“The side effects of prednisone are getting really tough.”
Sabrina T. is 35, with generalized myasthenia gravis that has persisted through
multiple immunosuppressive therapies. She first presented with partial right‑sided ptosis
and intermittent horizontal diplopia, then developed generalized weakness, fatigue, worsening
ptosis and intermittent dysphagia. Initial treatment was prednisone 30 mg/day and
pyridostigmine 240 mg/day.
She recently had a myasthenic exacerbation — dyspnoea, generalized weakness,
dysarthria and severe dysphagia — and was admitted to intensive care with respiratory
compromise requiring non‑invasive ventilation. Intravenous immunoglobulin brought
clear improvement, and she moved out of the ICU into physical therapy.
She is back in your office on prednisone 40 mg/day and mycophenolate mofetil
3 g/day, with mild worsening of bulbar symptoms and occasional shortness of breath,
improving with rest. On examination: thin skin with mild bruising and mild facial rounding,
consistent with steroid use.
Weight · BMI
97 kg · 32.4
Disease duration
7 years
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Case · Workup
She feels a little better. The scores say otherwise.
MG‑ADL has risen from 8 to 14 — on a scale of 0 to 24, where
higher means poorer function. QMG is 26, indicating significant muscle weakness across
multiple domains. MG‑QoL15r is 24 of 30.
MGFA classification is IIIb: moderate generalized weakness with bulbar
involvement. She has moved from IIIa to IIIb over the course of several years, with
two myasthenic crises in her record.
Applying MG‑specific scores is what allows mild and moderate disease to be
told apart from highly active — including refractory — MG
(Wiendl H et al, 2023).
AChR antibody
3.1 nmol/L
EMG decrement
40.3 %
Case · Decision
What would you add?
Symptoms persist and worsen on pyridostigmine, prednisone and further
immunosuppression. Choose one.
Select an option to continue
Case · Verdict
Six are open to her. One has closed.
ARavulizumabAppropriateFDA 2019 · EMA 2019 · add‑on to standard therapy in adults who are AChR antibody‑positive (Ultomiris FDA, 2019; Ultomiris SmPC EMA, 2019).
BEculizumabAppropriateFDA 2007 · EMA 2007 · refractory gMG from age 6, AChR antibody‑positive (SOLIRIS FDA, 2023; SOLIRIS SmPC EMA, 2007).
CZilucoplanAppropriateFDA 2023 · EMA 2023 · add‑on in adults who are AChR antibody positive (Zilbrysq FDA, 2023; Zilbrysq SmPC EMA, 2023).
DEfgartigimod alfa/hyaluronidaseAppropriateFDA 2021 · EU 2022 · add‑on in adults who are AChR‑Ab‑positive (Wiendl H et al., 2023).
ERozanolixizumabAppropriateFDA 2023 · EMA 2024 · add‑on in adults who are AChR or MuSK antibody positive (Rystiggo FDA, 2023; Rystiggo SmPC EMA, 2024).
FIntravenous immunoglobulinAppropriateSecond‑line in the German guidelines for severe or refractory gMG. But it would require frequent hospital visits, which does not align with Sabrina's desire for less burdensome options (Wiendl H et al, 2023).
GThymectomyNot appropriateRecommended between ages 18 and 65, ideally within two years of diagnosis and no later than five. With a greater than 5‑year history it is not a valid option — and she has refused it (Wiendl H et al, 2023).
Case · Evidence
Sabrina meets the definition of highly active gMG.
Highly active gMG, including refractory gMG, is defined as any of the following
(Wiendl H et al, 2023). She meets them.
01Moderate to high MGFA classification, class IIb or
above, and / or two or more severe exacerbations or myasthenic crises within a
year of diagnosis requiring IV immunoglobulin or plasmapheresis, despite sufficient
disease‑modifying and symptomatic therapy.
02Ongoing symptoms affecting daily functioning, class IIa or
above, with at least one severe exacerbation or crisis in the past year, despite
adequate treatment.
03Persistent mild to moderate symptoms affecting daily life,
class IIa or above, for over two years, even with appropriate treatment.
ThenKeep measuring. Monitoring should be continuous, with
physical examination, MGFA classification and the MG‑specific tools — QMG,
MG‑ADL and MG‑QoL15 — used to track change and guide adjustment
(Narayanaswami P et al, 2020).
AndKeep moving. Physical activity is generally recommended
to help prevent muscle weakness and reduce comorbidity risk, adapted to capability with
frequent rest (Gilhus NE et al, 2024).