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Thymectomy Trial in Non-Thymomatous Myasthenia Gravis Patients Receiving Prednisone Therapy

Phase 3
Completed
Conditions
Myasthenia Gravis
Interventions
Procedure: thymectomy plus prednisone
Registration Number
NCT00294658
Lead Sponsor
University of Alabama at Birmingham
Brief Summary

The purpose of this trial is to determine if thymectomy combined with prednisone therapy is more beneficial in treating non-thymomatous myasthenia gravis than prednisone therapy alone.

Detailed Description

Myasthenia gravis (MG) is an autoimmune disease involving the thymus in which 85 percent of patients have antibodies to muscle acetylcholine receptors (AchR-Ab) that interfere with neuromuscular transmission. MG frequently causes severe disability that can be life-threatening. Thymectomy-a surgical procedure that removes thymus gland tissue from the chest cavity-has been an established therapy for non-thymomatous MG, or MG without thymoma, for more than 60 years (based on retrospective, non-randomized studies). Corticosteroids are now being used increasingly either as the sole treatment or in combination with thymectomy. Both therapies have associated adverse effects and indications for their use based on randomized trial data are lacking.

The purpose of this 5-year trial is to determine if the surgical procedure, extended transsternal thymectomy (ETTX), combined with prednisone therapy is more beneficial in treating individuals with non-thymomatous MG than prednisone therapy alone. More specifically, this study will determine 1) if ETTX combined with prednisone results in a greater improvement in myasthenic weakness, compared to prednisone alone; 2) if ETTX combined with prednisone results in a lower total dose of prednisone, thus decreasing the likelihood of concurrent and long-term toxic effects, compared to prednisone alone; and 3) if ETTX combined with prednisone enhances quality of life by reducing adverse events and symptoms associated with the therapies, compared to prednisone alone.

Learning that thymectomy results in a meaningful reduction of prednisone dosage or even full withdrawal or reduces side effects related to prednisone would support using the two treatments-thymectomy and prednisone-together. However, if no meaningful reduction of prednisone dosage or side effects is shown, the results would mean that using the two treatments together offers no advantages over prednisone treatment alone.

After an initial screening, study participants will be randomized either to undergo the surgical procedure ETTX and receive prednisone treatment, or to receive prednisone treatment alone without surgery. Participants will be followed for at least 3 years.

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
126
Inclusion Criteria
  • Male and female MG patients age greater than 18 and less than 65 years
  • Onset of generalized MG within the last 5 years
  • Positive serum anti-acetylcholine receptor binding antibodies (muscle acetylcholine receptors, AchRAb =/> 1.00 nmol/L. AchRAb levels of 0.50-0.99 nmol/L will be acceptable if there is another confirmatory test for MG, including single-fiber electromyography (EMG), repetitive nerve stimulation, or unequivocal edrophonium testing.)
  • MGFA class II-IV at entry, using the MG Foundation of America (MGFA) classification, while receiving optimal anti-cholinesterase treatment with or without oral prednisone
Exclusion Criteria
  • Ocular MG without generalized weakness (MGFA Class I) or minimal weakness that would not require the use of corticosteroids
  • Myasthenic weakness requiring intubation (MGFA Class IV) in the prior month
  • Immunosuppressive therapy other than corticosteroids in the preceding year
  • Medically unfit for thymectomy
  • Chest CT evidence of thymoma.
  • Pregnancy or lactation; contraindications to the use of corticosteroids, unless postmenopausal or surgically sterile. Women considering becoming pregnant during the period of the study are to be excluded.
  • A serious concurrent medical, neurological or psychiatric condition that would interfere with thymectomy or subsequent clinical assessments
  • Current alternate day dose of prednisone > than 1.5 mg/kg or 100 mg or the equivalent daily doses (> 0.75 mg/kg or 50 mg).
  • Participation in another experimental clinical trial
  • History of alcohol or drug abuse within the 2 years prior to randomization.

