Epidemiological Study of Treatment Approaches in AChR-Antibody Positive Generalized Myasthenia Gravis in Russia

Patient-reported MG-ADL total score (range 0-24; higher=worse). Report descriptive statistics at each time point and change from Baseline (visit value minus Baseline). Scores are abstracted from routine care records; only total scores documented in source are collected.

Physician-reported QMG total score (0-39; higher=worse). Metrics: absolute score and change from baseline. Summarized descriptively. Collected only if present in routine practice records.

Proportion of participants in each MGFA class (I, IIa/IIb, IIIa/IIIb, IVa/IVb, V) at each time point. Also report proportion improving (lower class) or worsening (higher class) versus Baseline. MGFA class is abstracted only if recorded in routine practice.

Patients experiencing ≥1 hospitalization for any cause during prospective follow-up. Metric: count and proportion (percentage) of patients with ≥1 event. Each participant's status (yes/no) assessed across the follow-up period.

Total days hospitalized for any cause per participant during prospective follow-up. Metric: sum of hospital-day durations per participant; summarized descriptively.

Patients with ≥1 hospitalization attributed to gMG during follow-up. Categorize reasons: related to MG exacerbation (myasthenic crisis) vs not related (thymectomy, investigation, social reason, other). Record ICU admission for gMG-related hospitalizations (yes/no). Report count and proportion (percentage) of affected patients overall and by categories.

Total days hospitalized for gMG-related reasons per participant during prospective follow-up. Metric: sum of hospital-day durations for gMG-related stays; summarized descriptively.

Interval between the date of first MG symptoms/suspicion (if known) and the date of generalized MG (gMG) diagnosis. Metric: duration in months (or days if available); summarized descriptively. Evaluated only for patients with a documented onset date.

Proportion of participants with ≥1 prior misdiagnosis and distribution by category (myopathies, polyneuropathies, Lambert-Eaton, mitochondrial encephalomyopathies, congenital myasthenic syndromes, cerebrovascular accidents, brainstem encephalitis, brain tumors, ALS).

Proportion with each examination and latest available result: AChR-Ab (required positive), CT/MRI chest/mediastinum, brain/spinal MRI, ENMG, single-fiber EMG, RNS. Report counts, percentages (95% CI), and result categories if available.

Proportion with each test performed and latest result (positive/negative/unknown) as documented in routine care; report counts and percentages with 95% CI.

Proportion of patients receiving each therapy class: anticholinesterase (neostigmine), IVIG, corticosteroids (prednisolone/methylprednisolone), complement inhibitors (ravulizumab/eculizumab), diuretics, potassium, immunosuppressants (e.g., azathioprine, mycophenolate, cyclosporine), rituximab. Summarized as counts/percentages by class.

For each therapy class received, total days on therapy per participant during the assessment window; summarized descriptively.

Among CS users, average daily dose recorded in mg; convert methylprednisolone to prednisolone equivalent for analysis. Summarized with descriptive statistics.

Proportion of patients receiving plasmapheresis, plasma exchange, or plasmafiltration for gMG; summarized as counts and percentages.

Total treatment days per participant for plasmapheresis, plasma exchange, or plasmafiltration within the assessment window; summarized descriptively.

Proportion of patients who underwent thymectomy for gMG; summarized as counts and percentages.

Proportion of patients requiring invasive mechanical ventilation for gMG; summarized as counts and percentages.

Distribution of initial treatment components at diagnosis (same therapy categories as above); summarized as counts and percentages by regimen.

Proportion with ≥1 crisis and distribution by number of episodes (1, 2, ≥3) during the 12 months prior to enrollment; counts and percentages from medical records.

Proportion with ≥1 crisis and distribution by number of episodes (1, 2, ≥3) during prospective follow-up; counts and percentages from medical records.

Proportion of crisis episodes associated with predefined physical precipitants: aspiration pneumonitis, infection, perimenstrual state, pregnancy, sleep deprivation, surgery, environmental stressors, emotional stress, pain, temperature extremes, tapering of immune-modulating medications.

Proportion of crisis episodes associated with predefined medications: α-interferon; antibiotics (e.g., aminoglycosides, macrolides, quinolones, polymyxin); antiepileptics (e.g., gabapentin, phenytoin, carbamazepine, high-dose benzodiazepines); β-blockers; calcium channel antagonists; contrast media; magnesium; hormonal drugs (e.g., corticosteroids, ACTH, oral contraceptives, oxytocin, thyroid hormones); antithyroid agents; antiarrhythmics (e.g., procainamide, quinidine).

Change in MGFA class from the most recent pre-crisis assessment to the nearest post-crisis scheduled assessment; proportion improved, unchanged, or worsened.

Proportion of participants who initiated C5IT during observation; counts and percentages; with start/stop dates abstracted from records.

Among those who initiated C5IT, proportion with documented meningococcal vaccination before first dose; time from gMG diagnosis to first vaccination; record revaccinations. Report counts, percentages, and time-to-vaccination summary.

Among those who initiated C5IT, proportion with documented prophylactic antibiotic use prior to first dose; time from gMG diagnosis to first prophylactic use; record repeated prophylaxis. Report counts, percentages, and time-to-prophylaxis summary.