ph-0207
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Ruconest® (C1 Esterase Inhibitor [recombinant])

Policy Number: PH-0207

Intravenous

 

Last Review Date: 10/01/2020

Date of Origin: 08/26/2014

Dates Reviewed: 03/2015, 06/2015, 09/2015, 12/2015, 03/2016, 06/2016, 09/2016, 12/2016, 03/2017, 06/2017, 09/2017, 12/2017, 03/2018, 06/2018, 10/2018, 10/2019, 03/2020, 10/2020

 

FOR PEEHIP Members Only -Coverage excludes the provider-administered medication(s) outlined in this drug policy from being accessed through a specialty pharmacy. It must be obtained through buy and bill.

 

  1. Length of Authorization

Coverage will be provided for 12 weeks and is eligible for renewal (unless otherwise specified).

The cumulative amount of medication(s) the patient has on-hand, indicated for the acute treatment of HAE, will be taken into account when authorizing. The authorization will provide a sufficient quantity in order for the patient to have a cumulative amount of HAE medication(s) on-hand in order to treat up to 4 acute attacks per 4 weeks for the duration of the authorization (unless otherwise specified).

  1. Dosing Limits

A.  Quantity Limit (max daily dose) [Pharmacy Benefit]:

  • Ruconest 2100 IU vial: 16 vials every 28 days

B.  Max Units (per dose and over time) [Medical Benefit]:

  • 3360 billable units per 28 days
  1. Initial Approval Criteria1

Submission of medical records related to the medical necessity criteria is REQUIRED on all requests for authorizations. Records will be reviewed at the time of submission. Please provide documentation via direct upload through the PA web portal or by fax.

Coverage is provided in the following conditions:

  • Patient is at least 13 years of age; AND

Universal Criteria1,13,19

  • Must be prescribed by, or in consultation with, a specialist in: allergy, immunology, hematology, pulmonology, or medical genetics; AND
  • Confirmation the patient is avoiding the following possible triggers for HAE attacks:
  • Estrogen-containing oral contraceptive agents AND hormone replacement therapy; AND
  • Antihypertensive agents containing ACE inhibitors; AND
  • Dipeptidyl peptidase IV (DPP-IV) inhibitors (e.g., sitagliptin); AND
  • Neprilysin inhibitors (e.g., sacubitril)

Treatment of acute abdominal, peripheral, or facial attacks of Hereditary Angioedema (HAE) † Ф 1,13,19

  • Patient has a history of moderate to severe cutaneous attacks (without concomitant hives) OR abdominal attacks OR mild to severe airway swelling attacks of HAE (i.e. debilitating cutaneous/gastrointestinal symptoms OR laryngeal/pharyngeal/tongue swelling); AND
  • Patient has one of the following clinical presentations consistent with a HAE subtype, which must be confirmed by repeat blood testing (treatment for acute attack should not be delayed for confirmatory testing):

HAE I (C1-Inhibitor deficiency)

  • Low C1 inhibitor (C1-INH) antigenic level (C1-INH antigenic level below the lower limit of normal as defined by the laboratory performing the test); AND
  • Low C4 level (C4 below the lower limit of normal as defined by the laboratory performing the test); AND
  • Low C1-INH functional level (C1-INH functional level below the lower limit of normal as defined by the laboratory performing the test); AND
    • Patient has a  family history of HAE; OR
    • Acquired angioedema has been ruled out (i.e., patient onset of symptoms occur prior to 30 years old, normal C1q levels, patient does not have underlying disease such as lymphoma or benign monoclonal gammopathy [MGUS], etc.)

HAE II (C1-Inhibitor dysfunction)

  • Normal to elevated C1-INH antigenic level; AND
  • Low C4 level (C4 below the lower limit of normal as defined by the laboratory performing the test); AND
  • Low C1-INH functional level (C1-INH functional level below the lower limit of normal as defined by the laboratory performing the test)

HAE with normal C1INH (formerly known as HAE III)

  • Normal C1-INH antigenic level; AND
  • Normal C4 level; AND
  • Normal C1-INH functional level; AND
  • Repeat blood testing during an attack has confirmed the patient does not have abnormal lab values indicative of HAE I or HAE II; AND
  • Either of the following:
    • Patient has a known HAE-causing mutation (e.g., mutation of coagulation factor XII gene [F12 mutation], mutation in the angiopoietin-1 gene, mutation in the plasminogen gene, etc.); OR
    • Patient has a  family history of HAE and documented evidence of lack of efficacy of chronic high-dose antihistamine therapy (e.g. cetirizine standard dosing at up to four times daily or an alternative equivalent, given for at least one month or an interval long enough to expect three or more angioedema attacks) AND corticosteroids with or without omalizumab

