PMID- 28801338
OWN - NLM
STAT- MEDLINE
DCOM- 20170908
LR  - 20180124
IS  - 1526-632X (Electronic)
IS  - 0028-3878 (Linking)
VI  - 89
IP  - 10
DP  - 2017 Sep 5
TI  - Rituximab as treatment for anti-MuSK myasthenia gravis: Multicenter blinded 
      prospective review.
PG  - 1069-1077
LID - 10.1212/WNL.0000000000004341 [doi]
AB  - OBJECTIVE: To evaluate the efficacy of rituximab in treatment of 
      anti-muscle-specific kinase (MuSK) myasthenia gravis (MG). METHODS: This was a 
      multicenter, blinded, prospective review, comparing anti-MuSK-positive patients 
      with MG treated with rituximab to those not treated with rituximab. The primary 
      clinical endpoint was the Myasthenia Gravis Status and Treatment Intensity 
      (MGSTI), a novel outcome that combines the Myasthenia Gravis Foundation of 
      America (MGFA) postintervention status (PIS) and the number and dosages of other 
      immunosuppressant therapies used. A priori, an MGSTI of level </=2 was used to 
      define a favorable outcome. Secondary outcomes included modified MGFA PIS of 
      minimal manifestations or better, mean/median prednisone dose, and mean/median 
      doses of other immunosuppressant drugs. RESULTS: Seventy-seven of 119 patients 
      with anti-MuSK MG evaluated between January 1, 2005, and January 1, 2015, at 10 
      neuromuscular centers were selected for analysis after review of limited clinical 
      data by a blinded expert panel. An additional 22 patients were excluded due to 
      insufficient follow-up. Baseline characteristics were similar between the 
      rituximab-treated patients (n = 24) and the controls (n = 31). Median follow-up 
      duration was >3.5 years. At last visit, 58% (14/24) of rituximab-treated patients 
      reached the primary outcome compared to 16% (5/31) of controls (p = 0.002). 
      Number needed to treat for the primary outcome is 2.4. At last visit, 29% of 
      rituximab-treated patients were taking prednisone (mean dose 4.5 mg/day) compared 
      to 74% of controls (mean dose 13 mg/day) (p = 0.001 and p = 0.005). 
      CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that for 
      patients with anti-MuSK MG, rituximab increased the probability of a favorable 
      outcome.
CI  - (c) 2017 American Academy of Neurology.
FAU - Hehir, Michael K
AU  - Hehir MK
AD  - From the Larner College of Medicine at the University of Vermont (M.K.H., D.H., 
      S.M.G.), Burlington; Duke University School of Medicine (L.D.H.-W.), Durham, NC; 
      University of Miami Health System (M.B., M.A.F.), FL; University of Toronto 
      School of Medicine (C.B.), Canada; SUNY Buffalo Jacobs School of Medicine 
      (N.J.S.), NY; UNC School of Medicine (J.F.H.), Chapel Hill, NC; Mayo Clinic 
      (A.V., B.A.C.), Rochester, MN; Yale School of Medicine (R.N., R.B., A.K.), New 
      Haven, CT; Medical University of South Carolina (K.R., I.-H.A.C.), Columbia; 
      University of Florida (M.T.P., S.M.L., Gainesville; University of Kansas Medical 
      Center (M.P.), Kansas City; and University of Virginia School of Medicine 
      (T.M.B.), Charlottesville. Michael.hehir@uvmhealth.org.
FAU - Hobson-Webb, Lisa D
AU  - Hobson-Webb LD
AD  - From the Larner College of Medicine at the University of Vermont (M.K.H., D.H., 
      S.M.G.), Burlington; Duke University School of Medicine (L.D.H.-W.), Durham, NC; 
      University of Miami Health System (M.B., M.A.F.), FL; University of Toronto 
      School of Medicine (C.B.), Canada; SUNY Buffalo Jacobs School of Medicine 
      (N.J.S.), NY; UNC School of Medicine (J.F.H.), Chapel Hill, NC; Mayo Clinic 
      (A.V., B.A.C.), Rochester, MN; Yale School of Medicine (R.N., R.B., A.K.), New 
      Haven, CT; Medical University of South Carolina (K.R., I.-H.A.C.), Columbia; 
      University of Florida (M.T.P., S.M.L., Gainesville; University of Kansas Medical 
      Center (M.P.), Kansas City; and University of Virginia School of Medicine 
      (T.M.B.), Charlottesville.