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
Prednisone aloneprednisone aloneDrug: prednisone alone protocol
Thymectomy plus prednisonethymectomy plus prednisoneProcedure: Extended Transsternal Thymectomy plus prednisone treatment
Primary Outcome Measures
NameTimeMethod
Time-weighted Average Quantitative Myasthenia Gravis Weakness Score Over 3 Yearsbaseline, month 3, 4, 6 and every 3 months through 36 months

Myasthenia Gravis (QMG) test. QMG total scores range from 0 to 39 for a given visit, with higher scores indicating more severe disease. The time weighted average is a calculation that provides an integrated measure of the outcome over the time of followup. The denominator that was used to compute the time-weighted average for the Quantitative Myasthenia Gravis (QMG) score and the prednisone dose was the number of days from randomization to the last visit. Computations used the trapezoidal method where in the QMG score is multiplied by the number of days at this level from one visit to the next and added up over the entire followup experience and divided by the total number of days from randomization.

Time-weighted Average Alternate-day Prednisone Dose (mg) Measured Over 3 Yearsbaseline, month 1 , 2 , 3, 4, 6 and every 3 months through 36 months

Participants reported alternate-day prednisone dose (mg) intake from baseline through withdrawn or completed 3 years follow up. The prednisone dosages had been weighted over the days of reporting period.

Secondary Outcome Measures
NameTimeMethod
Subgroup Analyses of Time-weighted Average Quantitative Myasthenia Gravis Score by Prednisone Use at Enrollmentbaseline, month 3, 4, 6 and every 3 months through 36 months

Myasthenia Gravis (QMG) test. QMG total scores range from 0 to 39 for a given visit, with higher scores indicating more severe disease.

Subgroup Analyses of Time-weighted Average Quantitative Myasthenia Gravis Score by Sexbaseline, month 3, 4, 6 and every 3 months through 36 months

Myasthenia Gravis (QMG) test. QMG total scores range from 0 to 39 for a given visit, with higher scores indicating more severe disease.

Subgroup Analyses of Time-weighted Average Quantitative Myasthenia Gravis Score by Age at Disease Onsetbaseline, month 3, 4, 6 and every 3 months through 36 months

Myasthenia Gravis (QMG) test. QMG total scores range from 0 to 39 for a given visit, with higher scores indicating more severe disease.

Subgroup Analyses of Time-weighted Average Alternate-day Prednisone Dose (mg) by Prednisone Use at Enrollmentbaseline, month 3, 4, 6 and every 3 months through 36 months

Participants reported alternate-day prednisone dose (mg) intake from baseline through withdrawn or completed 3 years follow up. The prednisone dosages had been weighted over the days of reporting period.

Subgroup Analyses of Time-weighted Average Alternate-day Prednisone Dose (mg) by Sexbaseline, month 3, 4, 6 and every 3 months through 36 months

Participants reported alternate-day prednisone dose (mg) intake from baseline through withdrawn or completed 3 years follow up. The prednisone dosages had been weighted over the days of reporting period.

Subgroup Analyses of Time-weighted Average Average Alternate-day Prednisone Dose (mg) by Age at Disease Onsetbaseline, month 3, 4, 6 and every 3 months through 36 months

Participants reported alternate-day prednisone dose (mg) intake from baseline through withdrawn or completed 3 years follow up. The prednisone dosages had been weighted over the days of reporting period.

Number of Serious Adverse Eventsbaseline to 3 years

Number of participant who experienced at least one serious adverse events over 3 years: Thymectomy plus prednisone n=25 (out of 66); Prednisone alone n=33 (out of 60)

Number of Patients With at Least One Serious Adverse Eventsbaseline to 3 years

Number of participant who experienced at least one serious adverse events over 3 years: Thymectomy plus prednisone n=25 (out of 66); Prednisone alone n=33 (out of 60)

Classification of Serious Adverse Eventsbaseline to 3 years
Hospitalization for Exacerbation of Myasthenia Gravisbaseline to 2 years and baseline to 3 years
Cumulative Number of Hospital Daysbaseline to 3 years

Number who had hospitalization: Thymectomy plus prednisone n=15 (out of 66); Prednisone alone n=31 (out of 60)

Reason for Hospitalization According to Medical Dictionary for Regulatory Activities Termbaseline to 3 years

Number who had hospitalization: Thymectomy plus prednisone n=15 (out of 66); Prednisone alone n=31 (out of 60)

Time-weighted Average Prescribed Alternate Day Prednisone Dose (mg)baseline-day 20, month 1,2, 3, 4, 6 and every 3 months through 36 months

Physicians reported prescribed alternate-day prednisone dose (mg) intake from baseline through withdrawn or completed 3 years follow up. The prescribed prednisone dosages had been weighted over the days of reporting period.

