Adult dermatomyositis with treatment-resistant oropharyngeal dysphagia — SCE Rheumatology MCQ

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ModerateDermatomyositisAdult dermatomyositis with treatment-resistant oropharyngeal dysphagiaSCE Rheumatology

A 47-year-old woman with biopsy-confirmed dermatomyositis presented 7 weeks ago with severe proximal weakness and a creatine kinase of 7,400 IU/L. She received intravenous methylprednisolone followed by oral prednisolone 60 mg daily and methotrexate 20 mg weekly. Limb power has improved from MRC grade 3 to grade 4+, and creatine kinase is now 620 IU/L. Despite this, she has developed worsening nasal regurgitation, coughing during meals and a 5 kg weight loss. Fibreoptic endoscopic evaluation of swallowing shows impaired pharyngeal propulsion, substantial liquid residue and aspiration. Structural obstruction and infection have been excluded. Nasogastric feeding, swallowing therapy and aspiration precautions have been instituted. Which immunomodulatory escalation should now be prioritised?

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Correct answer: A — Intravenous immunoglobulin

This patient has objectively confirmed, aspiration-producing dermatomyositis-associated dysphagia that is progressing despite high-dose glucocorticoids and methotrexate. Improvement in creatine kinase and appendicular muscle strength does not establish control of bulbar disease. British Society for Rheumatology guidance specifically recommends intravenous immunoglobulin for idiopathic inflammatory myopathy-associated dysphagia resistant to other treatment. Its prioritisation is reinforced here by the immediate risks of aspiration and malnutrition. Enteral nutrition, aspiration precautions and specialist swallowing rehabilitation should continue concurrently. Rituximab is a credible treatment for refractory inflammatory myopathy, but the UK guideline contains a manifestation-specific recommendation for intravenous immunoglobulin in treatment-resistant dysphagia. Cyclophosphamide may be selected for severe refractory disease, particularly major myositis-associated interstitial lung disease, which is not present. Mycophenolate mofetil and tacrolimus are recognised glucocorticoid-sparing therapies and may be useful for persistent muscle, cutaneous or pulmonary disease; however, adding another conventional immunosuppressant is less directly supported for this immediately threatening bulbar manifestation. Evidence from a cohort using fibreoptic endoscopic evaluation also supports adding intravenous immunoglobulin to glucocorticoids and conventional immunosuppression in refractory myositis-associated dysphagia.

Reference: British Society for Rheumatology guideline on management of paediatric, adolescent and adult patients with idiopathic inflammatory myopathy (5 May 2022) — https://pubmed.ncbi.nlm.nih.gov/35355064/ BSR launches new myositis guideline (31 March 2022) — https://www.rheumatology.org.uk/news/details/BSR-launches-new-myositis-guideline Long-term efficacy of adding intravenous immunoglobulins as treatment of refractory dysphagia related to myositis: a retrospective analysis (2021) — https://pubmed.ncbi.nlm.nih.gov/32911543/