Alpha-1-proteinase inhibitor

ARALAST NP is an Alpha 1 -Proteinase Inhibitor (Alpha 1 -PI) indicated for chronic augmentation therapy in adults with clinically evident emphysema due to severe congenital deficiency of Alpha 1 -PI (alpha 1 -antitrypsin deficiency). ARALAST NP increases antigenic and functional (anti-neutrophil elastase capacity, ANEC) serum levels and antigenic lung epithelial lining fluid levels of Alpha 1 -PI. The effect of augmentation therapy with any Alpha 1 -PI, including ARALAST NP, on pulmonary exacerbations and on the progression of emphysema in alpha 1 -antitrypsin deficiency has not been conclusively demonstrated in randomized, controlled clinical trials. Clinical data demonstrating the long-term effects of chronic augmentation and maintenance therapy with ARALAST NP or ARALAST are not available.

ARALAST NP administration is intended to inhibit serine proteases such as neutrophil elastase (NE), which is capable of degrading protein components of the alveolar walls and which is chronically present in the lung. Alpha 1 -PI deficiency is an autosomal, co-dominant, hereditary disorder characterized by low serum and lung levels of Alpha 1 -PI. 1,2,4,5 Severe forms of the deficiency are frequently associated with slowly progressive, moderate-to-severe panacinar emphysema that most often manifests in the third to fourth decades of life. 1,2,3,5,6 However, an unknown percentage of individuals with severe Alpha 1 -PI deficiency are not diagnosed with or may never develop clinically evident emphysema during their lifetimes.

For Intravenous Use Only For Intravenous Use Only Recommended dosage is 60 mg/kg body weight administered once weekly by intravenous infusion. Administer at a rate not to exceed 0.2 mL/kg body weight/minute, and as determined by the response and comfort of the patient.

ARALAST NP is contraindicated in immunoglobulin A (IgA) deficient patients with antibodies against IgA, due to the risk of severe hypersensitivity. Immunoglobulin A (IgA) deficient patients with antibodies against IgA.

Severe hypersensitivity and anaphylactic reactions may occur in IgA deficient patients with antibodies against IgA. May carry a risk of transmitting infectious agents such as viruses, the variant Creutzfeldt-Jakob disease (vCJD) and theoretically, the Creutzfeldt-Jakob disease (CJD) agent, despite manufacturing steps designed to minimize the risk of viral transmission.

Hypersensitivity reactions have been reported in patients following administration of ARALAST/ARALAST NP. No serious adverse reactions related to the use of ARALAST NP were reported in clinical trials. The most common adverse reactions occurring in ≥5% of infusions in clinical trials were headache, musculoskeletal discomfort, vessel puncture site bruise, nausea, and rhinorrhea. Most common adverse reaction occurring in ≥5% of infusions in clinical studies were headache, musculoskeletal discomfort, vessel puncture site bruise, nausea, and rhinorrhea.

Store ARALAST NP at temperatures not to exceed 25°C (77°F). Do not freeze. Store ARALAST NP in the original carton to protect from light.