COVERED USES:

FDA Approved Indications:

Congenital alpha1-proteinase inhibitor (alpha1-PI; alpha1-antitrypsin) deficiency: For chronic augmentation therapy in patients having congenital deficiency of alpha1-PI with clinically evident emphysema.

Clinical data demonstrating the long-term effects of chronic augmentation or replacement therapy of individuals with alpha1-PI are not available.

Aralast and Zemaira are not indicated as therapy for lung disease patients in whom congenital alpha1-PI deficiency has not been established.

Prolastin is not indicated for use in patients other than those with PiZZ, PiZ(null), or Pi(null)(null) phenotypes.

All FDA-approved indications not otherwise excluded from Part D.

AGE RESTRICTIONS:

NA

PRESCRIBER RESTRICTIONS:

NA

REQUIRED MEDICAL INFORMATION:

AAT serum concentrations

FEV₁

For initiation of treatment, a prior authorization form and relevant chart notes documenting medical rationale are required and for continuation of therapy, ongoing documentation of successful response to the medication may be necessary.

CRITERIA:

Must meet all of the following:

• Member is non-smoker, AND
• Member has alpha 1 antitrypsin (AAT) deficiency defined as AAT serum concentrations less than 80 mg/dL (or 11 µM/L); AND
• For Aralast and Zemaira, member has clinical emphysema, or for Prolastin, member has clinical evidence of panacinar emphysema.

EXCLUSION CRITERIA:

NA

COVERAGE DURATION:

Initial authorization will be approved for up to six months. Reauthorization will be approved for up to one year.