PMID- 32417849
OWN - NLM
STAT- MEDLINE
DCOM- 20210115
LR  - 20210115
IS  - 1941-5923 (Electronic)
IS  - 1941-5923 (Linking)
VI  - 21
DP  - 2020 May 17
TI  - Successful Transition from Plasma Exchange to Eculizumab in Acetylcholine 
      Receptor Antibody- and Muscle-Specific Kinase (MuSK) Antibody-Negative Myasthenia 
      Gravis: A Case Report.
PG  - e921431
LID - 10.12659/AJCR.921431 [doi]
AB  - BACKGROUND The effectiveness of eculizumab (a terminal complement inhibitor) in 
      acetylcholine receptor (AChR) antibody-negative generalized myasthenia gravis 
      (gMG) is unknown. CASE REPORT A female patient was diagnosed with AChR-antibody 
      and muscle-specific kinase (MuSK) antibody-negative gMG in March 2016. In January 
      2017, the patient was referred for plasma exchange (PLEX) because of continuing 
      symptoms. She was also receiving azathioprine, mycophenolate mofetil, and 
      pyridostigmine (all were continued during subsequent therapies). PLEX (5 sessions 
      over 10 days) was initially effective, but over the following month the patient 
      received PLEX weekly, then twice weekly, followed by 3-times weekly because of 
      worsening symptoms. In April 2018, PLEX was reduced to twice weekly following 
      initiation of eculizumab (weekly induction dose of 900 mg 1 day after first PLEX, 
      plus 600 mg on the day of the second PLEX session, for 4 weeks). The patient was 
      then stabilized on eculizumab 1200 mg every 2 weeks and the frequency of PLEX 
      treatment was reduced, until PLEX was discontinued at Week 39 after eculizumab 
      initiation. During eculizumab treatment, the patient's myasthenia gravis 
      activities of daily living (MG-ADL) score decreased from 9 to 1 or 2 at most 
      assessments, with a transient increase to 4 or 5 between Weeks 19 and 27 
      following less frequent eculizumab treatment. There were no eculizumab-related 
      adverse events. CONCLUSIONS Following transition from 3-times weekly PLEX to 
      eculizumab in a patient with treatment-refractory, AChR antibody- and MuSK 
      antibody-negative gMG, there were clinically significant improvements in everyday 
      activities affected by MG symptoms. Further investigation of eculizumab in 
      antibody-negative MG is required.
FAU - Greenwood, Gregory T
AU  - Greenwood GT
AD  - Nephrology Section, Forsyth Medical Center, Winston-Salem, NC, USA.
FAU - Lynch, Zachary
AU  - Lynch Z
AD  - Nephrology Section, Forsyth Medical Center, Winston-Salem, NC, USA.
LA  - eng
PT  - Case Reports
DEP - 20200517
PL  - United States
TA  - Am J Case Rep
JT  - The American journal of case reports
JID - 101489566
RN  - 0 (Antibodies, Monoclonal, Humanized)
RN  - 0 (Autoantibodies)
RN  - 0 (Complement Inactivating Agents)
RN  - 0 (Receptors, Cholinergic)
RN  - A3ULP0F556 (eculizumab)
RN  - EC 2.7.10.1 (MUSK protein, human)
RN  - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases)
SB  - IM
MH  - Activities of Daily Living
MH  - Aged
MH  - Antibodies, Monoclonal, Humanized/*therapeutic use
MH  - Autoantibodies
MH  - Complement Inactivating Agents/*therapeutic use
MH  - Female
MH  - Humans
MH  - Myasthenia Gravis/*therapy
MH  - *Plasma Exchange
MH  - Receptor Protein-Tyrosine Kinases/immunology
MH  - Receptors, Cholinergic/immunology
MH  - Surveys and Questionnaires
PMC - PMC7262480
COIS- Conflict of interest: Dr. Gregory T. Greenwood has served as a paid consultant 
      for Alexion Pharmaceuticals. Mr. Zachary Lynch has no conflicts of interest 
      Conflicts of interest Dr. Gregory T. Greenwood has served as a paid consultant 
      for Alexion Pharmaceuticals. Mr. Zachary Lynch has no conflicts of interest.
EDAT- 2020/05/18 06:00
MHDA- 2021/01/16 06:00
PMCR- 2020/05/17
CRDT- 2020/05/18 06:00
PHST- 2020/05/18 06:00 [entrez]
PHST- 2020/05/18 06:00 [pubmed]
PHST- 2021/01/16 06:00 [medline]
PHST- 2020/05/17 00:00 [pmc-release]
AID - 921431 [pii]
AID - 10.12659/AJCR.921431 [doi]
PST - epublish
SO  - Am J Case Rep. 2020 May 17;21:e921431. doi: 10.12659/AJCR.921431.