@article{49d0bb555236440db27cbb4b5d9ff3fa,
title = "Thymectomy may not be associated with clinical improvement in MuSK myasthenia gravis",
abstract = "Introduction: A randomized trial demonstrated benefit from thymectomy in nonthymomatous acetylcholine receptor (AChR)-antibody positive myasthenia gravis (MG). Uncontrolled observational and histologic studies suggest thymectomy may not be efficacious in anti–muscle-specific kinase (MuSK)-MG. Methods: The therapeutic impact of thymectomy was evaluated from data collected for a multicenter, retrospective blinded review of rituximab in MuSK-MG. Results: Baseline characteristics were similar between thymectomy (n = 26) and nonthymectomy (n = 29) groups, including treatment with rituximab (42% vs. 45%). At last visit, 35% of thymectomy subjects reached the primary endpoint, a Myasthenia Gravis Foundation of America (MGFA) post-intervention status (PIS) score of minimal manifestations (MM) or better, compared with 55% of controls (P = 0.17). After controlling for age at onset of MG, rituximab, prednisone, and intravenous immunoglobulin/plasma exchange treatment, thymectomy was not associated with greater likelihood of favorable clinical outcome (odds ratio = 0.43, 95% confidence interval 0.12–1.53, P = 0.19). Discussion: Thymectomy was not associated with additional clinical improvement in this multicenter cohort of MuSK-MG patients. Muscle Nerve 59:404–410, 2019.",
keywords = "immunosuppressant therapy, muscle-specific kinase (MuSK) antibodies, myasthenia gravis, rituximab, thymectomy",
author = "Clifford, {Katherine M.} and Hobson-Webb, {Lisa D.} and Michael Benatar and Burns, {Ted M.} and Carolina Barnett and Silvestri, {Nicholas J.} and Howard, {James F.} and Amy Visser and Crum, {Brian A.} and Richard Nowak and Rachel Beekman and Aditya Kumar and Katherine Ruzhansky and Chen, {I. Hweii Amy} and Pulley, {Michael T.} and Laboy, {Shannon M.} and Fellman, {Melissa A.} and Howard, {Diantha B.} and Kolb, {Noah A.} and Greene, {Shane M.} and Mamatha Pasnoor and Dimachkie, {Mazen M.} and Barohn, {Richard J.} and Hehir, {Michael K.}",
note = "Funding Information: The authors are grateful to Michelle Turner and Carol Denny (associate clinical research coordinators, Mayo Clinic Rochester), who assisted with data collection and coordination. Ethical Publication Statement: We (the authors) confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this report is consistent with those guidelines. Funding Information: Abbreviations: AChR, acetylcholine receptor; IRB, institutional review board; IVIg, intravenous immunoglobulin; MG, myasthenia gravis; MGFA, Myasthenia Gravis Foundation of America; MGSTI, Myasthenia Gravis Status and Treatment Intensity; MM, minimal manifestations; MMF, mycophenolate mofetil; MuSK, muscle-specific kinase; PIS, postintervention status; PLEx, plasma exchange Key words: immunosuppressant therapy; myasthenia gravis; muscle-specific kinase (MuSK) antibodies; rituximab; thymectomy Funding: American Academy of Neurology (to M.K.H.); American Brain Foundation (to M.K.H.); Myasthenia Gravis Foundation of America (Clinician Scientist Development Award to M.K.H.); University of Vermont Department of Neurosciences (to M.K.H.); National Institutes of Health/National Institute of Neurologic Disorders and Stroke (U01NS084495 to R.N.); and Genentech (to R.N.). Disclosures: M.K.H. has served on medical advisory board for Octa-pharma, CSL Behring, and Alexion Pharmaceuticals. N.S. has consulted for Alexion and OptionCare for unrelated myasthenia gravis projects. A.V. has served on the medical advisory board for Alexion Pharmaceuticals. K.R. has consulted for Alexion Pharmaceuticals for unrelated work. The remaining authors have no conflicts of interest. Publisher Copyright: {\textcopyright} 2019 Wiley Periodicals, Inc.",
year = "2019",
month = apr,
doi = "10.1002/mus.26404",
language = "English (US)",
volume = "59",
pages = "404--410",
journal = "Muscle and Nerve",
issn = "0148-639X",
publisher = "John Wiley and Sons Inc.",
number = "4",
}