FAU - Benatar, Michael
AU  - Benatar M
AD  - From the Larner College of Medicine at the University of Vermont (M.K.H., D.H., 
      S.M.G.), Burlington; Duke University School of Medicine (L.D.H.-W.), Durham, NC; 
      University of Miami Health System (M.B., M.A.F.), FL; University of Toronto 
      School of Medicine (C.B.), Canada; SUNY Buffalo Jacobs School of Medicine 
      (N.J.S.), NY; UNC School of Medicine (J.F.H.), Chapel Hill, NC; Mayo Clinic 
      (A.V., B.A.C.), Rochester, MN; Yale School of Medicine (R.N., R.B., A.K.), New 
      Haven, CT; Medical University of South Carolina (K.R., I.-H.A.C.), Columbia; 
      University of Florida (M.T.P., S.M.L., Gainesville; University of Kansas Medical 
      Center (M.P.), Kansas City; and University of Virginia School of Medicine 
      (T.M.B.), Charlottesville.
FAU - Barnett, Carolina
AU  - Barnett C
AD  - From the Larner College of Medicine at the University of Vermont (M.K.H., D.H., 
      S.M.G.), Burlington; Duke University School of Medicine (L.D.H.-W.), Durham, NC; 
      University of Miami Health System (M.B., M.A.F.), FL; University of Toronto 
      School of Medicine (C.B.), Canada; SUNY Buffalo Jacobs School of Medicine 
      (N.J.S.), NY; UNC School of Medicine (J.F.H.), Chapel Hill, NC; Mayo Clinic 
      (A.V., B.A.C.), Rochester, MN; Yale School of Medicine (R.N., R.B., A.K.), New 
      Haven, CT; Medical University of South Carolina (K.R., I.-H.A.C.), Columbia; 
      University of Florida (M.T.P., S.M.L., Gainesville; University of Kansas Medical 
      Center (M.P.), Kansas City; and University of Virginia School of Medicine 
      (T.M.B.), Charlottesville.
FAU - Silvestri, Nicholas J
AU  - Silvestri NJ
AD  - From the Larner College of Medicine at the University of Vermont (M.K.H., D.H., 
      S.M.G.), Burlington; Duke University School of Medicine (L.D.H.-W.), Durham, NC; 
      University of Miami Health System (M.B., M.A.F.), FL; University of Toronto 
      School of Medicine (C.B.), Canada; SUNY Buffalo Jacobs School of Medicine 
      (N.J.S.), NY; UNC School of Medicine (J.F.H.), Chapel Hill, NC; Mayo Clinic 
      (A.V., B.A.C.), Rochester, MN; Yale School of Medicine (R.N., R.B., A.K.), New 
      Haven, CT; Medical University of South Carolina (K.R., I.-H.A.C.), Columbia; 
      University of Florida (M.T.P., S.M.L., Gainesville; University of Kansas Medical 
      Center (M.P.), Kansas City; and University of Virginia School of Medicine 
      (T.M.B.), Charlottesville.
FAU - Howard, James F Jr
AU  - Howard JF Jr
AD  - From the Larner College of Medicine at the University of Vermont (M.K.H., D.H., 
      S.M.G.), Burlington; Duke University School of Medicine (L.D.H.-W.), Durham, NC; 
      University of Miami Health System (M.B., M.A.F.), FL; University of Toronto 
      School of Medicine (C.B.), Canada; SUNY Buffalo Jacobs School of Medicine 
      (N.J.S.), NY; UNC School of Medicine (J.F.H.), Chapel Hill, NC; Mayo Clinic 
      (A.V., B.A.C.), Rochester, MN; Yale School of Medicine (R.N., R.B., A.K.), New 
      Haven, CT; Medical University of South Carolina (K.R., I.-H.A.C.), Columbia; 
      University of Florida (M.T.P., S.M.L., Gainesville; University of Kansas Medical 
      Center (M.P.), Kansas City; and University of Virginia School of Medicine 
      (T.M.B.), Charlottesville.
