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Inflammatory Myositis in a Child due to Anti-NXP2 Antibody, First Case Report from India

Ashok V Reddy Taallapalli1, Sumanth Shivaram1, Manisha Gupta1

  • 1Department of Neurology, National Institute of Mental Health and Neuro sciences (NIMHANS), Hosur Road, Bengaluru, Karnataka, India.

Neurology India
|July 22, 2022
PubMed

Insights

This study reports a rare case of anti-nuclear matrix protein 2 (NXP2) antibody-mediated inflammatory myositis in a child. Early diagnosis and immunomodulatory treatment led to significant improvement, highlighting the importance of NXP2 antibody testing.

Area of Science:

  • Pediatric Rheumatology
  • Neuromuscular Disorders
  • Autoimmune Diseases

Background:

  • Inflammatory myopathies are rare autoimmune disorders affecting muscles.
  • Anti-NXP2 antibody-associated myositis is a distinct subtype with specific clinical and imaging features.
  • Early diagnosis and treatment are crucial for managing pediatric inflammatory myopathies.

Observation:

  • A 10-year-old boy presented with proximal limb weakness, myalgias, contractures, and bulbar symptoms.
  • Elevated creatine phosphokinase (CPK) and positive anti-nuclear matrix protein 2 (NXP2) antibodies were detected.
  • Muscle MRI revealed active myositis with extensive signal changes in lower limb muscles.

Findings:

  • The patient showed significant clinical improvement following treatment with steroids, intravenous immunoglobulins (IVIg), and mycophenolate mofetil (MMF).
  • This case represents the first report of anti-NXP2 antibody-mediated inflammatory myositis from India.
  • The findings underscore the utility of NXP2 antibody testing in pediatric myositis.

Implications:

  • This case highlights the importance of considering anti-NXP2 antibody myositis in children with inflammatory muscle disease.
  • Prompt immunomodulatory therapy can lead to favorable outcomes in anti-NXP2 antibody-positive patients.
  • Further research is needed to understand the long-term prognosis and management strategies for this rare condition.

Keywords:
Anti-NXP 2 inflammatory myositiscapillaroscopymyositis with contractures

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