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.

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 Heart–Lung–Muscle Anti-SAE Syndrome: An Atypical Severe Combination

2018 ◽  
Vol 8 (1) ◽  
pp. 20
Author(s):  
Elisabet Zamora ◽  
Elena Seder-Colomina ◽  
Susana Holgado ◽  
Bibiana Quirant-Sanchez ◽  
José Mate ◽  
...  
Keyword(s):  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Resonance Imaging ◽  
Cardiac Enzymes ◽  
Muscle Involvement ◽  
Progressive Dyspnea ◽  
Nonspecific Interstitial Pneumonia ◽  
Work Up

A 78-year-old man with 3 months of progressive dyspnea, dysphony, dysgeusia, and proximal muscle weakness was diagnosed of probably idiopathic inflammatory myopathy with nonspecific interstitial pneumonia. Variable degrees of atrioventricular block and persistently elevated cardiac enzymes indicated a diagnosis of myocarditis, confirmed with cardiac magnetic resonance imaging and endomyocardial biopsy. A comprehensive immune work-up revealed anti-small ubiquitin-like modifier-1 activating enzyme (anti-SAE) antibody, a novel myositis-specific antibody, previously described mainly with overt cutaneous dermatomyositis and late skeletal muscle manifestations. Here, heart–lung–muscle involvement combined with anti-SAE antibodies was a severe combination.

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 &lt; 0.01; aVF and V3, p &lt; 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.

Sign in / Sign up

Export Citation Format

Share Document