FAU - Howard, Diantha
AU  - Howard D
AD  - From the Larner College of Medicine at the University of Vermont (M.K.H., D.H., 
      S.M.G.), Burlington; Duke University School of Medicine (L.D.H.-W.), Durham, NC; 
      University of Miami Health System (M.B., M.A.F.), FL; University of Toronto 
      School of Medicine (C.B.), Canada; SUNY Buffalo Jacobs School of Medicine 
      (N.J.S.), NY; UNC School of Medicine (J.F.H.), Chapel Hill, NC; Mayo Clinic 
      (A.V., B.A.C.), Rochester, MN; Yale School of Medicine (R.N., R.B., A.K.), New 
      Haven, CT; Medical University of South Carolina (K.R., I.-H.A.C.), Columbia; 
      University of Florida (M.T.P., S.M.L., Gainesville; University of Kansas Medical 
      Center (M.P.), Kansas City; and University of Virginia School of Medicine 
      (T.M.B.), Charlottesville.
FAU - Visser, Amy
AU  - Visser A
AD  - From the Larner College of Medicine at the University of Vermont (M.K.H., D.H., 
      S.M.G.), Burlington; Duke University School of Medicine (L.D.H.-W.), Durham, NC; 
      University of Miami Health System (M.B., M.A.F.), FL; University of Toronto 
      School of Medicine (C.B.), Canada; SUNY Buffalo Jacobs School of Medicine 
      (N.J.S.), NY; UNC School of Medicine (J.F.H.), Chapel Hill, NC; Mayo Clinic 
      (A.V., B.A.C.), Rochester, MN; Yale School of Medicine (R.N., R.B., A.K.), New 
      Haven, CT; Medical University of South Carolina (K.R., I.-H.A.C.), Columbia; 
      University of Florida (M.T.P., S.M.L., Gainesville; University of Kansas Medical 
      Center (M.P.), Kansas City; and University of Virginia School of Medicine 
      (T.M.B.), Charlottesville.
FAU - Crum, Brian A
AU  - Crum BA
AD  - From the Larner College of Medicine at the University of Vermont (M.K.H., D.H., 
      S.M.G.), Burlington; Duke University School of Medicine (L.D.H.-W.), Durham, NC; 
      University of Miami Health System (M.B., M.A.F.), FL; University of Toronto 
      School of Medicine (C.B.), Canada; SUNY Buffalo Jacobs School of Medicine 
      (N.J.S.), NY; UNC School of Medicine (J.F.H.), Chapel Hill, NC; Mayo Clinic 
      (A.V., B.A.C.), Rochester, MN; Yale School of Medicine (R.N., R.B., A.K.), New 
      Haven, CT; Medical University of South Carolina (K.R., I.-H.A.C.), Columbia; 
      University of Florida (M.T.P., S.M.L., Gainesville; University of Kansas Medical 
      Center (M.P.), Kansas City; and University of Virginia School of Medicine 
      (T.M.B.), Charlottesville.
FAU - Nowak, Richard
AU  - Nowak R
AD  - From the Larner College of Medicine at the University of Vermont (M.K.H., D.H., 
      S.M.G.), Burlington; Duke University School of Medicine (L.D.H.-W.), Durham, NC; 
      University of Miami Health System (M.B., M.A.F.), FL; University of Toronto 
      School of Medicine (C.B.), Canada; SUNY Buffalo Jacobs School of Medicine 
      (N.J.S.), NY; UNC School of Medicine (J.F.H.), Chapel Hill, NC; Mayo Clinic 
      (A.V., B.A.C.), Rochester, MN; Yale School of Medicine (R.N., R.B., A.K.), New 
      Haven, CT; Medical University of South Carolina (K.R., I.-H.A.C.), Columbia; 
      University of Florida (M.T.P., S.M.L., Gainesville; University of Kansas Medical 
      Center (M.P.), Kansas City; and University of Virginia School of Medicine 
      (T.M.B.), Charlottesville.
