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Guidelines and recommendations
The international consensus guidance for the management of MG uses the post-intervention status (PIS) classification introduced by the Myasthenia Gravis Foundation of America (MGFA) to define the treatment goals in MG.3 More specifically, it recommends that the goal of treatment is to achieve a MGFA-PIS of minimal manifestations (MM) or better, with no more than grade 1 Common Terminology Criteria for Adverse Events (CTCAE) medication side effects.3 The MM status is defined as a patient having no symptoms of functional limitations from MG but some weakness upon examination of some muscles.4
In addition to the international consensus,3 local recommendations for the management of patients with MG are available and continue to evolve,5–9 and have been considered when summarising the treatment options presented here. The various local recommendations provide further insights into the treatment goals for MG.5,6,8 For instance, the German Neurological Society suggests that the treatment goal for MG is to achieve the best possible disease control while restoring the patient’s quality of life.5 Furthermore, a recent study reported that Minimal Symptom Expression (MSE) is an appropriate treatment goal, which can help guide long-term treatment strategy in patients with generalised MG (gMG).10
In about 5–15% of patients with gMG, the disease is deemed refractory to treatment with acetylcholinesterase (AChE) inhibitors and/or conventional immunosuppressive (IS) therapies.11 These patients continue to have gMG symptoms despite multiple therapy attempts or experience intolerable treatment-related adverse effects.5,11 Uncontrollable symptoms may result in a number of clinical and functional sequelae that increase patients’ burden of disease.5,11

Persistent symptoms relevant to daily living (≥ MGFA class IIa) and/or at least two recurrent severe exacerbations/myasthenic crises with a need for therapeutic intervention – intravenous immunoglobulin (IVIg), plasma exchange (PE), immunoadsorption (IA) – within one year after diagnosis despite adequate course-modifying and symptomatic therapy.
Or

Persistent symptoms relevant to daily living (≥ MGFA class IIa) and severe exacerbation/myasthenic crisis within the past year despite adequate course-modifying and symptomatic therapy.
Or

Persistent symptoms relevant to daily living even mild/moderate (≥ MGFA class IIa) for more than two years despite adequate course-modifying and symptomatic therapy.

Symptomatic therapy in patients with (highly) active MG (including refractory MG) can be supplemented by a number of biologics – such as complement inhibitors, molecules inhibiting receptors expressed by various immune cells or antibodies for depletion of B cells.1,5 The choice of the specific therapy depends on the MG subtype.5 Second-line treatment can involve IVIg, PE or IA, while other procedures may also be considered on a case-by-case basis.5
Between 10–20% of patients with MG have a myasthenic crisis, where risk is highest in the first 2–3 years after disease onset.12 Short-term IS therapy with PE or IVIg is often used as first-line treatment to manage myasthenic crises.3,12
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AChE, acetylcholinesterase; CSR, complete stable remission; CTCAE, Common Terminology Criteria for Adverse Events; gMG, generalised myasthenia gravis; IA, immunoadsorption; IVIg, intravenous immunoglobulin; MG, myasthenia gravis; MGFA, Myasthenia Gravis Foundation of America; MM, Minimal Manifestations; MSE, Minimal Symptom Expression; PE, plasma exchange; PIS, post-intervention status; PR, pharmacologic remission.
Gilhus NE, et al. Myasthenia gravis. Nat Rev Dis Primers. 2019;5(1):30. Riley TR, et al. An update of the pharmacological treatment options for generalized myasthenia gravis in adults with anti-acetylcholine receptor antibodies. Am J Health Syst Pharm. 2023:zxad035.  Sanders DB, et al. International consensus guidance for management of myasthenia gravis: Executive summary. Neurology. 2016;87(4):419–425.  Jaretzki A, 3rd, et al. Myasthenia gravis: Recommendations for clinical research standards. Task Force of the Medical Scientific Advisory Board of the Myasthenia Gravis Foundation of America. Ann Thorac Surg. 2000;70(1):327–334. Wiendl H, et al. Guideline for the management of myasthenic syndromes. Therap Adv Neurol Disord. 2023;16:17562864231213240. Evoli A, et al. Italian recommendations for the diagnosis and treatment of myasthenia gravis. Neurol Sci. 2019;40(6):1111–1124. Li Z-Y. China guidelines for the diagnosis and treatment of myasthenia gravis. Neuroimmunol Neuroinflammation. 2016;3:1–9. Murai H, et al. The Japanese clinical guidelines 2022 for myasthenia gravis and Lambert–Eaton myasthenic syndrome. Clin Exp Neuroimmunol. 2023;14:19–27. Sussman J, et al. Myasthenia gravis: Association of British Neurologists' management guidelines. Pract Neurol. 2015;15(3):199–206. Uzawa A, et al. Minimal symptom expression achievement over time in generalized myasthenia gravis. Acta Neurol Belg;. 2023. doi: 10.1007/s13760-13022-02162-13761. Online ahead of print. Last accessed: September 2023. Harris L, et al. Longitudinal analysis of disease burden in refractory and nonrefractory generalized myasthenia gravis in the United States. J Clin Neuromuscul Dis. 2020;22(1):11–21. Huang Y, et al. Patients with myasthenia gravis with acute onset of dyspnea: Predictors of progression to myasthenic crisis and prognosis. Front Neurol. 2021;12:767961.