[Pubmed] Successful treatment with eculizumab in refractory myasthenia gravis with both anti-AChR and anti-LRP4 antibodi

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J Neuroimmunol. 2026 Apr 12;417:578934. doi: 10.1016/j.jneuroim.2026.578934. Online ahead of print.

ABSTRACT

INTRODUCTION: Generalized myasthenia gravis (gMG), an antibody-induced autoimmune disorder of the neuromuscular junction, is characterized by fluctuating skeletal muscle weakness and fatigability. Approximately 85% of patients with MG have antibodies against the acetylcholine receptor (AChR), 5-10% against muscle-specific kinase receptor, and 1-5% against lipoprotein-related protein 4 (LRP4). In addition to the detection of antibodies providing insight into the diagnosis and prognosis of gMG, it also affects the choice of treatment. In the majority of cases, gMG can be managed effectively using a tailored regimen of acetylcholinesterase inhibitors, immunosuppressive medication, and thymectomy. However, existing treatments fail to adequately manage the manifestations in 10-15% of individuals with gMG (refractory gMG). Eculizumab, a recombinant humanized monoclonal antibody that specifically targets the complement protein C5, is approved for anti-AChR-positive gMG patients. Anti-AChR antibodies, anti-LRP4 antibodies, and anti-Titin antibodies all have the capacity to activate the complement protein signaling pathway.

CASE PRESENTATION: We report two cases of refractory gMG with multiple antibody positivity. The first case, an 82-year-old male patient, was positive for anti-AChR, anti-LRP4, and anti-Titin antibodies; the second case, a 67-year-old female, was positive for anti-AChR and anti-LRP4 antibodies. Both cases of refractory gMG responded well to eculizumab treatment.

CONCLUSION: This report of highlights two cases of refractory gMG with multiple antibody positivity (both patients had anti-AChR and anti-LRP4 antibodies) that responded to eculizumab. These findings warrent further experience with eculizumab treatment in gMG patients with multiple antibody positivity.

PMID:42035597 | DOI:10.1016/j.jneuroim.2026.578934


Source: https://pubmed.ncbi.nlm.nih.gov/4203559 ... t6+133c1fe