Penalized Time-weighted Average Alternative Day Prednisone Dose (mg; Method 1: Penalized Using Maximum Dose Before Azathioprine)baseline, month 3, 4, 6 and every 3 months through 36 months

For each participant who took azathioprine, we penalized them by taking the maximum dose of prednisone before azathioprine was added. We then applied the same method to compute the time-weighted alternative day prednisone dose from baseline, month 3, 4, 6 and every 3 months through 36 months.

Penalized Time-weighted Average Alternative Day Prednisone Dose (mg; Method 2: Penalized Using Dose at Time of Starting Azathioprine)baseline, month 1 , 2 , 3, 4, 6 and every 3 months through 36 months

For each participant who took azathioprine, we penalized them by taking the prednisone dose at the time azathioprine commenced. We then applied the same method to compute the time-weighted alternative day prednisone dose from baseline, month 3, 4, 6 and every 3 months through 36 months.

Time-Weighted Average MG Activity of Daily Living (MG-ADL)baseline, month 4, 6 and every 3 months through 36 months

MG Activity of Daily Living total scores range from 0 to 24, with the lower scores indicating better daily living quality of life.

Time-Weighted Average MG Activity of Daily Living (MG-ADL) at Month 12, 24, and 36Month 12, 24, and 36

MG Activity of Daily Living total scores range from 0 to 24 by visit, with the lower scores indicating better daily living quality of life.

Azathioprine Usebaseline to 3 years
Plasma Exchange Usebaseline to 3 years
Intravenous Immunoglobulin Usebaseline to 3 years
Minimal Manifestation (MM) Status at Month 12, 24 and 36Month 12, 24 and 36

Number of participants who were in minimal manifestation status at month 12, 24 and 36.

Cumulative Days in Hospital for Myasthenia Gravis Exacerbationbaseline to 3 years

Number of patients with MG exacerbation: Thymectomy plus prednisone=6 (out of 66); Prednisone alone=22 (out of 60)

Short Form-36 Standardized Physical ComponentMonth 0, Month 12, Month 24 and Month 36

Range from 0 to 100, the higher the physical component value, the better the mental health.

Short Form-36 Standardized Mental ComponentMonth 0, Month 12, Month 24 and Month 36

Range from 0 to 100, the higher the mental component value, the better the mental health.

Treatment Associated Complications (TAC)Month 0, 1, 2, 3, 4 then every 3 months through Month 36

Treatment associated complications measured complications occurred by myasthenia gravis patients. Report number of participant with at least one complications by each visit.

Treatment Associated Symptoms (TAS)Month 0, 1, 2, 3, 4 then every 3 months through Month 36

Treatment associated symptoms measured myasthenia gravis symptoms such as back pain and/or bruises. Report number of participant with at least one treatment associated symptoms by each visit.

Trial Locations

Locations (70)

University of Southern California, Doheny Institute, 1450 San Pablo St

🇺🇸

Los Angeles, California, United States

Emory University, 201 Dowman Dr

🇺🇸

Atlanta, Georgia, United States

University of Miami, 1120 NW 14th Street, Suite 1300

🇺🇸

Miami, Florida, United States

Nerve and Muscle Center of Texas, 6624 Fannin St # 1670

🇺🇸

Houston, Texas, United States

The University of Kansas Medical Center, 3901 Rainbow Blvd.

