Table 5.

Recommendations for routine baseline and risk mitigation checklist in pediatric immunosuppression.

1- BASELINE DIAGNOSTIC TESTS

• Brain, orbit, and spinal cord MRI, with and without gadolinium • Visual battery as clinically indicated (High Contrast and Low Contrast Visual Acuity, Visual Evoked Potentials, Visual Fields, Optical Coherence Tomography, Color Vision) • Full blood cell count – leukocytes / platelets • Liver function tests, urea, creatinine • Cholesterol, triglycerides • Pregnancy test (in juvenile female patients) • Immunoglobulin levels • Lymphocyte subsets (CD19, CD20) • Serum complement (C3, C4) • Serum protein electrophoresis • Vitamin D serum level • AQP4-IgG test in serum (CBA) • MOG-IgG test in serum (CBA) • CSF analysis - cell count, cultures, IEF for oligoclonal bands in CSF and serum • Infection screening

a) HCV and HIV serology  b) HBV serology: HBV surface antigen and core antibody  c) TB tests: QuantiFERON or tuberculin skin testing and chest radiograph

2- BASELINE INVESTIGATION FOR ASSOCIATED AUTOIMMUNITY • Antinuclear antibodies (ANA) • Extractable nuclear antigen-antibodies (ENA) • Anti-DNA antibodies • Antithyroid antibodies and thyroid function tests (TSH, T3, Free T4, Total T4) • Celiac disease 3- RISK MITIGATION FOR INFECTIOUS EVENT AT BASELINE • Varicella zoster virus (VZV) serology • HBV serology: surface antibody • Vaccinations: complete immunization according to age (non-live vaccines >4 weeks, live vaccines >8 weeks prior to first infusion); complete pneumococcal vaccine • Complete immunization if necessary, according to VZV and HBV serology

4- INFUSION DAY CHECKLIST FOR RITUXIMAB/OCRELIZUMAB • Evaluations:

∘ Clinical assessment to exclude active infection

∘ Body weight, body temperature

∘ Blood cell count and liver function tests

•Pre-treatment regimen (to reduce infusion-related reactions)  a) Corticosteroids: Hydrocortisone, 1 mg/kg/dose  b) Antihistamines: Diphenhydramine, 1 mg/kg/dose (Max 50 mg)  c) Antipyretics: Acetaminophen, 10 mg/kg/dose 5- GENERAL ASPECTS OF MONITORING IMMUNOSUPPRESSION (specific requirements depending on the molecule) • Clinical disease activity • Repeat neuroimaging for new baseline: Brain, orbit, and spinal cord MRI (4–6 months) • Blood

a) Full blood cell count—leukopenia, lymphopenia  b) Liver enzymes  c) Urea, creatinine  d) Cholesterol, triglycerides  e) Lymphocyte subsets  f) Serum complement  g) Immunoglobulin levels • Urine  a) Infection;

b) Renal dysfunction • New associated autoimmunity

a) Thyroid function tests and thyroid antibodies  b) Antinuclear antibodies/ENA  c) Celiac disease