Ravulizumab

ULTOMIRIS is a complement inhibitor indicated for: the treatment of adult and pediatric patients one month of age and older with paroxysmal nocturnal hemoglobinuria (PNH). the treatment of adult and pediatric patients one month of age and older with atypical hemolytic uremic syndrome (aHUS) to inhibit complement-mediated thrombotic microangiopathy (TMA). Limitations of Use: ULTOMIRIS is not indicated for the treatment of patients with Shiga toxin E. coli related hemolytic uremic syndrome (STEC-HUS). the treatment of adult patients with generalized myasthenia gravis (gMG) who are anti-acetylcholine receptor (AChR) antibody-positive. the treatment of adult patients with neuromyelitis optica spectrum disorder (NMOSD) who are anti-aquaporin-4 (AQP4) antibody-positive. to reduce proteinuria in adults with primary immunoglobulin A nephropathy (IgAN) at risk for disease progression.

Ravulizumab-cwvz is a terminal complement inhibitor that specifically binds to the complement protein C5 with high affinity, thereby inhibiting its cleavage to C5a (the proinflammatory anaphylatoxin) and C5b (the initiating subunit of the membrane attack complex [MAC or C5b-9]) thus preventing MAC formation. ULTOMIRIS inhibits terminal complement-mediated intravascular hemolysis in patients with PNH and complement-mediated thrombotic microangiopathy (TMA) in patients with aHUS. The precise mechanism by which ravulizumab-cwvz exerts its therapeutic effect in gMG patients is unknown, but is presumed to involve reduction of terminal complement complex C5b-9 deposition at the neuromuscular junction.

See Full Prescribing Information for instructions on dosage, preparation, and administration. Dilute ULTOMIRIS before use. Only administer as an intravenous infusion through a 0.2 or 0.22 micron filter.

ULTOMIRIS is contraindicated for initiation in patients with unresolved serious Neisseria meningitidis infection.

Boxed warning (serious meningococcal infections ultomiris): a complement inhibitor, increases the risk of serious infections caused by Neisseria meningitidis. Life-threatening and fatal meningococcal infections have occurred in patients treated with complement inhibitors. These infections may become rapidly life-threatening or fatal if not recognized and treated early. Other Infections: Use caution when administering ULTOMIRIS to patients with any other systemic infection. Infusion-Related Reactions: Monitor during infusion, interrupt for reactions, and institute appropriate supportive measures.

The following clinically significant adverse reactions are discussed in greater detail in other sections of the labeling: Serious Meningococcal Infections Other Infections Infusion-Related Reactions Most common adverse reactions in patients with PNH (incidence ≥ 10%) were upper respiratory tract infection and headache. Most common adverse reactions in patients with aHUS (incidence ≥ 20%) were upper respiratory tract infection, diarrhea, nausea, vomiting, headache, hypertension, and pyrexia. Most common adverse reactions in adult patients with gMG (incidence ≥ 10%) were diarrhea and upper respiratory tract infection. Most common adverse reactions in adult patients with NMOSD (incidence ≥ 10%) were COVID-19, headache, back pain, arthralgia, and urinary tract infection. Most common adverse reaction in adult patients with IgAN (incidence ≥ 10%) was upper respiratory tract infection.

Plasma Exchange, Plasmapheresis, or Intravenous Immunoglobulins: concomitant use requires supplemental dose of ULTOMIRIS. Neonatal Fc Receptor Blockers (FcRn): Closely monitor for reduced effectiveness of ULTOMIRIS.

Store ULTOMIRIS vials refrigerated at 2°C to 8°C (36°F to 46°F) in the original carton to protect from light. Do not freeze. Do not shake. Refer to Dosage and Administration for information on the stability and storage of diluted solutions of ULTOMIRIS.