The authors declare that they have no competing interests.
LN wrote the discussion, completed edits, submitted the article and did much of the literature review. EG completed most of the edits and completed the remainder of the literature review. AN wrote half of the case report section. AW created the figures, collected laboratory data and formulated the table. LM wrote the abstract and a portion of the case report. All authors read and approved the final manuscript.
Severe dysphagia may occur in the immune mediated necrotizing myopathies (IMNM). Neck swelling and severe dysphagia as the initial symptoms upon presentation has not been previously described.
A 55-year-old male with a 4 week history of neck swelling, fatigue, dysphagia, myalgias, night sweats, and cough was admitted for an elevated CK. He underwent extensive infectious and inflammatory evaluation including neck imaging and muscle biopsy.
Neck CT and MRI showed inflammation throughout his strap muscles, retropharyngeal soft tissues and deltoids. Infectious work up was negative. Deltoid muscle biopsy demonstrated evidence of IMNM. Lab tests revealed anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) antibodies confirming the diagnosis of HMGCR IMNM.
HMGCR IMNM is a rare and incompletely understood disease process. Awareness of HMGCR IMNM could potentially lead to earlier diagnosis, treatment and improved clinical outcomes as disease progression can be rapid and severe.
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Mammen AL et al. Increased frequency of DRB1* 11: 01 in anti–hydroxymethylglutaryl-coenzyme A reductase–associated autoimmune myopathy. Arthritis Care Res. 2012;64:1233–7. CrossRef
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- A case report of autoimmune necrotizing myositis presenting as dysphagia and neck swelling
Linh Q. Ngo
Andrew G. Wu
Matthew A. Nguyen
Lauren E. McPherson
- BioMed Central
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