Impact and Management of Dysphagia in Inflammatory Myopathies

Zeng, Rachel; Schmidt, Jens

doi:10.1007/s11926-020-00950-3

Cited by 18 publications

(25 citation statements)

References 89 publications

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“…Anakinra, an IL-1 receptor blocker, had also shown mild short-term improvements in a subset of patients in the first series [ 27 ] and in a later study of 15 patients [ 28 ]. Because of the common presence of a cricopharyngeal bar, invasive procedures including balloon dilatation and myotomy can lead to improvement of symptoms in patients with serious dysphagia and weight loss prior to PEG insertion [ 20 , 29 – 33 ], although this effect was not long lasting after 5 years [ 17 ]. In our experience, Botox injections have not been helpful.…”

Section: Autoimmune Neurological Disorders With Dysphagiamentioning

confidence: 99%

Autoimmune Neurogenic Dysphagia

Stathopoulos

Dalakas

2021

Dysphagia

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Autoimmune neurogenic dysphagia refers to manifestation of dysphagia due to autoimmune diseases affecting muscle, neuromuscular junction, nerves, roots, brainstem, or cortex. Dysphagia is either part of the evolving clinical symptomatology of an underlying neurological autoimmunity or occurs as a sole manifestation, acutely or insidiously. This opinion article reviews the autoimmune neurological causes of dysphagia, highlights clinical clues and laboratory testing that facilitate early diagnosis, especially when dysphagia is the presenting symptom, and outlines the most effective immunotherapeutic approaches. Dysphagia is common in inflammatory myopathies, most prominently in inclusion body myositis, and is frequent in myasthenia gravis, occurring early in bulbar-onset disease or during the course of progressive, generalized disease. Acute-onset dysphagia is often seen in Guillain–Barre syndrome variants and slowly progressive dysphagia in paraneoplastic neuropathies highlighted by the presence of specific autoantibodies. The most common causes of CNS autoimmune dysphagia are demyelinating and inflammatory lesions in the brainstem, occurring in patients with multiple sclerosis and neuromyelitis optica spectrum disorders. Less common, but often overlooked, is dysphagia in stiff-person syndrome especially in conjunction with cerebellar ataxia and high anti-GAD autoantibodies, and in gastrointestinal dysmotility syndromes associated with autoantibodies against the ganglionic acetyl-choline receptor. In the setting of many neurological autoimmunities, acute-onset or progressive dysphagia is a potentially treatable condition, requiring increased awareness for prompt diagnosis and early immunotherapy initiation.

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Section: Autoimmune Neurological Disorders With Dysphagiamentioning

confidence: 99%

Autoimmune Neurogenic Dysphagia

Stathopoulos

Dalakas

2021

Dysphagia

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“…Motility in the pharyngeal and esophageal muscles can be investigated by videofluoroscopy, fiberoptic endoscopic evaluation of swallowing, esophageal manometry, or real‐time MRI. These techniques can be helpful for identifying the cause and location of the disturbance causing dysphagia, including any disturbance to the pharyngeal muscles or esophagus (7).…”

Section: Clinical Challengementioning

confidence: 99%

Expert Perspective: Management of Refractory Inflammatory Myopathy

undberg

2021

Arthritis & Rheumatology

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The idiopathic inflammatory myopathies (IIMs) are chronic disorders characterized by inflammation in skeletal muscle but also in other organs such as the skin, lungs, joints, gastrointestinal tract, and heart. The effect of immunosuppressive treatment varies between individual patients and between organ manifestations within the same individual. Many patients respond poorly to first-line treatment with glucocorticoids and other immunosuppressive agents such as methotrexate or azathioprine, with symptoms persisting in the muscles, skin, and lungs, leading to refractory disease. Management of refractory IIM is a clinical challenge, and a systematic approach is proposed to better understand the lack of treatment response, in order to guide disease management. The first step in the management of refractory IIM is to recognize whether remaining symptoms are caused by persistent inflammation in the affected tissue or whether the symptoms may be attributable to damage preceding inflammation. Thus, a second diagnostic examination is recommended. Second, in particular for patients with remaining muscle weakness, it is important to ascertain whether the diagnosis of myositis is correct or whether another underlying muscle disorder could explain the symptoms. Third, with confirmation of remaining inflammation in the tissues, a strategy to change treatment needs to be undertaken. Few controlled trials are available to guide our treatment strategies. Furthermore, different subgroups of patients may benefit from different therapies, and different organ manifestations may respond to different therapies. In this context, subgrouping of patients with IIM based on autoantibody profile may be helpful, as there are emerging data from open studies and case series to support the notion of a varying treatment response in different autoantibody-defined subgroups of IIM patients. Clinical challengeThe index patient, a 38-year-old woman, presented with weakness of the proximal leg muscles, elevated creatine kinase (CK) levels (5 times the upper limit of normal), signs of myopathy on electromyogram, and atrophy of the gluteus muscles on magnetic resonance imaging (MRI). A biopsy sample from the thigh muscle showed some fiber size variation and internal nuclei, but otherwise normal histopathology. She was diagnosed as having polymyositis (PM). Despite having received treatment with high-dose glucocorticoids in combination with azathioprine followed by cyclosporine (methotrexate treatment was excluded due to pregnancy planning) and despite a routine of regular physical exercise, the patient's muscle weakness progressed and she developed atrophy of her hamstring muscles-a refractory myopathy.

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“…Formal standardized patient-based questionnaires can be used as a screening tool in clinic to evaluate dysphagia initially and in follow-up to monitor treatment response. Several standardized swallowing questionnaires are available; of these, the Sydney Swallow Questionnaire and the Swallowing-Related Quality of Life (SWAL-QOL) have demonstrated high validity and reliability for the evaluation of neuromyogenic dysphagia [26,27]. Thus, we recommend that patients should perform one of these questionnaires prior to other evaluations in the presence of a caregiver (if present) to improve performance.…”

Section: Diagnostic Challengesmentioning

confidence: 99%

Dysphagia in Inclusion Body Myositis, A Disease of the Elderly

Goyal¹,

Mozaffar²

2021

OBM Geriatrics

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Inclusion body myositis is one of the most common acquired myopathies seen in the aging population. The muscle weakness is of insidious onset, frequently starting in the legs, but over time gradually progresses to eventually involve all skeletal muscles of the limbs, face, swallowing and respiratory muscles, leading to significant morbidity and mortality. Complications resulting from dysphagia are the leading cause of poor quality of life and mortality, while highly prevalent, are still underappreciated and underrecognized. This review highlights the importance of understanding the urgent need for recognizing and improving the evaluation, outcome measures and therapeutic options of dysphagia in inclusion body myositis patients.

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Impact and Management of Dysphagia in Inflammatory Myopathies

Cited by 18 publications

References 89 publications

Autoimmune Neurogenic Dysphagia

Autoimmune Neurogenic Dysphagia

Expert Perspective: Management of Refractory Inflammatory Myopathy

Dysphagia in Inclusion Body Myositis, A Disease of the Elderly

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