FAU - Beekman, Rachel
AU  - Beekman R
AD  - From the Larner College of Medicine at the University of Vermont (M.K.H., D.H., 
      S.M.G.), Burlington; Duke University School of Medicine (L.D.H.-W.), Durham, NC; 
      University of Miami Health System (M.B., M.A.F.), FL; University of Toronto 
      School of Medicine (C.B.), Canada; SUNY Buffalo Jacobs School of Medicine 
      (N.J.S.), NY; UNC School of Medicine (J.F.H.), Chapel Hill, NC; Mayo Clinic 
      (A.V., B.A.C.), Rochester, MN; Yale School of Medicine (R.N., R.B., A.K.), New 
      Haven, CT; Medical University of South Carolina (K.R., I.-H.A.C.), Columbia; 
      University of Florida (M.T.P., S.M.L., Gainesville; University of Kansas Medical 
      Center (M.P.), Kansas City; and University of Virginia School of Medicine 
      (T.M.B.), Charlottesville.
FAU - Kumar, Aditya
AU  - Kumar A
AD  - From the Larner College of Medicine at the University of Vermont (M.K.H., D.H., 
      S.M.G.), Burlington; Duke University School of Medicine (L.D.H.-W.), Durham, NC; 
      University of Miami Health System (M.B., M.A.F.), FL; University of Toronto 
      School of Medicine (C.B.), Canada; SUNY Buffalo Jacobs School of Medicine 
      (N.J.S.), NY; UNC School of Medicine (J.F.H.), Chapel Hill, NC; Mayo Clinic 
      (A.V., B.A.C.), Rochester, MN; Yale School of Medicine (R.N., R.B., A.K.), New 
      Haven, CT; Medical University of South Carolina (K.R., I.-H.A.C.), Columbia; 
      University of Florida (M.T.P., S.M.L., Gainesville; University of Kansas Medical 
      Center (M.P.), Kansas City; and University of Virginia School of Medicine 
      (T.M.B.), Charlottesville.
FAU - Ruzhansky, Katherine
AU  - Ruzhansky K
AD  - From the Larner College of Medicine at the University of Vermont (M.K.H., D.H., 
      S.M.G.), Burlington; Duke University School of Medicine (L.D.H.-W.), Durham, NC; 
      University of Miami Health System (M.B., M.A.F.), FL; University of Toronto 
      School of Medicine (C.B.), Canada; SUNY Buffalo Jacobs School of Medicine 
      (N.J.S.), NY; UNC School of Medicine (J.F.H.), Chapel Hill, NC; Mayo Clinic 
      (A.V., B.A.C.), Rochester, MN; Yale School of Medicine (R.N., R.B., A.K.), New 
      Haven, CT; Medical University of South Carolina (K.R., I.-H.A.C.), Columbia; 
      University of Florida (M.T.P., S.M.L., Gainesville; University of Kansas Medical 
      Center (M.P.), Kansas City; and University of Virginia School of Medicine 
      (T.M.B.), Charlottesville.
FAU - Chen, I-Hweii Amy
AU  - Chen IA
AD  - From the Larner College of Medicine at the University of Vermont (M.K.H., D.H., 
      S.M.G.), Burlington; Duke University School of Medicine (L.D.H.-W.), Durham, NC; 
      University of Miami Health System (M.B., M.A.F.), FL; University of Toronto 
      School of Medicine (C.B.), Canada; SUNY Buffalo Jacobs School of Medicine 
      (N.J.S.), NY; UNC School of Medicine (J.F.H.), Chapel Hill, NC; Mayo Clinic 
      (A.V., B.A.C.), Rochester, MN; Yale School of Medicine (R.N., R.B., A.K.), New 
      Haven, CT; Medical University of South Carolina (K.R., I.-H.A.C.), Columbia; 
      University of Florida (M.T.P., S.M.L., Gainesville; University of Kansas Medical 
      Center (M.P.), Kansas City; and University of Virginia School of Medicine 
      (T.M.B.), Charlottesville.
