Therapy in the Acute Phase of NMOSD: A Multicenter Prospective Real-World Study

Korábbi kezelés

• Those who have received PE/IA/IVIG/FcRn/B-cell deletion/C5/IL-6 treatment within 1 month before enrollment.

Más betegség

• Active infections: active hepatitis B, hepatitis C, syphilis or HIV infection; active systemic infections or immunodeficiency diseases; unrelieved meningococcal infection of the meninges, or patients with severe infections that cannot use immunosuppressive drugs.
• Patients with severe internal or external diseases (not limited to such as heart failure, unstable angina pectoris, respiratory failure, pulmonary insufficiency, cachexia, organ transplantation, etc.).
• Those who have had or currently have an untreated malignant tumor that is not well controlled.
• Patients with serious physical or mental diseases that may affect the smooth implementation of the study.
• Patients known to be allergic to monoclonal drugs, murine proteins or any excipients.

Laborértékek

• Abnormal laboratory indicators that need to be excluded from the subjects include, but are not limited to the following indicators:
  • Neutrophils \< 1.5 × 109/L, Hemoglobin \< 90g/L, Platelet count \< 75 × 109/L; Serum creatinine \> 1.5 × ULN, Total bilirubin \> 1.5 × ULN, Aspartate aminotransferase (AST) \> 1.5 × ULN, Alanine aminotransferase (ALT) \> 1.5 × ULN, Alkaline phosphatase \> 2 × ULN ； HbA1c \> 8% (for diabetic patients); GFR \< 60 mL/minute/1.73m2.

Egyéb

• Damage to the optic nerve or spinal cord caused by other non-NMOSD-related factors.
• Pregnant or lactating women, as well as those planning to become pregnant during the study period.
• Patients who are intolerant to methylprednisolone or gamma globulin.
• Patients who cannot complete the magnetic resonance enhanced scan screening.
• Patients who are participating in other interventional clinical trials.
• Patients who cannot understand the questionnaire questions or cooperate with the questionnaire survey.
• Situations that the research team collectively deems unsuitable for participation in this study.

• Age: 18 - 65 years old, gender not restricted.
• Patients who meet the diagnostic criteria for NMOSD as set by the International Panel for NMO Diagnosis (IPND) in 2015, and have positive serum AQP4-IgG (by CBA method or live cell method).
• Acute phase of NMOSD-ON, defined as new or worsening optic nerve dysfunction (visual acuity decline accompanied or not by eye pain and visual field defect), with an onset duration of ≤ 21 days, and clear evidence of new or recurrent optic nerve damage on imaging (new or expanded T2WI lesions, with enhancement); the best corrected visual acuity (BCVA) of the affected eye during the acute phase of NMOSD-ON (if both eyes are affected simultaneously, the worse eye is considered) drops from above 0.3 to ≤ 0.1.
• Acute phase of NMOSD-TM, defined as new or worsening spinal cord dysfunction (limb weakness or numbness, accompanied or not by urinary and defecation disorders), with an onset duration of ≤ 21 days, and clear evidence of new or recurrent spinal cord damage on imaging (new or expanded T2WI lesions, with enhancement); the EDSS score during the acute phase of NMOSD-TM increases from ≤ 4.0 to ≥ 6.0.
• Clinical onset and recurrence determination requires unanimous judgment by each center and the center committee (an independent group of 3 people).
• Agree to receive meningococcal vaccine or use eculizumab during and 2 weeks after the medication.
• Sign the informed consent.