Severe and refractory myositis in mixed connective tissue disease: a description of a rare case

Bonin, C C; Silva, B Santos Pires da; Mota, Licia Maria Henrique da; Carvalho, Jozélio Freire de

doi:10.1177/0961203310376637

Cited by 11 publications

(6 citation statements)

References 11 publications

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“…There are several articles reporting positive therapeutic effects of immunomodulatory medication on symptoms and/or on findings of objective swallowing evaluations. These include intravenous methylprednisolone pulse therapy [ 19 , 33 , 70 , 81 , 87 , 134 , 154 , 155 , 156 , 157 , 158 , 159 ], methotrexate [ 30 , 76 , 89 , 124 , 136 , 154 , 159 , 160 , 161 ], long-term prednisone/prednisolone [ 29 , 30 , 33 , 59 , 64 , 70 , 74 , 76 , 81 , 83 , 84 , 87 , 89 , 101 , 103 , 111 , 118 , 122 , 124 , 134 , 136 , 143 , 154 , 158 , 159 , 161 , 162 , 163 , 164 , 165 ,…”

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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“…Bonin et al . [17] described a unique case of an adult female patient, in whom initial presentation of MCTD included myositis, characterized by dysphagia, progressive myopathy and muscle weakness, mostly concerning the neck and also shoulder and pelvic girdle. While there was no response to the administration of an initial dose of corticosteroids, improvement was observed after implementation of pulse therapy with methylprednisolone accompanied by methotrexate.…”

Section: Discussionmentioning

confidence: 99%

Mixed connective tissue disease presenting with progressive scleroderma symptoms in a 10-year-old girl

Latuśkiewicz-Potemska¹,

Zygmunt²,

Biernacka-Zielińska³

et al. 2013

pdia

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Mixed connective tissue disease (MCTD) is a systemic inflammatory disease affecting connective tissue with the underlying autoimmunological mechanism. The core of MCTD is an appearance of symptoms of several other inflammatory diseases of connective tissue – systemic lupus erythematosus, systemic scleroderma, poly- or dermatomyositis, rheumatoid arthritis at the same time, accompanied by a high level of anti-ribonucleoprotein antibodies (anti-U1RNP). The disease was described more than 40 years ago by Sharp et al. During recent years, many efforts to better understand clinical and serological features of MCTD have been made. Diagnosis of MCTD can be difficult. Obligatory international diagnostic criteria are required to be fulfilled. Several versions of such criteria have been proposed, but the most widely used one was described by Kasukawa. There is no consensus about treatment – a choice of drugs depends on symptoms. We present a case of a 10-year-old girl with sclerodactyly and trophic damages of fingers accompanied by symptoms of Raynaud's phenomenon. After an almost 2-year course of the disease, a diagnosis of MCTD has been established.

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“…Since then, the concept of MCTD has persisted, but it is still debated whether the disease is a distinct entity or represents an overlap between systemic sclerosis (SSc), systemic lupus erythematosus (SLE) and polymyositis or dermatomyositis (PM/DM) 2–5. MCTD is characterised by serum autoantibodies targeting the U1-ribonucleoprotein6–8 and distinct clinical features which include Raynaud's phenomenon,1 9–12 ‘puffy’ or swollen hands,10–12 arthritis,10–13 pleuritis,10–12 pericarditis,10–12 myositis,10 14–16 oesophageal dysmotility,10–12 pulmonary hypertension8 11 17 18 and interstitial lung disease (ILD) 8 11 19–24. Many of these clinical features are present in other diseases, but their combined presence and a positive antiribonucleoprotein (RNP), appear to be unique for MCTD.…”

Section: Introductionmentioning

confidence: 99%