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Successful Transition from Plasma Exchange to Eculizumab in Acetylcholine Receptor Antibody- and Muscle-Specific Kinase (MuSK) Antibody-Negative Myasthenia Gravis: A Case Report.
- Source :
-
The American journal of case reports [Am J Case Rep] 2020 May 17; Vol. 21, pp. e921431. Date of Electronic Publication: 2020 May 17. - Publication Year :
- 2020
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Abstract
- 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.
- Subjects :
- Activities of Daily Living
Aged
Autoantibodies
Female
Humans
Receptor Protein-Tyrosine Kinases immunology
Receptors, Cholinergic immunology
Surveys and Questionnaires
Antibodies, Monoclonal, Humanized therapeutic use
Complement Inactivating Agents therapeutic use
Myasthenia Gravis therapy
Plasma Exchange
Subjects
Details
- Language :
- English
- ISSN :
- 1941-5923
- Volume :
- 21
- Database :
- MEDLINE
- Journal :
- The American journal of case reports
- Publication Type :
- Academic Journal
- Accession number :
- 32417849
- Full Text :
- https://doi.org/10.12659/AJCR.921431