The Clinical Features, Muscle Pathology, and Role of Autophagy in Anti-Ku-Positive Patients

This study examined 6 patients with isolated anti-Ku antibodies, a rare condition found in 0.5% of 1,120 idiopathic inflammatory myopathy cases. Patients, averaging 47.6 years at onset, showed extramuscular symptoms like scleroderma, systemic lupus erythematosus, and hair loss. Muscle weakness was typically symmetrical and proximal, with some distal involvement. Muscle biopsies revealed neurogenic atrophy and myositis with vacuoles, indicating potential autophagy dysregulation. The study suggests that anti-Ku antibodies are linked to unique disease manifestations and highlights the need for further research to understand the role of autophagy in this condition.