Rarity Redefined: Anti-Ku Antibody–Associated Myopathy In a Child

Authors

  • Sabavath Arun Senior Resident, Department of Pediatric, All India Institute of medical Science, Raebaerli Author
  • Shivangi Sinha Junior Resident, Department of Pediatric, All India Institute of medical Science, Bhopal Author
  • Shivani Khandelwal Junior Resident, Department of Pediatric, All India Institute of medical Science, Raebaerli Author

DOI:

https://doi.org/10.69980/vrhs1f47

Keywords:

Anti-Ku antibody, Inflammatory myopathy, pediatric myositis, autoimmune myopathy, rare case

Abstract

Background                                                                                                

Anti-Ku antibody–associated inflammatory myopathy is a rare autoimmune disorder, predominantly described in adults and only rarely reported in pediatric patients.

Case-Presentation

An 8-year-old male presented with a one-month history of high-grade fever followed by progressive proximal muscle weakness, leading to difficulty in walking and performing overhead activities. There was no history of rash, joint symptoms, or trauma. Examination revealed symmetric proximal muscle weakness.

Management & Outcome

Laboratory evaluation showed markedly elevated muscle enzymes (CK, AST, LDH) and inflammatory markers. Autoimmune workup revealed positivity for anti-Ku antibodies. MRI of lower limbs demonstrated T2/STIR hyperintensities suggestive of inflammatory myopathy. The patient was treated with pulse methylprednisolone followed by oral corticosteroids and methotrexate, resulting in significant clinical improvement and restoration of functional mobility.

Conclusion

This case highlights a rare early presentation of anti-Ku myopathy in childhood. Early recognition using serological and radiological correlation and prompt immunosuppressive therapy can significantly improve outcomes.

References

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Published

2026-06-17