Excellent response to therapeutic plasma exchange in myasthenia gravis patients irrespective of antibody status

Abstract

Introduction The primary objective of this study was to assess response to plasma exchange (PLEX) in myasthenia gravis (MG) patients with and without autoantibodies (Ab) to acetylcholine receptor (AChR) or muscle‐specific kinase (MuSK). Analysis was also done to determine if correlation existed between sex, early or late onset MG, thymoma, or thymectomy and response to PLEX. Materials and Methods Data was analyzed on 58 consecutive MG patients treated with PLEX. Responses were categorized as complete response, clinical improvement requiring maintenance PLEX, or no/minimal response to PLEX. Results Eighty‐eight percent (51/58) of patients were Ab‐positive; 44 had AChR and 7 had MuSK Ab. Complete response was seen in 26 patients (24 Ab+), 24 remain on maintenance PLEX (19 Ab+), and 2 had no/minimal response (both AChR Ab+). Ab status (P = 0.43), AChR Ab (P = 0.10), MuSK Ab (P = 0.45), early onset MG (P = 0.63), thymoma (P = 0.46), and thymectomy (P = 0.16) were not significantly associated with outcome. Patient sex did show significant association with outcome (P = 0.01), with men more likely to have complete response and women more likely to require maintenance. Late onset MG is significantly associated with higher likelihood of complete response (P = 0.03). Antibody titers declined after PLEX in 83% of patients with complete response, in whom pre‐ and post‐PLEX titers were available (n = 6). Conclusions In conclusion, our study showed 96% response rate to PLEX in MG; however, only patient gender and late onset MG were significantly associated with treatment response.

Response to immunotherapy in anti‐IgLON5 disease: A systematic review

“…Among the regimens not considered maintenance treatments, PLEX obtained the highest IT response rate; however, AZA and MMF showed the highest response and sustained response rates when all types of treatment were considered. A good response rate has been reported for PLEX in other immune‐mediated diseases involving autoantibodies, notably a response rate of 96% in myasthenia gravis, although it should be taken into account that the action mechanisms of PLEX are not limited to autoantibody removal alone . We highlight the excellent response of patients treated with AZA (5/5) and MMF (3/4), which were always administered in combination (AZA always with corticosteroids).…”

“…30 Among the F I G U R E 2 Response to IT type are not limited to autoantibody removal alone. 31 We highlight the excellent response of patients treated with AZA (5/5) and MMF (3/4), which were always administered in combination (AZA always with corticosteroids).…”

No correlation between acetylcholine receptor antibody concentration and individual clinical symptoms of myasthenia gravis: A systematic retrospective study involving 67 patients

“…Rajesh, K and Carandina‐Maffeis, R have have shown that plasma exchange might improve short‐term prognosis in patients with MG (Carandina‐Maffeis et al., 2004 ; Kumar et al., 2015 ), wherese, Newsom‐Davis, J reported that the AChR‐Ab concentration negatively correlates with muscle strength after plasma exchange (Newsom‐Davis et al., 1978 ). Usmani, A found that LOMG and male patients are more likely to have symptomatic relief after using plasma exchange, and antibody titers may be an effective way to monitor the plasma exchange response of patients with MG (Usmani et al., 2019 ). The two patients who received plasma exchange treatment in our study showed reduced AChR‐Ab concentration and improved clinical symptoms after treatment.…”

Myasthenia Gravis With Antibodies Against Muscle Specific Kinase: An Update on Clinical Features, Pathophysiology and Treatment

“…The treatment of severe relapses, with mainly bulbar symptomatology, and life-threatening myasthenic crisis are similar between MuSK- and AChR-MG. Acute administration of intravenous immunoglobulins (IVIg) and plasma exchange are both usually effective (for the latter up to 96% of positive response irrespective of patients’ antibody status) with rapid control of the symptoms which last for about 4–6 weeks (Usmani et al, 2019 ). Although considered equal in the treatment of myasthenia (Rønager et al, 2001 ), plasma exchange has been reported to be faster and more effective compared to IVIg in MuSK-MG patients (Guptill and Sanders, 2010 ; Pasnoor et al, 2010 ) and, therefore, should be prioritized.…”