Treatment of refractory myasthenia gravis by double‐filtration plasmapheresis and rituximab: A case series of nine patients and literature review

Abstract

Introduction Myasthenia gravis (MG) is an autoimmune disease mediated by circulating autoantibodies (anti‐AchR, anti‐MuSK, etc.). More than 20% of myasthenic patients are refractory to conventional treatments (plasma exchange, IVIg, steroids, azathioprine, mycophenolate mofetil). Rituximab (B‐lymphocyte‐depleting anti‐CD20) and apheresis (double‐filtration plasmapheresis [DFPP] and immunoadsorption [IA]) are interesting therapeutic alternatives. Methods This monocentric pilot study included nine refractory myasthenic patients (March 2018 to May 2020) treated by DFPP and/or IA associated with rituximab (375 mg/m2). Clinical responses were assessed using the Myasthenia Gravis Foundation of America (MGFA) score. Results Average age of patients was 53 ± 17 years. Gender ratio (M/F) was 3:6. The combination of apheresis and rituximab reduced median MGFA score from IV to II after 12 months of follow‐up. Clinical improvement assessed by MGFA score was sustained in the long‐term for all patients, during an average follow‐up of 14 ± 9 months, allowing them to be self‐sufficient and out sick‐leave. The median number of apheresis sessions was 7 (5‐30). The dose of prednisolone was reduced in two patients from 40 mg/d and 30 mg/d to 7.5 mg/d and 10 mg/d, respectively. It was stopped in a patient who was taking 30 mg/d. No infectious, bleeding, or thrombosis complications were noted. Conclusion The combination of rituximab and DFPP was effective to treat refractory MG.

Our Clinical Experience in the Treatment of Myasthenia Gravis Acute Exacerbations with a Novel Nanomembrane-Based Therapeutic Plasma Exchange Technology

“…An important research perspective could be to study the best combinations between nanomembrane-based TPE and other treatments. For example, the combination of double-filtration plasmapheresis and rituximab was proven to be effective in treatment of refractory MG [ 37 ], as were our combination of nanomembrane-based TPE and IVIG in this clinical scenario. The combination using double-filtration plasmapheresis proved to be more effective in lowering IgA levels than one using immunoabsorption [ 37 ].…”

“…For example, the combination of double-filtration plasmapheresis and rituximab was proven to be effective in treatment of refractory MG [ 37 ], as were our combination of nanomembrane-based TPE and IVIG in this clinical scenario. The combination using double-filtration plasmapheresis proved to be more effective in lowering IgA levels than one using immunoabsorption [ 37 ]. The choice of these possible (but not exclusive) combinations could be influenced by the IgA levels.…”

Combination Therapy of Plasma Exchange and Rituximab to Treat Cicatricial Pemphigoid and Bullous Pemphigoid

“…A literature review has also demonstrated the success of this approach in treating refractory cases of myasthenia gravis (MG), another autoimmune condition. Nine patients with refractory MG who were treated with plasmapheresis and rituximab showed an overall decrease in disease severity, long-term clinical improvement, and self-sufficiency [ 9 ].…”

Changes in Water Properties in Human Tissue after Double Filtration Plasmapheresis—A Case Study

“…The DFPP technique allows pathophysiological relevant molecules (e.g., circulating autoantigens, autoantibodies, circulating immune complexes, damaged proteins) and toxins (e.g., environmental toxins and toxins from microorganisms) to be removed from the blood of a subject. This blood cleaning procedure has been successfully used therapeutically in many diseases [2][3][4], including myasthenia gravis [5][6][7][8][9][10][11][12], chronic inflammatory demyelinating polyneuropathy [12], anti-glomerular basement membrane disease [13], hypoglycemia and hyperglycemia induced by insulin antibodies [14], pancreatitis induced by hypertriglyceridemia [15][16][17], Guillain-Barré syndrome [12,[18][19][20][21], Crow-Fukase syndrome [12], rheumatoid arthritis [22][23][24], chronic hepatitis C [25,26], pemphigus [27,28], bullous pemphigoid [29,30], atopic dermatitis [31], dermatomyositis [12], polymyositis [12], membranous nephropathy [32], acute thallotoxicosis [33], antibody-associated vasculitis [34][35][36], antisynthetase syndrome [37], diffuse proliferative lupus nephritis…”