A complex presentation of anti-NXP2 antibody positive inflammatory myositis with peripheral neuropathy

Idiopathic inflammatory myositis (IIM) encompasses a group of rare autoimmune disorders characterized by muscle inflammation and weakness. This case report details a rare association of IIM with neuropathy in a 55-year-old woman at presentation referred to as neuromyositis.

The patient had rapidly progressing proximal muscle weakness, difficulty in swallowing, and respiratory muscle weakness. Clinical examination, laboratory tests, imaging studies and histopathological examination confirmed the diagnosis of inflammatory myositis and axonal neuropathy. The coexistence of myositis and neuropathy suggests a complex autoimmune process affecting both muscles and peripheral nerves. Histological findings revealed myofiber necrosis, myophagocytosis, and neuropathic changes, indicating a potential common underlying mechanism. Our patient improved with biologic therapy. This case report emphasizes the complexity of neuromyositis and the importance of a multidisciplinary approach for accurate diagnosis and management.