Background: Myasthenia gravis(MG), a chronic T-cell-dependent autoimmune disease impacting the postsynaptic membrane neuromuscular junction, manifests as exertional muscle fatigability.
Objective: C5-complement inhibitor, Ravulizumab prevents the cascade leading to the destruction of postsynaptic neuromuscular junction, causing muscle weakness in patients with anti-acetylcholine receptor (AChR) antibody-positive generalized myasthenia gravis. We describe the long-term effect of Ravulizumab in three patients with MG receiving concomitant immunomodulatory drugs.
Methods: We retrospectively reviewed all the patients at our clinic with MG on Ravulizumab.
Results:
Case 1: A 38-year-old woman with acetylcholine receptor antibody-positive generalized MG, ocular predominant, non-thymoma, diagnosed in 2016, with acute exacerbations of right eye ptosis, worsening limb weakness, and shortness of breath, previously on Prednisone, Mycophenolate, Azathioprine, Immunoglobulins, and Efgartigimod. The patient reported symptom reduction, improved MG-ADL (8 to 5), and Morse Fall Scale (60 to 15). No exacerbations were observed at the 8-month follow-up after starting Ravulizumab with Pyridostigmine ER 180 mg in May 2023.
Case 2: A 68-year-old male with long-standing generalized myasthenia gravis (MG) initially managed with steroids, pyridostigmine, azathioprine, and immunoglobulin. Due to persistent symptoms, Eculizumab was introduced but it caused adverse effects. Switching to Ravulizumab in December 2022, alongside azathioprine 50 mg, resulted in stable clinical improvement. At the 11-month follow-up, the patient achieved an MG-ADL Score of 0, resuming normal activities.
Case 3: A 32-year-old woman with Hashimoto thyroiditis and myasthenia gravis diagnosed at age 15 experienced an average of 2 exacerbations per year despite being on steroids, immunosuppressant, and plasmapheresis. Due to scheduling difficulties, in August 2023, she switched to Ravulizumab infusions every 8 weeks with immunoglobulins as needed. The change led to symptomatic improvement, no exacerbations, and a stable MG-ADL score of 6.
Conclusions: This case series suggests Ravulizumab as a potential chronic treatment for AChR ab-positive MG, with extended 7-8-week convenient infusion intervals, symptom-free intervals, stabilizing symptoms, and improving MG-ADL scores. We noted decreased exacerbations and no significant adverse effects; however, continuous monitoring is essential.