomyositis are two types of idiopathic inflammatory myopathy. The pathological findings in polymyositis suggest an HLA class I-restricted immune response mediated by cytotoxic T cells; dermatomyositis appears to be associated with humorally mediated destruction of muscle-associated microvasculature. Clinical features—polymyositis is characterized by symmetrical painless proximal muscle weakness that develops slowly, usually over weeks to months, and typically associated with significant elevation of serum creatine kinase and other muscle enzymes. The pattern of muscle involvement in dermatomyositis is clinically indistinguishable from that of polymyositis, but with cutaneous manifestations including Gottron’s sign, heliotrope rash, erythema, ‘mechanic’s hands’, periungual abnormalities, and calcinosis cutis. Extra-muscular features include interstitial lung disease (30% of cases), aspiration pneumonia, and associated malignancy (polymyositis 9%, dermatomyositis 15%)....

scholarly journals Risk factors associated with Pneumocystis jirovecii pneumonia in juvenile myositis in North America

Rheumatology ◽  
2020 ◽  
Author(s):  
Sara E Sabbagh ◽  
Jessica Neely ◽  
Albert Chow ◽  
Marietta DeGuzman ◽  
Jamie Lai ◽  
...  
Keyword(s):  
Risk Factors ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Immunosuppressive Therapy ◽  
Inflammatory Myopathy ◽  
Severe Infection ◽  
Idiopathic Inflammatory Myopathy ◽  
Pneumocystis Jirovecii ◽  
Pneumocystis Jirovecii Pneumonia ◽  
The Usa

Abstract Objectives Pneumocystis jirovecii pneumonia (PJP) is associated with significant morbidity and mortality in adult myositis patients; however, there are few studies examining PJP in juvenile myositis [juvenile idiopathic inflammatory myopathy (JIIM)]. The purpose of this study was to determine the risk factors and clinical phenotypes associated with PJP in JIIM. Methods An research electronic data capture (REDCap) questionnaire regarding myositis features, disease course, medications and PJP infection characteristics was completed by treating physicians for 13 JIIM patients who developed PJP (PJP+) from the USA and Canada. Myositis features and medications were compared with 147 JIIM patients without PJP (PJP–) from similar geographic regions who enrolled in National Institutes of Health natural history studies. Results PJP+ patients were more often of Asian ancestry than PJP– patients [odds ratio (OR) 8.7; 95% CI 1.3, 57.9]. Anti- melanoma differentiation associated protein 5 (MDA5) autoantibodies (OR 12.5; 95% CI 3.0, 52.4), digital infarcts (OR 43.8; 95% CI 4.2, 460.2), skin ulcerations (OR 12.0; 95% CI 3.5, 41.2) and interstitial lung disease (OR 10.6; 95% CI 2.1, 53.9) were more frequent in PJP+ patients. Before PJP diagnosis, patients more frequently received pulse steroids, rituximab and more immunosuppressive therapy compared with PJP– patients. Seven PJP+ patients were admitted to the intensive care unit and four patients died due to PJP or its complications. Conclusions PJP is a severe infection in JIIM that can be associated with mortality. Having PJP was associated with more immunosuppressive therapy, anti-MDA5 autoantibodies, Asian race and certain clinical features, including digital infarcts, cutaneous ulcerations and interstitial lung disease. Prophylaxis for PJP should be considered in juvenile myositis patients with these features.

scholarly journals ASSESSMENT AND MANAGEMENT OF A PATIENT WITH PARANEOPLASTIC DERMATOMYOSITIS

2017 ◽  
Vol 16 (4) ◽  
pp. 166-169
Author(s):  
Ioan-Cristian Lupescu ◽  
◽  
Adriana Octaviana Dulamea ◽  
◽  
Keyword(s):  
Skeletal Muscles ◽  
Inflammatory Myopathy ◽  
Elevated Serum ◽  
Idiopathic Inflammatory Myopathy ◽  
Intravenous Immunoglobulins ◽  
Left Breast ◽  
Motor Deficits ◽  
Muscle Enzymes ◽  
Extensive Search ◽  
Residual Neoplasm

Dermatomyositis (DM) is an idiopathic inflammatory myopathy, that can be associated with malignancy. We report the case of a 60-years-old woman, diagnosed and treated for left breast cancer, with residual neoplasm following treatment, who was admitted for generalized myalgias and tetraparesis with predominance of paraparesis. Clinical exam revealed heliotrope rash with bilateral palpebral edema and an erythematous eruption on both thighs and anterior thorax. Motor deficits were predominantly proximal. Paraclinical evaluation excluded other etiologies and revealed elevated serum muscle enzymes and electromyographic pattern of myopathy. An MRI was performed and showed diffuse edematous infiltration of skeletal muscles. Symptoms diminished under corticotherapy, intravenous immunoglobulins and cyclophosphamide. Left mastectomy was eventually performed, but DM symptoms reappeared a few months later. Despite extensive search, no recurrence of tumor was found. However, under treatment, the patient once again recovered, and later resumed her activity.

scholarly journals AB0726 BIOLOGICS IN CHILDREN WITH IDIOPATHIC INFLAMMATORY MYOPATHIES: AN EXPERIENCE OF SINGLE CENTER

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1394.1-1394
Author(s):  
M. Kaleda ◽  
I. Nikishina ◽  
V. Matkava ◽  
Z. Kolkhidova ◽  
S. Arsenyeva ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Lupus Erythematosus ◽  
Disease Duration ◽  
Intensive Therapy ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Muscle Enzymes ◽  
Overlap Syndromes ◽  
Median Disease Duration

