Neuroimmune Diseases
Conditions
Interventions
MG:Telitacicept 160mg/240mg
NMOSD:Telitacicept 160mg/240mg
AE:Telitacicept 160mg/240mg
Sponsors
Linyi People's Hospital
Eligibility
Sex/Gender
All
Age
18 Years to 70 Years
Inclusion criteria
Inclusion criteria: 1. MG group (1) The patient voluntarily signs an informed consent form. (2) The age range is 18-70 years old, regardless of gender. (3) Patients diagnosed with general myasthenia gravis according to the following : 1) Fluctuating and fatigable skeletal muscle weakness is the important characteristic. Patients describe worsening of their symptoms late in the day or during sustained exercise. Transient improvement occurs with rest, and symptoms often progress during the day. 2) Pharmacological examination: 1.0-1.5mg (0.02mg/kg) of neostigmine and 0.5mg of atropine are administered intramuscularly to adult patients. Clinical absolute scores (CAS) and clinical relative scores (CRS) are estimated. CRS = 25% is negative, 25-60% is suspicious positive, and = 60% is positive. 3) EMG: The routinely detected muscles are the abductor digitorum, trapezius, and orbicularis oculi muscles. The presence of an electrodecremental response of the CMAP amplitude (=10%) during slow (2-5Hz) repetitive motor nerve stimulation is considered positive. If no significant decrease is found in the baseline, further RNS should be performed immediately, 1 minute, 2 minutes, and 3 minutes after 1 minute of exercise to determine whether there is wave amplitude attenuation caused by post exercise exhaustion. 4) Serological test: Positive for AchR-Ab, Musk-Ab, or LRP4-Ab, or negative for serum. 5) Excluding other muscle weakness related diseases such as Lambert-Eaton syndrome, Guillain Barre syndrome, etc. (4) The clinical classification of Myasthenia Gravis Foundation of America (MGFA) is grade II (including IIa and IIb) or grade III (including IIIa and IIIb). (5) The Quantitative Myasthenia Gravis Scale (QMGS) = 8 and at least 2 items QMGS = 8. (6) Maintain a stable standard treatment protocol: 1) Cholinesterase inhibitors: remain stable for at least 2 week before the study, with a required dosage of = 480mg/d; 2) Corticosteroids: Prednisone dose = 40mg/d or other equivalent dose of corticosteroids, stable for at least one month prior to study; 3) Tacrolimus or Mycophenolate Mofetil: stable for at least 3 months prior to study; 2. NMOSD group (1) The patient voluntarily signs an informed consent form. (2) The age range is 18-70 years old, regardless of gender. (3) Conform to the 2021 international consensus diagnostic criteria for Neuromyelitis Optica Spectrum Disorders, based on the combination of medical history, core clinical symptoms, imaging evidence, and biomarkers, stratificated by the level of AQP4-IgG, referenced other subclinical and immunological evidence, the patients were diagnosed. In addition, the possibility of other diseases needs to be excluded. (4) Acute phase of NMOSD: new onset of neurological symptoms within 30 days before screening or aggravated original symptoms, lasting for at least 24 hours. (5) NMOSD recurrence (including acute recurrence) = 2 relapses occurred in the 12 months before enrollment. (6) EDSS score = 7.5. 3. AE group (1) The patient voluntarily signs an informed consent form. (2) The age range is 18-70 years old, regardless of gender. (3) Conform to the Chinese expert consensus on the diagnosis and management of autoimmune encephalitis (2022 edition), based on the combination of core clinical symptoms, CSF tests, imaging evidence, and EEG characteristics, the patients were diagnosed as AE. 1) Clinical manifestation: acute or subacute onset (<3 months), with one or more of the following neurological and psychiatric symptoms or
Exclusion criteria
Exclusion criteria: 1)Comorbidity with other autoimmune diseases, such as SLE, rheumatoid arthritis, Sjogren's syndrome, etc; 2)Within 1 month before the study, patients were taking other immunosuppressive medicines other than standard treatment; 3)Within 6 months prior to enrollment, other biological targeted therapies such as rituximab were taken; 4)Intravenous immunoglobulin injection or plasma exchange therapy were adminstered within 2 months prior to enrollment; 5)The patient has significant cardiovascular, liver, kidney, respiratory system, endocrine or hematologic diseases; 6)Active present or chronic infection patients, such as HIV and acute hepatitis; 7)COVID-19 infection within the first 4 weeks and severe COVID-19 infections requiring hospitalization within the first 12 months prior to the study; 8) Patients with concomitant thymic tumors or after thymectomy within the past 6 months; 9)Received any live vaccine within the first 3 months of the study or planned to receive any vaccine during the study period; 10)Other malignant tumor patients; 11)Allergies to any human derived biological products; 12)Has participated in any clinical trial 28 days prior to the study or within 5 times the half-life of the study drug participating in the clinical trial; 13) Pregnant or lactating women and patients with fertility plans during the trial period; 14)The researchers believe that patients who are not suitable to participate in the study.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Changes in Modified Rankin Scale Score (mRS) from Baseline to week 24;The proportion of patients with adverse events (AE) and severe adverse events (SAE) ;Changes in serum antibodies, B-lymphocyte subpopulation, IgG, BlyS/BAFF, and APRIL compared to baseline at 12w and 24w; | — |
Secondary
| Measure | Time frame |
|---|---|
| Changes in Modified Rankin Scale Score (mRS) from Baseline to 4w and 12w;Changes in Quantitative Myasthenia Gravis score (QMG) from Baseline to 4w, 12w, 24w (MG);The proportion of patients who compliant with MMS and CTCAE = 1 at weeks 4w, 12w, and 24w (group MG);Changes in Expanded Disability Status Scale (EDSS) from Baseline to 4w, 12w and 24w (NMOSD);Changes in daily dosage of glucocorticoids/immunosuppressive compared baseline to 4w, 12w, and 24w; | — |
Countries
China
Contacts
Public ContactFaying QI
Linyi People's Hospital
Outcome results
None listed