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Report of the ASFA apheresis registry on muscle specific kinase antibody positive myasthenia gravis
Chisa Yamada1,*, Huy P. Pham2, Yanyun Wu3,4, Laura Cooling1, Haewon C. Kim5, Shanna Morgan6, Joseph Schwartz7, Jeffrey L. Winters8 andEdward C.C. Wong9,10
Article first published online: 6 MAR 2016 DOI: 10.1002/jca.21454
J. Clin. Apheresis, 2016.
Anti-muscle specific kinase antibody positive (MuSK Ab) myasthenia gravis (MG) patients are known to have different clinical course compared to anti-acetylcholine receptor Ab positive MG patients.
Therapeutic plasma exchange (TPE) has been reported to be effective; however, little is known of the response and of TPE procedural information. An ASFA Apheresis Registry was developed to analyze those data.
The study collected detailed de-identified patient data, TPE procedures, and treatment outcome/complications. Collected data was described in aggregate.
A total of 15 MuSK Ab MG patients with exacerbation of MG symptoms, 13 females/2 males, median age 44, were investigated. Thirty TPE courses (median 5 procedures/course, total 145 procedures) were evaluated.
All TPE procedures were performed with citrate anticoagulation, 1 − 1.25 plasma volume exchange in 100% fluid balance, and 90% of courses used only albumin as replacement. Calcium was added to albumin or given orally as needed.
TPE was performed every other day in 55% of courses.
Adverse events occurred in 3.4% of procedures. Ten patients (67%) experienced relapses within a median of 7 weeks.
Objective symptoms were resolved in more than 75% of courses. Overall subjective improvement rates were 94.1%/93.3% after 3/4 TPE procedures, respectively. Thirty-one percent of patients responded poorly with minimal recovery.
Overall subjective improvement was seen up to 94% of patients after one course of TPE. Some patients were poor-responders. Five TPE may be adequate for initial course with additional TPE as needed. Based upon this preliminary data, we will modify our future data collection.
© 2016 Wiley Periodicals, Inc.