Ravulizumab

eMC (Ultomiris 1,100 mg/11 mL concentrate for solution for infusion). Must be administered by a healthcare professional under the supervision of a physician experienced in haematological, renal, neuromuscular or neuroinflammatory disorders. The dosing schedule is allowed to vary occasionally by +/- 7 days of the scheduled infusion day (except for the first maintenance dose), but the subsequent dose should be given according to the original schedule. TREATMENT INITIATION: patients not currently on ravulizumab or eculizumab receive the loading dose at treatment start; patients currently treated with eculizumab receive the ravulizumab loading dose at the time of the next scheduled eculizumab dose — in both cases the first maintenance dose follows 2 weeks after the loading dose. SUPPLEMENTAL DOSING after plasma exchange (PE), plasmapheresis (PP) or IVIg (given within 4 hours of each PE/PP intervention or of completing an IVIg cycle): >= 40 to < 60 kg — most recent dose 2,400 mg: 1,200 mg after PE/PP; most recent dose 3,000 mg: 1,500 mg after PE/PP; 600 mg after an IVIg cycle. >= 60 to < 100 kg — most recent dose 2,700 mg: 1,500 mg after PE/PP; most recent dose 3,300 mg: 1,800 mg after PE/PP; 600 mg after an IVIg cycle. >= 100 kg — most recent dose 3,000 mg: 1,500 mg after PE/PP; most recent dose 3,600 mg: 1,800 mg after PE/PP; 600 mg after an IVIg cycle. DURATION: PNH is chronic and treatment is recommended for the patient's lifetime unless discontinuation is clinically indicated; in aHUS, treatment to resolve thrombotic microangiopathy should be for a minimum of 6 months, with longer/chronic therapy considered individually for patients at higher risk of recurrence; in gMG and NMOSD only chronic administration has been studied. Not studied in gMG patients with MGFA Class V. PAEDIATRIC (recorded here because the SPC paediatric doses are fixed weight-band milligram doses, not per-kg, so paedDose is null): children with PNH or aHUS weighing >= 40 kg are treated per the adult table; for PNH or aHUS with body weight >= 10 to < 20 kg — 600 mg loading and 600 mg maintenance every 4 weeks; >= 20 to < 30 kg — 900 mg loading and 2,100 mg maintenance every 8 weeks; >= 30 to < 40 kg — 1,200 mg loading and 2,700 mg maintenance every 8 weeks; the first maintenance dose is given 2 weeks after the loading dose. When switching from eculizumab, the paediatric loading dose is given 2 weeks after the last eculizumab infusion. Ravulizumab has not been studied in paediatric PNH patients weighing less than 30 kg (the recommended posology for these patients is extrapolated from aHUS PK/PD data). Safety and efficacy have not been established in children below 10 kg with PNH or aHUS, nor in children with gMG or NMOSD (no data). Clinician to verify all paediatric use against a children's formulary. PREPARATION: concentrate is supplied in 3 mL and 11 mL vials and must be diluted to a final concentration of 50 mg/mL; after administration flush the entire line with 0.9% sodium chloride injection. ELDERLY: no dose adjustment required for patients aged 65 and over.