A Study of Orelabrutinib in Patients With AQP4-IgG Positive Neuromyelitis Optica Spectrum Disorder

This is an interventional treatment trial for Neuromyelitis Optica Spectrum Disorder focused on measuring Neuromyelitis Optica Spectrum Disorder, BTK inhibitors, Orelabrutinib Read More

Inclusion Criteria:

• 1) 18-75 years old (inclusive) at the time of signing the informed consent form
• 2)Diagnosed with AQP4-IgG positive NMOSD in accordance with 2015 IPND diagnostic criteria.
• 3)Relapse ≥ 2 within 1 year before screening, and at least 1 relapse within 6 months before screening
• 4)If the subject has stable steroids treatment (≤ 7.5mg prednisone, or equivalent dose of steroids), the treatment needs to be stable more than 1 month before starting the study drug treatment.
• 5)EDSS ≤7.5 at screening
• 6)Negative pregnancy test for female of childbearing potential at screening
• 7)Understood the study procedure and voluntarily signed written informed consent

Exclusion Criteria:

• 1) History of serious heart, lung, liver, kidney, blood disease, etc.
• 2) Any major infection judged by the investigator requiring hospitalization and parenteral antimicrobial treatment within 1 month before screening
• 3) History of episodes of herpes zoster ≥ 2 or disseminated herpes zoster ≥ 1
• 4) History of or having any of the following medication / treatment: ① Received BTK inhibitor at any time in the past; ② B-cell targeted therapy within 12 weeks before the first dose; ③ Received biological agents within 12 weeks before the first dose; ④ Received live virus vaccine or live attenuated vaccine within 8 weeks before the first dose; ⑤ Received steroids treatment for other diseases within 6 months before screening, the dosage > 20mg / day for more than 21 days; ⑥ Used a study drug or other experimental treatment within 4 weeks before screening or 5 half-lives, or participating in any other intervention clinical trial.

• 5) During screening or baseline examination, laboratory results meet the exclusion criteria:

  • Human immunodeficiency virus (HIV) positive
  • Hepatitis C virus (HCV) antibody positive. (If a subject has a history of HCV infection, has completed and recorded appropriate treatment at least 1 year before screening, and the HCV RNA measured by PCR at the time of screening is negative, the subject will not be excluded from this study.)
  • Hepatitis B surface antigen (HBsAg) positive and / or hepatitis B core antibody (HBcAb) positive
  • Estimated glomerular filtration rate (eGFR) < 30 mL/min/1.73 m2
  • ALT/AST > 2 x ULN, Total Bilirubin > 1.5 x ULN, or any other clinically significant laboratory abnormality
  • Neutrophil < 1500 / mm3, platelet < 75000 / mm3, lymphocyte < 1000 / mm3 or leukocyte < 3500 / mm3.
  • International standardized ratio (INR) ≥ 1.5 or activated partial thromboplastin time (APTT) ≥ 1.5x ULN.
  • CD19 B cells lower than the lower limit of the normal range
• 6) Used strong to medium CYP3A inducers within 3 weeks before treatment, or strong to medium CYP3A inhibitors within 1 week before treatment, or strong to medium CYP3A inducers or inhibitors may be used during treatment.
• 7) There are situations that other researchers think are not suitable to participate in this study.