Clinical, Histological, and Immunohistochemical Findings in Inclusion Body Myositis

Camargo, Leonardo Valente de; Carvalho, Mary S.; Shinjo, Samuel Katsuyuki; Oliveira, Acary Souza Bullé; Zanoteli, Edmar

doi:10.1155/2018/5069042

Cited by 21 publications

(18 citation statements)

References 42 publications

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“…Besides relapsing–remitting episodes of dysphagia, several authors report that the prevalence of dysphagia increases as the disease progresses [ 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 ]. Nevertheless, dysphagia can also be the initial [ 17 , 18 , 19 , 20 , 21 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 ] or even the only symptom [ 18 , 29 , 31 , 33 ]. Therefore, dysphagia should not be considered a late symptom in IIM.…”

Section: Resultsmentioning

confidence: 99%

The Impact of Dysphagia in Myositis: A Systematic Review and Meta-Analysis

Labeit

Pawlitzki

Ruck

et al. 2020

JCM

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(1) Background: Dysphagia is a clinical hallmark and part of the current American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) diagnostic criteria for idiopathic inflammatory myopathy (IIM). However, the data on dysphagia in IIM are heterogenous and partly conflicting. The aim of this study was to conduct a systematic review on epidemiology, pathophysiology, outcome and therapy and a meta-analysis on the prevalence of dysphagia in IIM. (2) Methods: Medline was systematically searched for all relevant articles. A random effect model was chosen to estimate the pooled prevalence of dysphagia in the overall cohort of patients with IIM and in different subgroups. (3) Results: 234 studies were included in the review and 116 (10,382 subjects) in the meta-analysis. Dysphagia can occur as initial or sole symptom. The overall pooled prevalence estimate in IIM was 36% and with 56% particularly high in inclusion body myositis. The prevalence estimate was significantly higher in patients with cancer-associated myositis and with NXP2 autoantibodies. Dysphagia is caused by inflammatory involvement of the swallowing muscles, which can lead to reduced pharyngeal contractility, cricopharyngeal dysfunction, reduced laryngeal elevation and hypomotility of the esophagus. Swallowing disorders not only impair the quality of life but can lead to serious complications such as aspiration pneumonia, thus increasing mortality. Beneficial treatment approaches reported include immunomodulatory therapy, the treatment of associated malignant diseases or interventional procedures targeting the cricopharyngeal muscle such as myotomy, dilatation or botulinum toxin injections. (4) Conclusion: Dysphagia should be included as a therapeutic target, especially in the outlined high-risk groups.

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Section: Resultsmentioning

confidence: 99%

The Impact of Dysphagia in Myositis: A Systematic Review and Meta-Analysis

Labeit

Pawlitzki

Ruck

et al. 2020

JCM

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“…Such a finding, associated with the presence of the “undulating fascia” signal (Fig. G), a distal quadriceps atrophy consequence, may cause diagnostic confusion with sporadic inclusion body myositis (sIBM), especially because sIBM also occurs in individuals over 40 years of age and has a male predominance . However, sIBM presents a distal involvement in upper limbs, while in our patients, there was a predominant involvement of proximal muscles of the upper and lower limbs, and the typical degenerative histological changes observed in sIBM muscles are not described in patients with ANO5 mutations.…”

Section: Discussionmentioning

confidence: 64%

Clinical and molecular findings in a cohort of ANO5‐related myopathy

Silva

Coimbra-Neto

Souza

et al. 2019

Ann Clin Transl Neurol

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Objective ANO5‐related myopathy is an important cause of limb‐girdle muscular dystrophy (LGMD) and hyperCKemia. The main descriptions have emerged from European cohorts, and the burden of the disease worldwide is unclear. We provide a detailed characterization of a large Brazilian cohort of ANO5 patients. Methods A national cross‐sectional study was conducted to describe clinical, histopathological, radiological, and molecular features of patients carrying recessive variants in ANO5. Correlation of clinical and genetic characteristics with different phenotypes was studied. Results Thirty‐seven patients from 34 nonrelated families with recessive mutations of ANO5 were identified. The most common phenotype was LGMD, observed in 25 (67.5%) patients, followed by pseudometabolic presentation in 7 (18.9%) patients, isolated asymptomatic hyperCKemia in 4 (10.8%) patients, and distal myopathy in a single patient. Nine patients presented axial involvement, including one patient with isolated axial weakness. The most affected muscles according to MRI were the semimembranosus and gastrocnemius, but paraspinal and abdominal muscles, when studied, were involved in most patients. Fourteen variants in ANO5 were identified, and the c.191dupA was present in 19 (56%) families. Sex, years of disease, and the presence of loss‐of‐function variants were not associated with specific phenotypes. Interpretation We present the largest series of anoctaminopathy outside Europe. The most common European founder mutation c.191dupA was very frequent in our population. Gender, disease duration, and genotype did not determine the phenotype.

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“…In a survey on idiopathic inflammatory myositis and their comorbidities in Australia, the IBM subgroup also showed arterial hypertension in 65% (n = 65) [32]. A Brazilian cohort (n = 18) reported arterial hypertension in 72% [33].…”

Section: Discussionmentioning

confidence: 99%

Sporadic inclusion body myositis: no specific cardiac involvement in cardiac magnetic resonance tomography

et al. 2020

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Objective To investigate cardiac involvement in patients with sporadic inclusion body myositis (IBM) by cardiac magnetic resonance tomography (CMR). Methods A case series of 20 patients with IBM underwent basic cardiac assessment and CMR including functional imaging, native and contrast-enhanced T1-weighted, and late gadolinium enhancement (LGE) imaging. Results All IBM patients reported no cardiac symptoms. Echocardiography was normal in 16/17 IBM patients. In CMR, IBM patients had normal ejection fractions (mean LVEF 63 ± 7%) and ventricular mass. They had reduced left (mean 55 versus 88 ml) and right ventricular stroke volumes (mean 54 versus 86 ml) and increased early myocardial enhancement (pathological T1 Ratio in 44% versus 5%), as compared to age-and gender-matched controls. Since arterial hypertension was more often observed in IBM patients, hypertensive heart disease can also be causative for these changes. Late gadolinium enhancement did not differ statistically from healthy controls. There was no apparent association between elevated biomarkers, echocardiography and CMR. Conclusion CMR revealed subtle changes in cardiac geometry and tissue characterization in IBM patients when compared to a gender-and age-matched control group. Findings in CMR indicated a higher extent of diffuse myocardial fibrosis as well as smaller left ventricular stroke volumes. These alterations may be due to a higher prevalence of arterial hypertension in the IBM cohort.

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Clinical, Histological, and Immunohistochemical Findings in Inclusion Body Myositis

Cited by 21 publications

References 42 publications

The Impact of Dysphagia in Myositis: A Systematic Review and Meta-Analysis

The Impact of Dysphagia in Myositis: A Systematic Review and Meta-Analysis

Clinical and molecular findings in a cohort of ANO5‐related myopathy

Sporadic inclusion body myositis: no specific cardiac involvement in cardiac magnetic resonance tomography

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