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Dermatomyositis-associated oropharyngeal dysphagia — SCE Rheumatology MCQ

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ModerateMusculoskeletalDermatomyositis-associated oropharyngeal dysphagiaSCE Rheumatology

A 46-year-old woman with anti-NXP2-positive dermatomyositis is treated with intravenous methylprednisolone followed by oral prednisolone and methotrexate. Eight weeks later, her creatine kinase has fallen from 7,800 IU/L to 420 IU/L and proximal limb power has improved from MRC grade 3 to grade 4+. However, she has developed worsening nasal regurgitation, dysphonia and recurrent coughing after drinking. Videofluoroscopy demonstrates impaired pharyngeal propulsion, reduced upper oesophageal sphincter opening and aspiration of thin fluids. CT of the thorax shows no interstitial lung disease. Speech and language therapy recommends temporary avoidance of oral feeding, and nasogastric feeding is commenced. She has normal renal function and no history of thrombosis or other contraindication to immunoglobulin therapy. Which additional immunomodulatory treatment is most appropriate?

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Correct answer: A — Administer intravenous immunoglobulin

Intravenous immunoglobulin is the best additional treatment. The decisive feature is objectively confirmed, aspiration-producing dermatomyositis-associated dysphagia that remains active despite high-dose glucocorticoids and a conventional steroid-sparing drug. Improvement in creatine kinase and limb power does not establish control of pharyngeal muscle disease. The NICE-accredited British Society for Rheumatology guideline specifically recommends considering intravenous immunoglobulin for active idiopathic inflammatory myopathy-associated dysphagia. Swallowing rehabilitation, aspiration precautions and enteral nutrition are required concurrently but do not treat the underlying inflammatory manifestation. Cyclophosphamide is a potent option for severe organ-threatening myositis, particularly rapidly progressive interstitial lung disease, which is absent. Rituximab may be used for refractory muscle, skin or pulmonary disease, but intravenous immunoglobulin has more directly applicable guidance and clinical evidence for severe dysphagia where a prompt response is desirable. Tacrolimus is particularly relevant to refractory myositis-associated interstitial lung disease and is not the preferred dysphagia-directed escalation here. Further methylprednisolone pulses might be justified during an acute, globally uncontrolled deterioration, but she has already received pulse therapy and now has persistent bulbar disease despite improvement in other disease domains. Her lack of major thrombotic or renal risk factors also removes important practical barriers to intravenous immunoglobulin.

Reference: British Society for Rheumatology clinical guidelines: paediatric, adolescent and adult idiopathic inflammatory myopathy (2022; current listing checked 19 August 2026) — https://www.rheumatology.org.uk/guidelines/clinicalguidelines/adultsratnfguideline/view 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/ Long-term efficacy of adding intravenous immunoglobulins as treatment of refractory dysphagia related to myositis: a retrospective analysis (2 March 2021) — https://pubmed.ncbi.nlm.nih.gov/32911543/