Title : Anti-NXP2 dermatomyositis in adults: A severe dysphagic form
Abstract:
Introduction: Dermatomyositis is a rare idiopathic inflammatory myopathy. Anti-NXP2 antibodies define a distinct and often severe subtype characterized by rapidly progressive proximal weakness, dysphagia, and an increased risk of systemic complications. Early recognition allows timely immunosuppressive treatment and improves outcomes. Observation: A 57-year-old woman with a history of uterine fibroids and chronic constipation presented with rapidly progressive proximal muscle weakness affecting all four limbs. She also developed severe dysphagia requiring nasogastric feeding. Physical examination revealed subcutaneous edema and erythematous skin lesions. Creatine kinase level was elevated at 4400 U/L. Initial myositis panel was negative. A subsequent extended immunologic panel identified anti-NXP2 antibodies. The overall presentation was compatible with anti-NXP2 dermatomyositis. Discussion: Anti-NXP2 dermatomyositis is associated with severe muscle involvement, dysphagia, and increased systemic risks. Diagnosis may be delayed when initial myositis panels are negative. Detection of specific antibodies guides prognosis and treatment. Early initiation of corticosteroids and immunosuppressive therapy is essential to prevent complications.
Conclusion: Anti-NXP2 dermatomyositis should be suspected in rapidly progressive weakness with dysphagia. Prompt antibody testing and early immunosuppression improve functional outcomes