FAU - Pulley, Michael T
AU  - Pulley MT
AD  - From the Larner College of Medicine at the University of Vermont (M.K.H., D.H., 
      S.M.G.), Burlington; Duke University School of Medicine (L.D.H.-W.), Durham, NC; 
      University of Miami Health System (M.B., M.A.F.), FL; University of Toronto 
      School of Medicine (C.B.), Canada; SUNY Buffalo Jacobs School of Medicine 
      (N.J.S.), NY; UNC School of Medicine (J.F.H.), Chapel Hill, NC; Mayo Clinic 
      (A.V., B.A.C.), Rochester, MN; Yale School of Medicine (R.N., R.B., A.K.), New 
      Haven, CT; Medical University of South Carolina (K.R., I.-H.A.C.), Columbia; 
      University of Florida (M.T.P., S.M.L., Gainesville; University of Kansas Medical 
      Center (M.P.), Kansas City; and University of Virginia School of Medicine 
      (T.M.B.), Charlottesville.
FAU - LaBoy, Shannon M
AU  - LaBoy SM
AD  - From the Larner College of Medicine at the University of Vermont (M.K.H., D.H., 
      S.M.G.), Burlington; Duke University School of Medicine (L.D.H.-W.), Durham, NC; 
      University of Miami Health System (M.B., M.A.F.), FL; University of Toronto 
      School of Medicine (C.B.), Canada; SUNY Buffalo Jacobs School of Medicine 
      (N.J.S.), NY; UNC School of Medicine (J.F.H.), Chapel Hill, NC; Mayo Clinic 
      (A.V., B.A.C.), Rochester, MN; Yale School of Medicine (R.N., R.B., A.K.), New 
      Haven, CT; Medical University of South Carolina (K.R., I.-H.A.C.), Columbia; 
      University of Florida (M.T.P., S.M.L., Gainesville; University of Kansas Medical 
      Center (M.P.), Kansas City; and University of Virginia School of Medicine 
      (T.M.B.), Charlottesville.
FAU - Fellman, Melissa A
AU  - Fellman MA
AD  - From the Larner College of Medicine at the University of Vermont (M.K.H., D.H., 
      S.M.G.), Burlington; Duke University School of Medicine (L.D.H.-W.), Durham, NC; 
      University of Miami Health System (M.B., M.A.F.), FL; University of Toronto 
      School of Medicine (C.B.), Canada; SUNY Buffalo Jacobs School of Medicine 
      (N.J.S.), NY; UNC School of Medicine (J.F.H.), Chapel Hill, NC; Mayo Clinic 
      (A.V., B.A.C.), Rochester, MN; Yale School of Medicine (R.N., R.B., A.K.), New 
      Haven, CT; Medical University of South Carolina (K.R., I.-H.A.C.), Columbia; 
      University of Florida (M.T.P., S.M.L., Gainesville; University of Kansas Medical 
      Center (M.P.), Kansas City; and University of Virginia School of Medicine 
      (T.M.B.), Charlottesville.
FAU - Greene, Shane M
AU  - Greene SM
AD  - From the Larner College of Medicine at the University of Vermont (M.K.H., D.H., 
      S.M.G.), Burlington; Duke University School of Medicine (L.D.H.-W.), Durham, NC; 
      University of Miami Health System (M.B., M.A.F.), FL; University of Toronto 
      School of Medicine (C.B.), Canada; SUNY Buffalo Jacobs School of Medicine 
      (N.J.S.), NY; UNC School of Medicine (J.F.H.), Chapel Hill, NC; Mayo Clinic 
      (A.V., B.A.C.), Rochester, MN; Yale School of Medicine (R.N., R.B., A.K.), New 
      Haven, CT; Medical University of South Carolina (K.R., I.-H.A.C.), Columbia; 
      University of Florida (M.T.P., S.M.L., Gainesville; University of Kansas Medical 
      Center (M.P.), Kansas City; and University of Virginia School of Medicine 
      (T.M.B.), Charlottesville.
