BACKGROUND: There are five major types of inflammatory myopathies (IM), including dermatomyositis, polymyositis, immune-mediated necrotizing myopathy, antisynthetase syndromes, and inclusion body myositis. Grouped together, the incidence of IM is >4 cases/100,000 with a prevalence of 14-32/100,000. Type-specific IM diagnoses are based on the pattern of muscle weakness and the results of electromyographs, MRIs, muscle biopsies, and measurements of myositis-specific autoantibodies1. We report the case of an elderly man with an inflammatory myopathy whose presenting complaint was dysphagia. OBJECTIVE: To review the clinical and laboratory manifestations of inflammatory myopathy-associated dysphagia. CASE REPORT: The patient is an 81-year-old retired Navy aviator who presented with a chief complaint of difficulty in swallowing both solids and liquids of several months duration. The dysphagia was associated with bouts of coughing, the expectoration of white foamy sputum, increased production of saliva, a weight loss of 5 kg, generalized myalgias, and worsening fatigue and weakness. The patient had a past medical history of essential hypertension, hyperlipidemia, autoimmune thyroiditis, V617F JAK2 + essential thrombocytosis, white matter microangiopathy, and a small lacunar infarction of the right caudate nucleus. There was no family history of autoimmune disease. On neurological examination, testing of cranial nerves II-XII and sensation to light touch, pin, temperature, and vibration was normal. The shoulder and upper arm strength was diminished bilaterally without evident muscle tenderness, atrophy or fasciculations. The patient could not rise from the supine position without assistance nor keep his arms elevated above his head without fatiguing. The strength in his lower extremities was normal. Deep tendon reflexes were normal and plantar responses were flexor. He had a single Gottron’s nodule and a faint erythematous rash involving his scalp, back and forearms. His Myositis Disease Activity Assessment (MDAAT) is shown in Table 1. Table 1. Myositis Disease Activity Assessment Tool (MDAAT) Findings on laboratory assessment included a normochromic normocytic anemia, thrombocyth-emia, a positive FANA IgG titer of 1:1280, and elevated levels of creatinine phosphokinase (1,373 U/L), aldolase (25.8 U/L), and antibodies to Mi-2 alpha, Mi-2 beta, MDA-5 and NXP-2 antigens. Anti-doubled stranded DNA, anti-RNP, anti-SM, anti-Jo-1, anti-SRP, anti-TIF-1ƴ and anti-synthetase antibody titers were negative. A modified barium swallow revealed severe oropharyngeal dysfunction with aspiration. An electromyograph, a muscle biopsy, and a MRI of cervical spinal muscles were indicative of an inflammatory myopathy. DISCUSSION: Dysphagia as a result of weakness of the oropharyngeal, laryngeal and esophageal musculature has been reported to develop in 10% to 73% of patients with inflammatory myopathy (IM) at some time during the course of their disease, and is most frequently seen in those with inclusion body myositis or malignancy- associated dermatomyositis. In a Mayo Clinic review of 62 patients with IM-associated dysphagia, 42% had inclusion body myositis, 29% dermatomyositis, 15% polymyosi-tis, and 15% an overlap syndrome. Dysphagia as the presenting complaint was most common in patients with inclusion body myositis (42%) followed by polymyositis and the overlap syndrome (11% each). IM-associated dysphagia had a mortality rate of 31% – 64% with the highest incidence of death occurring in patients with percutaneous endoscopic gastrostomies, an indirect measure of the severity of dysphagia. Death was most commonly the result of recurrent aspiration2. In a study of 92 patients with adult-onset dermatomyositis, Mugii and associates found that dysphagia was most prevalent in elderly patients, in males, and in patients with internal malignancies and/or elevated anti-TIF-1ƴ antibody levels3. In keeping with this report, the presented case is an elderly man with clinical and immunological evidence supporting a diagnosis of dermatomyositis, including an erythematous rash, a Gottron’s sign, and anti-Mi-2, anti-MDA-5, and anti-NXP-2 antibodies – autoantibodies associated with myositis, a classic dermatomyositis rash, and possible malignancy (see Table 2). Table 2. Patient’s myositis-specific autoantibodies* Agents most commonly used and reported to be beneficial in treating IM-associated dysphagia include prednisone, azathioprine, methotrexate, intravenous immunoglobulin, hydroxychloroqui-ne, and mycophenolate mofetil. Interventional procedures include enteral feeding and cricopharyngeal myotomies and dilations2. Inclusion body myositis is often recalcitrant to immunosuppressive treatment and is more likely to require interventional measures4. Our patient’s dysphagia is improving on methotrex-ate and prednisone. It is important to note that myositis patients presenting with complaints of dysphagia and weakness may be misdiagnosed as having amyotrophic lateral sclerosis, a disease in which serum CPK levels may be elevated and bulbar onset is common.
Search from 250 M+ research papersSearch from 250 M+ research papers
Mar 1, 2024
Jiaqi Liu + 6
Open Access