Ravulizumab in the Management of Refractory Myasthenic Crisis: Clinical and Ventilatory Evidence of Early Recovery-A Case Report.
Аннотация
INTRODUCTION
Generalized myasthenia gravis (gMG) is an autoimmune disorder impairing neuromuscular transmission, most commonly through anti-AChR antibodies that activate the complement cascade. This can lead to severe complications such as myasthenic crisis (MC), which often requires intensive care. While plasma exchange (PLEX) and intravenous immunoglobulins (IVIG) are standard first-line therapies, approximately 30% of patients may show suboptimal response. Ravulizumab, a long-acting C5 complement inhibitor, has been approved for anti-AChR-positive gMG, but data on its use in MC remain limited.
CASE PRESENTATION: We report a 62-year-old male with late-onset, anti-AChR-positive gMG who presented with refractory MC, unresponsive to five PLEX sessions and IVIG. After infectious disease evaluation, meningococcal prophylaxis, and antibiotic coverage, a single intravenous loading dose of ravulizumab (2700 mg) was administered on ICU Day 9.
CLINICAL RESPONSE: Marked clinical improvement was observed within 48 h, including reduction in ventilatory support (pressure support decreased from 16 to 6 cmH2O over five days), improved cough, secretion management, and eventual successful extubation on Day 17. By Day 21, the patient resumed oral feeding and was transferred out of ICU with stable respiratory function and neurological improvement.
CONCLUSION
This case suggests that ravulizumab may provide rapid and sustained benefit in anti-AChR-positive patients experiencing refractory MC. Complement inhibition led to early ventilatory and neuromuscular recovery despite prior treatment failure. These findings support further investigation of ravulizumab as rescue therapy in acute, treatment-resistant gMG exacerbations.
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История · было → стало
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