It is rare for two myositis-specific antibodies to co-exist in patients with immune-inflammatory myopathy. This overlap challenges the assumption of mutual exclusivity and may lead to conflicting diagnostic and therapeutic considerations. In this case, a 63-year-old female from the Philippines initially presented with chronic, progressive, and symmetrical proximal muscle weakness without cutaneous manifestations. Electrodiagnostic evaluation revealed a myopathic process while total creatine kinase levels were only mildly elevated. The myositis-specific antibody panel identified the presence of two distinct antibodies: anti-CN1A, which is a biomarker for inclusion body myositis, and anti-NXP2, which is specific to dermatomyositis. A muscle biopsy was performed, revealing fascicular atrophy, a characteristic feature of dermatomyositis. These findings presented a unique diagnostic challenge as dermatomyositis typically responds to immunosuppressive therapies, while inclusion body myositis is resistant to such treatments. This case illustrates the diagnostic complexity of overlapping myositis-specific antibodies and emphasizes the importance of integrating clinical, serological, and histopathological findings.
Cope et al. (Sun,) studied this question.