High-dose intravenous immunoglobulin therapy for myasthenia gravis

Abstract

The objective of this open, retrospective study was to investigate whether intravenous immunoglobulin (IVIg) could induce a clinically obvious improvement in patients with generalized myasthenia gravis (MG), as judged by MG functional status. Fourteen patients with generalized MG were treated during at least one episode with 0.4 g IVIg per kilogram body weight per day for 5 consecutive days. Patients with confounding variables were excluded; this left 11 patients (16 episodes) to be further analysed. We defined improvement as at least a one-step improvement in MG functional status (according to the University of Virginia's Modification of Osserman's classification). Of the treatment episodes, 56% were classified as positive responses. If improvement occurred, onset of improvement started after 3 (1-12) days and peak effect was reached after 7 (4-30) days (median and range). All four patients who required artificial ventilation could be weaned from it 8.5 (6-11) days after the start of IVIg (median and range). Of the patients treated on two occasions, only one patient had a positive response during both. In MG functional status 5, improvement was observed during five of seven episodes. None of the patients with MG functional status 3 responded. Patients with an acute relapse of MG seemed to respond equally well to IVIg compared with patients with subacute deterioration/ chronic-static state (50% versus 60%). The MG functional status at the start of IVIg and on the day of maximal improvement was compared for all episodes together, and significant improvement was noted (P = 0.0052). We did not see any serious side-effects after IVIg treatment. This retrospective analysis suggests that high-dose IVIg is an effective therapy in some patients with deterioration of generalized MG. If improvement occurs, it starts within a few days of the onset of IVIg and the effect seems to peak within 2 weeks.

Myasthenic crisis: Guidelines for prevention and treatment

“…Jongen [81] in a retrospective, unblinded and uncontrolled analysis of a small number of patients found in 56% (9 out of 16) patients improvement with the peak effect at 7 days. This was less than the 61 % of patient improvement noted by Gajdos [28].…”

Normal human immunoglobulin suppresses experimental myasthenia gravis in SCID mice

“…Controlled clinical trials have established the efficacy of IVIg in several neuromuscular diseases, including the Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, dermatomyositis and the Lambert-Eaton myasthenic syndrome [12,[15][16][17][18][19][20]. IVIg has also been demonstrated to be effective in the treatment of acute MG [21][22][23][24]. Although the mode of action of IVIg in MG is not fully understood, IVIg was shown to inhibit the binding of anti-AchR autoantibodies to AchR in vitro, by interacting with idiotypic determinants expressed by pathogenic autoantibodies [25].…”

Respiratory Complications of Rapidly Progressive Neuromuscular Syndromes: Guillain-Barré Syndrome and Myasthenia Gravis

“…More recently, small case series have shown that IVIG also improves weakness in myasthenic crisis. [25][26][27] Thus, when patients cannot tolerate plasmapheresis because of medical comorbidities such as sepsis, severe anemia, or difficult large-bore venous access, IVIG is used as an alternative, 28 at the same dose as for GBS.…”