Eculizumab

SOLIRIS is a complement inhibitor indicated for: the treatment of patients with paroxysmal nocturnal hemoglobinuria (PNH) to reduce hemolysis. the treatment of patients with atypical hemolytic uremic syndrome (aHUS) to inhibit complement-mediated thrombotic microangiopathy. Limitation of Use SOLIRIS is not indicated for the treatment of patients with Shiga toxin E. coli related hemolytic uremic syndrome (STEC-HUS). the treatment of generalized myasthenia gravis (gMG) in adult and pediatric patients six years of age and older who are anti-acetylcholine receptor (AChR) antibody positive. the treatment of neuromyelitis optica spectrum disorder (NMOSD) in adult patients who are anti-aquaporin-4 (AQP4) antibody positive.

Eculizumab, the active ingredient in SOLIRIS, is a monoclonal antibody that specifically binds to the complement protein C5 with high affinity, thereby inhibiting its cleavage to C5a and C5b and preventing the generation of the terminal complement complex C5b-9. SOLIRIS inhibits terminal complement-mediated intravascular hemolysis in PNH patients and complement-mediated thrombotic microangiopathy (TMA) in patients with aHUS. The precise mechanism by which eculizumab 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. The precise mechanism by which eculizumab exerts its therapeutic effect in NMOSD is unknown, but is presumed to involve inhibition of aquaporin-4-antibody induced terminal complement C5b-9 deposition.

For intravenous infusion only, recommended dosage for: PNH: aHUS, gMG, and NMOSD in adults: aHUS and gMG in pediatric patients:

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

Boxed warning (serious meningococcal infections soliris): 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. Use caution when administering SOLIRIS to patients with any other systemic infection. Infusion-Related Reactions: Monitor patients during infusion, interrupt for reactions, and institute appropriate supportive measures.

The following serious adverse reactions are discussed in greater detail in other sections of the labeling: Serious Meningococcal Infections Other Infections Monitoring Disease Manifestations after SOLIRIS Discontinuation Thrombosis Prevention and Management Infusion-Related Reactions The most frequently reported adverse reactions in the PNH randomized trial (≥10% overall and greater than placebo) are: headache, nasopharyngitis, back pain, and nausea. The most frequently reported adverse reactions in aHUS single arm prospective trials (≥20%) are: headache, diarrhea, hypertension, upper respiratory infection, abdominal pain, vomiting, nasopharyngitis, anemia, cough, peripheral edema, nausea, urinary tract infections, pyrexia. The most frequently reported adverse reaction in the gMG placebo-controlled clinical trial (≥10%) in adult patients is musculoskeletal pain.

Plasmapheresis, Plasma Exchange, Fresh Frozen Plasma Infusion or IVIg Concomitant use of SOLIRIS with plasma exchange (PE), plasmapheresis (PP), fresh frozen plasma infusion (PE/PI), or in patients with gMG on concomitant IVIg treatment can reduce serum eculizumab concentrations and requires a supplemental dose of SOLIRIS.

Store SOLIRIS vials refrigerated at 2°-8° C (36°-46° F) in the original carton to protect from light until time of use. SOLIRIS vials may be stored in the original carton at controlled room temperature (not more than 25° C/77° F) for only a single period up to 3 days. DO NOT FREEZE. Refer to Dosage and Administration for information on the stability and storage of diluted solutions of SOLIRIS. DO NOT SHAKE.