Background:Juvenile dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy (IIM) of childhood, but the involvement of muscle also can be complication of systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD) and different overlap syndromes. The presence of myopathy can significantly affect the prognosis of the disease and the quality of life. Despite of the absence of official indication for biological therapy fo IIM, it`s may be needed in severe course of disease.Objectives:To analyze in retrospective study the efficacy and safety of Biologics (B) in patients (pts) with IIM in our pediatric rheumatology center.Methods:The study included all pts with rheumatic diseases (RD), who had IIM and received B during past 10 years.Results:A total of 17 pts (10 females) were identified: 23.5% with JDM, 11.8% - SLE, 35.3% - MCTD, 29.4% - overlap syndromes. The median age at the onset of RD was 9.75 years [interquartile range (IQR) 6.9; 13.5]. The median disease duration at the onset of myopathy was 7.0 months [3.0; 10.0], in all pts with JDM – at onset. All pts had myopathic syndrome. The rash tipical for JDM was in 8 pts (47%). The lung involvement observed in 6 pts (35.3%). Levels of serum muscle enzymes (CK, ALT, AST and lactate dehydrogenase [LDH]) were elevated in 88.2% of pts: CK in 52.9% (mean ± std 624.35±1007.2 U/L), ALT – 82.4% (mean ± std 142.18±148.16U/L), AST – 82.4% (mean ± std 159.6±201.1 U/L), LDH – 88.2% (mean ± std 492.5±229.2 U/L). Abnormal EMG findings were in 10 pts (59.4%). Nailfold capillaroscopic changes tipical for IIM had 10 pts (59.4%). Before B 94.1% of pts received corticosteroids (CS), 58.8% - methotrexate, 29.4% - hydroxychloroquine, 17.6% - cyclophosphamide, 11.8% - cyclosporine, 5.9% - mycophenolate mofetil, 5.9% - azathioprine, 47% - IVIG. B therapy was started due to insufficient efficacy of previous therapy. The median age at start of B was 12.3 years [IQR 9.5; 15.0]. The median disease duration prior to treatment with B was 2.25 years [IQR 0,8; 3.6]. 58.8% of pts received rituximab (RTX), 41.2% - abatacept (ABA). The median time between each course of RTX was 182 days [IQR 156–315]. 80% of pts underwent more than one course of RTM therapy, with a maximum of 5 courses. The median duration of ABA therapy was 2.8 years [IQR 1.6; 5.0]. Discontinuation of B due to serious AE was observed in 1 patient with overlap syndrome (5.9%) – macrophage activation syndrome (MAS) 8 day after RTX infusion (5th course, 1st infusion). One patient (female, 12.3 yo) died from MAS (at onset of SLE, developed before B), the others achieved remission. B therapy allowed to reduce the dose of CS to maintenance in all pts, decrease of calcinosis in 2 pts and improve the quality of life.Conclusion:Our study demonstrated that B is highly effective in children with IIM and have the acceptable safety. Early diagnosis and initiation of intensive therapy are important in reducing symptoms of myopathy in RD. It seems that the development of IIM in other RDs, not JDM, indicates the need for B and its good efficacy. The further extended studies are needed.Disclosure of Interests:None declared

scholarly journals Muscle Pathological Features and Extra-Muscle Involvement in Idiopathic Inflammatory Myopathies With Anti-Mitochondrial Antibody

2020 ◽  
Author(s):  
Lu Zhang ◽  
Hanbo Yang ◽  
Jieping Lei ◽  
Qinglin Peng ◽  
Hongxia Yang ◽  
...  
Keyword(s):  
Protective Factor ◽  
Cardiac Involvement ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Primary Biliary Cholangitis ◽  
Pathological Features ◽  
Muscle Involvement ◽  
Disease Remission ◽  
Heart Abnormalities ◽  
Mitochondrial Antibody

Abstract BackgroudAnti-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.Methods We retrospectively analysed consecutive 1,167 patients with IIM for AMAs-associated myositis and compared them to age- and sex-matched AMA-negative patients. ResultsTwenty-nine patients (2.5%) were identified with AMAs-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 AMAs-positive patients and disease controls. 12/23(52.2%) cases showed immune-mediated necrotizing myopathy (IMNM)-like pathological features. Among AMAs-positive patients, 11 of 16 patients with isolated anti-AMA were classified as IMNM which was significantly higher than that of patients with coexistent anti-AMAs and myositis-specific antibodies (p=0.026). Moreover, AMAs-positive patients had a significantly higher cardiac involvement ratio (P<0.001) compared to controls. Comparsion in AMAs-positive IIM patients show the incidence of abnormal echocardiography findings was significantly higher in patients without primary biliary cholangitis (PBC) than in 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<0.001 and P=0.001, respectively).ConclusionsIMNM was a major histopathological finding in IIM patients with isolated anti-AMAs antibody. AMAs was significantly associated with cardiac involvement in IIM. PBC seemed to be a protective factor for abnormal echocardiography findings in AMAs-positive patients. Patients without heart involvement took less time to achieve disease remission and prednisone tapering off.

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