Efgartigimod Use in Guillain-Barré Syndrome and its Variants: A Systematic Review of Case Reports
Deep Chahodiya1, Krutika Katageri2
1Government medical college, Surat, 2Belagavi Institute of Medical Sciences
Objective:

The objective of this study is to analyse treatment outcomes and safety of efgartigimod in Guillain-Barré Syndrome (GBS) and its variants.

Background:

GBS is an immune mediated polyneuropathy, with treatment being IV immunoglobulins and plasma exchange. Efgartigimod, a neonatal Fc receptor blocker, is an emerging therapy option.


Design/Methods:

We searched PubMed, MEDLINE, Scopus, Embase and Scielo using keywords ”Guillain-Barré syndrome”, “Variants” and  “Efgartigimod” and retrieved 9 case reports/series. Two were excluded due to inaccessible text and 13 patients finalised.


Results:

Of 13 patients analysed, the mean age was 52 years, with 7 males (53.8%) and 6 females (46.1%). GBS variants included 1 patient with Miller-fisher syndrome (MFS), 6 with GBS/MFS overlap syndrome, 2 with Acute Motor Axonal Neuropathy (AMAN), 1 with Acute Motor Sensory Axonal Neuropathy (AMSAN), 2 with Acute Inflammatory Demyelinating Polyneuropathy (AIDP) and 1 with Myasthenia Gravis/Anti-Gq1b antibodies. 

The mean symptom onset to treatment time was 20.5 days, and Efgartigimod was administered in doses of 10mg/kg/week in 10 patients,  80mg/week in one and 800mg/week in two, with a mean of 1.3 cycles.

It was used as a first-line drug in 3 patients (23%), as rescue drug in 5 (38.4%) and as refractory drug in 5 (38.4%). Prior treatment in 11 patients was with IVIg alone (45.4%), IVIg and steroids (18.1%), Steroids alone (9.09%) and IVIg with Plasma Exchange (27.2%). Only one patient received concomitant therapy with IVIg. Clinical improvement was seen in all patients (100%), with the mean time to improvement being 15.6 days. Except for one upper respiratory infection,no serious adverse events or relapses occurred. Overall, the drug was well tolerated.


Conclusions:

Efgartigimod showed promising clinical improvement in GBS and can be considered as an adjunct in it’s treatment.


10.1212/WNL.0000000000217849
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