From Onset to Remission: Predictors of Trajectory and Personalized Risk in a Large French Myasthenia Gravis Cohort
Elena Faedo1, Sarah Souvannanorath2, Thierry Gendre3, Fatima Ikheteah2, Alice Dormeuil3, Valentine Perrain3, Gianmarco Severa4, Iman Tahiri2, Alban Gravier3, Tarik Nordine3, Violaine Planté-Bordeneuve3, Jean-Pascal Lefaucheur3, Alain Créange3, François-Jérôme Authier4, Edoardo Malfatti4
1Reference Center for Neuromuscular Disorders, APHP Henri Mondor University Hospital, Créteil, France and Department of Neurosciences, Rehabilitation, Ophthalmology, Genetic and Maternal and Infantile Sciences (DINOGMI), University of Genoa, Genova, Italy, 2Reference Center for Neuromuscular Disorders, APHP Henri Mondor University Hospital, Créteil, France, 3Department of Neurology, APHP Henri Mondor University Hospital, Créteil, France, 4University Paris Est Créteil, Inserm, U955, IMRB, F-94010 Créteil, France
Objective:

This retrospective cohort study aimed to delineate disease trajectories, antibody subtypes, and long-term outcomes in a large French Myasthenia gravis (MG) cohort.

Background:
MG is a heterogeneous autoimmune disorder with variable phenotypes and outcomes. Real-world studies are still scarce, especially regarding the trajectories of generalized, ocular, and seronegative cases in the era of new targeted therapies.
Design/Methods:

We retrospectively analyzed 119 MG patients followed over 18 year at the Mondor University Hospital neuromuscular reference center. Demographics, clinical, antibody status, thymic pathology, treatment patterns, and long-term outcome were collected. Outcomes included generalization, relapse, remission, and mortality. Kaplan-Meier, Cox regression, and logistic regression identified predictors.

Results:

The cohort included 77% (N= 92) anti-AChR, 5% (N=6) anti-MuSK, and 17.6% (N=21) seronegative patients. Generalized MG accounted 76% (N=90) , ocular MG 24% (N=29). Relapses occurred in 72% (N=86). About half of ocular MG later generalized, often after two years, with higher generalization risk in female and late-onset MG. Severity at diagnosis and of the first relapse independently predicted long-term burden. Remission (MGFA 0–I) was achieved in 42% (N=50), more frequently in ocular and relapses-free patients. Seronegative patients had longer relapse-free intervals and a higher proportion of sustained ocular disease. At last follow-up, 31% (N=37) used anticholinesterase monotherapy, 62% (N=74) received corticosteroids (45% discontinued, N=33), and 80% (N=95) received azathioprine or mycophenolate. Rituximab, FcRn and complement inhibitors were used in 15% (N=18). Mortality was comparable to the general population.

Conclusions:

Early disease severity and occurrence of the first relapse were strong predictors of long-term outcomes in MG, underscoring the importance of early, risk-adapted management. Ongoing vigilance for generalization remains essential, particularly in ocular-onset and elderly-onset cases. In this real-world cohort, both conventional therapies and newer agents targeting FcRn or complement pathways were associated with high remission rates and facilitated corticosteroid tapering.

10.1212/WNL.0000000000216950
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