Karthika A Valaparambil, Soumya Sundaram, Sruthi S Nair
{"title":"Rituximab in Refractory Myasthenia Gravis - Challenges and Lessons Learnt.","authors":"Karthika A Valaparambil, Soumya Sundaram, Sruthi S Nair","doi":"10.4103/aian.aian_579_24","DOIUrl":null,"url":null,"abstract":"<p><p>Nearly 10%-15% patients with generalized myasthenia gravis (MG) have refractory disease and are candidates for newer nonconventional immunotherapies. Rituximab has emerged as an attractive option in them. We describe the efficacy and safety of rituximab in 12 patients with refractory MG treated over a period of 4 years in a tertiary care center. We had a female-predominant cohort (10 females) in the age range 10-67 years. There were eight seropositive patients (acetylcholine receptor, muscle-specific kinase, or both antibodies) and eight had undergone thymectomy. Median number of crises was 2 before treatment with rituximab. Sustained improvement at 12 months was seen in seven patients and steroid dose could be reduced in them. Four worsened on therapy (including one death following prolonged ventilation and septicemia). One patient who had no response to therapy was re-evaluated and diagnosed with congenital myasthenic syndrome related to biallelic CHRNE mutation. In summary, nearly two-thirds (7/11, 63.6%) of autoimmune refractory MG in this cohort showed a good response to rituximab.</p>","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":"706-709"},"PeriodicalIF":1.9000,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Annals of Indian Academy of Neurology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.4103/aian.aian_579_24","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/12/2 0:00:00","PubModel":"Epub","JCR":"Q3","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Nearly 10%-15% patients with generalized myasthenia gravis (MG) have refractory disease and are candidates for newer nonconventional immunotherapies. Rituximab has emerged as an attractive option in them. We describe the efficacy and safety of rituximab in 12 patients with refractory MG treated over a period of 4 years in a tertiary care center. We had a female-predominant cohort (10 females) in the age range 10-67 years. There were eight seropositive patients (acetylcholine receptor, muscle-specific kinase, or both antibodies) and eight had undergone thymectomy. Median number of crises was 2 before treatment with rituximab. Sustained improvement at 12 months was seen in seven patients and steroid dose could be reduced in them. Four worsened on therapy (including one death following prolonged ventilation and septicemia). One patient who had no response to therapy was re-evaluated and diagnosed with congenital myasthenic syndrome related to biallelic CHRNE mutation. In summary, nearly two-thirds (7/11, 63.6%) of autoimmune refractory MG in this cohort showed a good response to rituximab.
期刊介绍:
The journal has a clinical foundation and has been utilized most by clinical neurologists for improving the practice of neurology. While the focus is on neurology in India, the journal publishes manuscripts of high value from all parts of the world. Journal publishes reviews of various types, original articles, short communications, interesting images and case reports. The journal respects the scientific submission of its authors and believes in following an expeditious double-blind peer review process and endeavors to complete the review process within scheduled time frame. A significant effort from the author and the journal perhaps enables to strike an equilibrium to meet the professional expectations of the peers in the world of scientific publication. AIAN believes in safeguarding the privacy rights of human subjects. In order to comply with it, the journal instructs all authors when uploading the manuscript to also add the ethical clearance (human/animals)/ informed consent of subject in the manuscript. This applies to the study/case report that involves animal/human subjects/human specimens e.g. extracted tooth part/soft tissue for biopsy/in vitro analysis.