FAU - Pasnoor, Mamatha
AU  - Pasnoor M
AD  - From the Larner College of Medicine at the University of Vermont (M.K.H., D.H., 
      S.M.G.), Burlington; Duke University School of Medicine (L.D.H.-W.), Durham, NC; 
      University of Miami Health System (M.B., M.A.F.), FL; University of Toronto 
      School of Medicine (C.B.), Canada; SUNY Buffalo Jacobs School of Medicine 
      (N.J.S.), NY; UNC School of Medicine (J.F.H.), Chapel Hill, NC; Mayo Clinic 
      (A.V., B.A.C.), Rochester, MN; Yale School of Medicine (R.N., R.B., A.K.), New 
      Haven, CT; Medical University of South Carolina (K.R., I.-H.A.C.), Columbia; 
      University of Florida (M.T.P., S.M.L., Gainesville; University of Kansas Medical 
      Center (M.P.), Kansas City; and University of Virginia School of Medicine 
      (T.M.B.), Charlottesville.
FAU - Burns, Ted M
AU  - Burns TM
AD  - From the Larner College of Medicine at the University of Vermont (M.K.H., D.H., 
      S.M.G.), Burlington; Duke University School of Medicine (L.D.H.-W.), Durham, NC; 
      University of Miami Health System (M.B., M.A.F.), FL; University of Toronto 
      School of Medicine (C.B.), Canada; SUNY Buffalo Jacobs School of Medicine 
      (N.J.S.), NY; UNC School of Medicine (J.F.H.), Chapel Hill, NC; Mayo Clinic 
      (A.V., B.A.C.), Rochester, MN; Yale School of Medicine (R.N., R.B., A.K.), New 
      Haven, CT; Medical University of South Carolina (K.R., I.-H.A.C.), Columbia; 
      University of Florida (M.T.P., S.M.L., Gainesville; University of Kansas Medical 
      Center (M.P.), Kansas City; and University of Virginia School of Medicine 
      (T.M.B.), Charlottesville.
LA  - eng
SI  - ClinicalTrials.gov/NCT02110706
PT  - Journal Article
PT  - Multicenter Study
DEP - 20170811
PL  - United States
TA  - Neurology
JT  - Neurology
JID - 0401060
RN  - 0 (Anti-Inflammatory Agents)
RN  - 0 (Autoantibodies)
RN  - 0 (Immunologic Factors)
RN  - 0 (Receptors, Cholinergic)
RN  - 4F4X42SYQ6 (Rituximab)
RN  - EC 2.7.10.1 (MUSK protein, human)
RN  - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases)
RN  - VB0R961HZT (Prednisone)
SB  - IM
CIN - Neurology. 2017 Sep 5;89(10):988-989. PMID: 28801339
MH  - Adult
MH  - Anti-Inflammatory Agents/therapeutic use
MH  - Autoantibodies/*metabolism
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Immunologic Factors/*therapeutic use
MH  - Male
MH  - Middle Aged
MH  - Myasthenia Gravis/*drug therapy/*immunology
MH  - Prednisone/therapeutic use
MH  - Prospective Studies
MH  - Receptor Protein-Tyrosine Kinases/*immunology
MH  - Receptors, Cholinergic/*immunology
MH  - Rituximab/*therapeutic use
MH  - Severity of Illness Index
MH  - Single-Blind Method
MH  - Treatment Outcome
EDAT- 2017/08/13 06:00
MHDA- 2017/09/09 06:00
CRDT- 2017/08/13 06:00
PHST- 2017/03/07 00:00 [received]
PHST- 2017/05/22 00:00 [accepted]
PHST- 2017/08/13 06:00 [pubmed]
PHST- 2017/09/09 06:00 [medline]
PHST- 2017/08/13 06:00 [entrez]
AID - WNL.0000000000004341 [pii]
AID - 10.1212/WNL.0000000000004341 [doi]
PST - ppublish
SO  - Neurology. 2017 Sep 5;89(10):1069-1077. doi: 10.1212/WNL.0000000000004341. Epub 
      2017 Aug 11.