🇺🇸

Kansas City, Kansas, United States

University of Buenis, Centro de Asistencia Docencia e Investigacion en Miastenia (CADIMI) Av. Forest 1146 - Ciudad Autonoma de Buenos Aires

🇦🇷

Buenos Aires, Argentina

Hospital Del Salvador, Departamento de Ciencias Neurológicas, Universidad de Chile, Salvador 95 Of 416, Providencia

🇨🇱

Santiago, Chile

Ramathibodi Hospital, Mahidol University

🇹🇭

Bangkok, Thailand

Johannes-Gutenberg University, Klinikum der Johannes Gutenberg-Universität, Klinik und Poliklinik für Neurologie, Langenbeckstr

🇩🇪

Mainz, Rhineland-Palatinate, Germany

McGill University Health Center

🇨🇦

Montreal, Quebec, Canada

Leiden University

🇳🇱

Leiden, Netherlands

University of Heidelberg, Seminarstraße 2

🇩🇪

Mannheim, Baden-Württemberg, Germany

Institute of Tuberculosis and Lung Disease

🇵🇱

Warsaw, Województwo, Poland

Porto University, Serviço de Neurologia,Hospital Geral de Santo António, Largo Prof Abel Salazar

🇵🇹

Porto, Portugal

University of Regensburg, Dept. of Neurology, Universitätsstr. 84, D

🇩🇪

Regensburg, Bavaria, Germany

University of Calgary, Heritage Medical Research Clinic Room 1132 3330 Hospital Dr NW

🇨🇦

Calgary, Alberta, Canada

National Neurological Institute "Carlo Besta", Myopathology and Immunology Unit, Dept of Neurology IV, Natl. Neurolog Inst. "C. Besta", Via Celoria, 11,

🇮🇹

Milan, Italy

Universidade Federal do Parana

🇧🇷

Curitiba, Brazil

William Beaumont Hospital, 3601 W. Thirteen Mile Road, Royal Oak

🇺🇸

Royal Oak, Michigan, United States

University of Texas Medical Branch, 301 University Blvd

🇺🇸

Galveston, Texas, United States

Robert Wood Johnson University,

🇺🇸

New Brunswick, New Jersey, United States

Mount Sinai Hospital,1 Gustave L. Levy Pl

🇺🇸

New York, New York, United States

University of Rochester, 601 Elmwood Ave

🇺🇸

Rochester, New York, United States

Case Western Reserve University, University Hospitals of Cleveland, 1100 Euclid Avenue

🇺🇸

Cleveland, Ohio, United States

University of Vermont College of Medicine, Given Bldg C225, 89 Beaumont Avenue

🇺🇸

Burlington, Vermont, United States

The Ohio State University Wexmer Medical Center, Rm 461 Means Hall, The Ohio State University Medical Center,1654 Upham Dr.

🇺🇸

Columbus, Ohio, United States

University of British Columbia

🇨🇦

Vancouver, British Columbia, Canada

University of Sydney, Royal Prince Alfred Hospital and The University of Sydney

🇦🇺

Sydney, Australia

University of Oxford, Dept of Clinical Neurology, University of Oxford, Radcliffe Infirmary

🇬🇧

Oxford, United Kingdom

University of Münster, Schlossplatz 2

🇩🇪

Münster, Germany

University of Ottawa, The Ottawa Hospital General Campus, Division of Neurology, 501 Smyth Rd. Box 601

🇨🇦

Ottawa, Ontario, Canada

Hospital de Base do Distrito Federal

🇧🇷

Brasilia, Brazil

University of Melbourne, Melbourne, The Royal Melbourne Hospital, Dept of Neurology, Royal Melbourne Hospital

🇦🇺

Victoria, Australia

University of Rome "Sapienza"

🇮🇹

Rome, Italy

Catholic University, Universita Cattolica del Sacro Cuore, Largo Agostino Gemelli 8, e

🇮🇹

Rome, Italy

Queen Elizabeth University Hospital, Glasgow

🇬🇧

Glasgow, United Kingdom

Instituto Nacional de la Nutrición

🇲🇽

Mexico, Mexico

University of Torino

🇮🇹

Torino, Italy

Walton Centre for Neurology and Neurosurgery, Liverpool Heart and Chest Hospital, Liverpool. The Walton Centre for Neurology and Neurosurgery, Lower Lane, Fazakerley

