ECULIZUMAB INJ,SOLN

Clinical Criteria Summary

Shiga toxin E. coli-related hemolytic uremic syndrome (STEC-HUS)
Active infection with Neisseria meningitidis, Neisseria gonorrhoeae, Haemophilus influenzae, or Streptococcus pneumoniae
Concomitant therapy with rituximab or any other complement inhibitor such as ravulizumab
Concomitant chronic therapy with plasma exchange

Must be prescribed by or in consultation with a VA or VA Community Care nephrology or hematology provider or locally designated expert
Intolerance, contraindication, or inadequate symptom control to ravulizumab over at least a 2-month period
Patient is vaccinated against pneumococcal disease
Patient is vaccinated with BOTH the protein conjugate ACWY meningococcal vaccine and the type B meningococcal vaccine (in emergent cases begin vaccination series at initial dosing and provide antibiotic prophylaxis until 2 weeks after vaccination)

Requires treatment of aHUS with documentation ruling out Shiga toxin E. coli-related hemolytic uremic syndrome (STEC-HUS) and thrombotic thrombocytopenia purpura (TTP) (e.g., clinical evaluation and/or rule out of ADAMTS13 deficiency)
Requires prophylaxis in renal transplant to ensure aHUS does not recur in the transplanted kidney

Active infection with Neisseria meningitidis, Neisseria gonorrhoeae, Haemophilus influenzae, or Streptococcus pneumoniae
Concomitant therapy with rituximab, neonatal Fc receptor antagonists (e.g., efgartigimod), or any other complement inhibitor (e.g., ravulizumab)
Concomitant chronic therapy with intravenous immunoglobulin or plasma exchange (these treatments may be used for exacerbations)
Thymectomy within 12 months

Care provided by a VA or VA Community Care neurology provider or locally designated expert
Diagnosis of acetylcholine receptor (AChR) antibody positive generalized myasthenia gravis (gMG)
Not a candidate for thymectomy
Patient has a Myasthenia Gravis Foundation of America (MGFA) Clinical Classification of class II to IV
Patient has a Myasthenia Gravis-specific Activities of Daily Living scale (MG-ADL) total score ≥ 6
Patient is vaccinated against pneumococcal disease and Neisseria meningitidis with both Meningococcal conjugate vaccine (MenACWY) and Meningococcal serogroup B vaccine
Intolerance, contraindication, or inadequate symptom control to ravulizumab or zilucoplan over at least a 6-month period
Additional Inclusion Criteria (One of the following must be met)
Inadequate symptom control to maximally tolerated pyridostigmine AND at least two immunosuppressive agents separately trialed for at least 6 months each [e.g., azathioprine, cyclosporine, mycophenolate, etc.]
History of intolerance or contraindication preventing trial of immunosuppressive agents [e.g., azathioprine, cyclosporine, mycophenolate, etc.]
Lack of symptom control despite 4 or more courses of plasma exchange, high-dose steroid bursts and/or intravenous immune globulin within a 12 month or less period

In emergent cases where waiting to initiate eculizumab at least two weeks after completion of vaccination is not possible, patients should begin the vaccination series at the time of initial eculizumab dosing and be provided with appropriate prophylaxis.

Active infection with Neisseria meningitidis, Neisseria gonorrhoeae, Haemophilus influenzae, or Streptococcus pneumoniae
Concomitant therapy with rituximab or any other complement inhibitor (such as ravulizumab)
Concomitant chronic therapy with intravenous immunoglobulin or plasma exchange (use of these treatments during exacerbations is reasonable while on eculizumab)

Care provided by VA or VA Community Care neurology provider
Vaccination against pneumococcal disease and Haemophilus influenzae type B
Vaccination with both the protein conjugate ACWY meningococcal vaccine and the type B meningococcal vaccine (in emergent cases, begin vaccination series at initial dosing and provide antibiotic prophylaxis until 2 weeks after vaccination)

Active infection with Neisseria meningitidis, Neisseria gonorrhoeae, Haemophilus influenzae, or Streptococcus pneumoniae

Prescribed by or in consultation with a VA or VA Community Care hematology, oncology, immunology provider or locally designated expert
Vaccinated against pneumococcal disease
Vaccinated with both the protein conjugate ACWY meningococcal vaccine and the type B meningococcal vaccine (in emergent cases, begin vaccination series at initial dosing and provide antibiotic prophylaxis until 2 weeks after vaccination)
Laboratory-confirmed diagnosis of paroxysmal nocturnal hemoglobinuria (PNH), evidenced by detectable glycosylphosphatidylinositol (GPI)-deficient hematopoietic clones (Type III PNH red blood cells [RBC]) via Flow Cytometry, with pathology report documentation indicating presence of PNH-type RBC
Lactate dehydrogenase (LDH) level of 1.5 times the upper limit of the normal range (laboratory results with reference range must be available for review)
Documentation that a meningococcal vaccine was administered at least two weeks prior to the first dose of eculizumab

Neisseria meningitidis, Neisseria gonorrhoeae, Haemophilus influenzae, or Streptococcus pneumoniae active infection
Active, clinically significant infection
Concomitant biologic therapy for neuromyelitis optica spectrum disorder (NMOSD)
Concomitant chronic therapy with intravenous immunoglobulin or plasma exchange (use of these treatments in exacerbations is reasonable)

Care provided by VA or VA Community Care neurology provider or locally designated expert
Diagnosis of aquaporin-4 antibody positive NMOSD
Patient is vaccinated against pneumococcal disease
Patient is vaccinated with BOTH the protein conjugate ACWY meningococcal vaccine and the type B meningococcal vaccine (in emergent cases begin vaccination series at initial dosing and provide antibiotic prophylaxis until 2 weeks after vaccination)
Contraindication, intolerance, or lack of therapeutic response to rituximab