🇬🇧

Liverpool, United Kingdom

Kanazawa University, Department of Neurology, Kanazawa University Hospital, 13-1 Takaramachi

🇯🇵

Kanazawa, Ishikawa, Japan

University of Tübingen

🇩🇪

Tübingen, Germany

St. Louis University, One North Grand St. Louis

🇺🇸

St. Louis, Missouri, United States

H. Sant Pau, Universitat Autònoma de Barcelona, Neurology Department, Hospital Sta Creu i Sant Pau, C/Mas Casanovas no 90 4o pis 4o modul.

🇪🇸

Barcelona, Spain

University of Texas Southwestern Medical Center, 5232 Harry Hines Blvd,

🇺🇸

Dallas, Texas, United States

University of Washington, 1410 NE Campus Pkwy

🇺🇸

Seattle, Washington, United States

University of Virginia, 1215 Lee St

🇺🇸

Charlottesville, Virginia, United States

University of California Irvine, 101 The City Drive S, Bldg. 22 C, Route 13

🇺🇸

Orange, California, United States

Augusta University, 1120 15th St

🇺🇸

Augusta, Georgia, United States

Indiana University, Dept of Neurology, Regenstrief Health Center, 6th floor, 1050 Walnut St, Indiana University Medical Center

🇺🇸

Indianapolis, Indiana, United States

Brigham and Women's Hospital, 75 Francis Street, 5th Floor Tower

🇺🇸

Boston, Massachusetts, United States

Federal University of Rio De Janeiro

🇧🇷

Rio De Janeiro, Brazil

West Virginia University, Dept of Neurology, WVU Eye Institute, Neurology Suite, 1 Stadium Drive,

🇺🇸

Morgantown, West Virginia, United States

Nagasaki University, First Department of Internal Medicine,Graduate School of Biomedical Sciences,1-7-1,Sakamoto

🇯🇵

Nagasaki, Kyushu, Japan

Medical University of Warsaw

🇵🇱

Warsaw, Województwo, Poland

University of Cape Town, Division of Neurology E8-74, Groote Schuur Hospital,Observatory

🇿🇦

Cape Town, South Africa

University of Sheffield, Western Bank

🇬🇧

Sheffield, United Kingdom

Fu-Jen Catholic University, No. 510, Zhongzheng Rd., Xinzhuang Dist

🇨🇳

New Taipei, Taiwan

University of Alabama at Birmingham, Department of Neurology, Sparks Center, Suite 350, 1720 7th Avenue South

🇺🇸

Birmingham, Alabama, United States

Wayne State University School of Medicine, 4201 St Antoine, 8D UHC

🇺🇸

Detroit, Michigan, United States

Data Coordination Center: University of Alabama at Birmingham

🇺🇸

Birmingham, Alabama, United States

Barrow Neurological Institute, Saint Joseph's Hospital and Medical Center, 350 W Thomas Rd

🇺🇸

Phoenix, Arizona, United States

California Pacific Medical Center, Castro St & Duboce Ave

🇺🇸

San Francisco, California, United States

University of Minnesota, Department of Neurology, MMC 295, 420 Delaware St. S.E.,

🇺🇸

Minneapolis, Minnesota, United States

Mayo Clinic Rochester, 200 First St. SW

🇺🇸

Rochester, Minnesota, United States

Duke University, 200 Trent Dr

🇺🇸

Durham, North Carolina, United States

University of Texas Health Science Center, Mail code 7883, 7703 Floyd Curl Drive

🇺🇸

San Antonio, Texas, United States

Medical College of Wisconsin, 8701 Watertown Plank Road

🇺🇸

Milwaukee, Wisconsin, United States

University of Florida Jacksonville, Tower I, 8th Floor, 580 W. 8th ST.

🇺🇸

Jacksonville, Florida, United States

University of Düsseldorf

🇩🇪

Düsseldorf, North Rhine-Westphalia, Germany

University of Manchester, Oxford Road

🇬🇧

Manchester, United Kingdom

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