FDA Approved Indication(s); Ф Orphan Drug

  1. Renewal Criteria1
  • Patient continues to meet the universal and other indication-specific relevant criteria identified in section III; AND
  • Significant improvement in severity and duration of attacks have been achieved and sustained; AND
  • Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include the following: hypersensitivity reactions, serious thromboembolic events (arterial or venous), etc.; AND
  • The cumulative amount of medication(s) the patient has on-hand, indicated for the acute treatment of HAE, will be taken into account when authorizing. The authorization will provide a sufficient quantity in order for the patient to have a cumulative amount of HAE medication(s) on-hand in order to treat up to 4 acute attacks per 4 weeks for the duration of the authorization (unless otherwise specified).
  1. Dosage/Administration1

Indication

Dose

Acute Hereditary Angioedema (HAE) attack

Body weight < 84 kg:

50 international units (IU) per kg body weight by intravenous injection

Body weight ≥ 84 kg:

4200 IU (2 vials) by intravenous injection

If the attack symptoms persist, an additional (second) dose can be administered at the recommended dose level. Do not exceed 4200 IU per dose. No more than two doses should be administered within a 24 hour period.

  1. Billing Code/Availability Information

HCPCS Code:

  • J0596 - Injection, c1 esterase inhibitor (recombinant), Ruconest, 10 units; 1 billable unit = 10 units

NDC:

  • Ruconest 2100 IU single-use 25 mL vial: 68012-0350-xx
  • Ruconest 2100 IU single-use 25 mL vial: 71274-0350-xx
  1. References
  1. Ruconest [package insert]. Bridgewater, NJ; Pharming Healthcare, Inc; December 2019.  Accessed September 2020.
  2. Riedl MA, Grivcheva-Panovska V, Moldovan D, et al. Recombinant human C1 esterase inhibitor for prophylaxis of hereditary angio-oedema: a phase 2, multicentre, randomised, double-blind, placebo-controlled crossover trial. Lancet. 2017;390(10102):1595-1602.
  3. Bowen T, Cicardi M, Farkas H, et al. Canadian 2003 International Consensus Algorithm For the Diagnosis, Therapy, and Management of Hereditary Angioedema. J Allergy Clin Immunol. 2004 Sep;114(3):629-37.
  4. Bygum A, Andersen KE, Mikkelsen CS. Self-administration of intravenous C1-inhibitor therapy for hereditary angioedema and associated quality of life benefits. Eur J Dermatol. Mar-Apr 2009;19(2):147-151.
  5. Bowen T, Cicardi M, Farkas H, et al. 2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema. Allergy Asthma Clin Immunol. 2010;6(1):24.
  6. Craig T, Aygören-Pürsün E, Bork K, et al.  WAO Guideline for the Management of Hereditary Angioedema.  World Allergy Organ J. 2012 Dec;5(12):182-99.
  7. Gompels MM, Lock RJ, Abinun M, et al.  C1 inhibitor deficiency: consensus document.  Clin Exp Immunol. 2005;139(3):379.
  8. Betschel S, Badiou J, Binkley K, et al. Canadian hereditary angioedema guideline. Asthma Clin Immunol. 2014 Oct 24;10(1):50. doi: 10.1186/1710-1492-10-50.
  9. Zuraw BL, Bernstein JA, Lang DM, et al. A focused parameter update: hereditary angioedema, acquired C1 inhibitor deficiency, and angiotensin-converting enzyme inhibitor-associated angioedema. J Allergy Clin Immunol. 2013 Jun;131(6):1491-3. doi: 10.1016/j.jaci.2013.03.034.
  10. Zuraw BL, Banerji A, Bernstein JA, et al. US Hereditary Angioedema Association Medical Advisory Board 2013 recommendations for the management of hereditary angioedema due to C1 inhibitor deficiency. J Allergy Clin Immunol Pract. 2013 Sep-Oct;1(5):458-67.
  11. Frank MM, Zuraw B, Banerji A, et al. Management of children with Hereditary Angioedema due to C1 Inhibitor deficiency. Pediatrics. 2016 Nov. 135(5)
  12. Zuraw BL, Bork K, Binkley KE, et al. Hereditary angioedema with normal C1 inhibitor function: Consensus of an international expert panel. Allergy Asthma Proc. 2012;33 Suppl 1:145-156.
  13. Maurer M, Mager M, Ansotegui I, et al. The international WAO/EAACI guideline for the management of hereditary angioedema-The 2017 revision and update. Allergy. 2018 Jan 10. doi: 10.1111/all.13384.
  14. Lang DM, Aberer W, Bernstein JA, et al. International consensus on hereditary and acquired angioedema. Ann Alergy Asthma Immunol. 2012;109:395-402.
  15. Wintenberger C, Boccon-Gibod I, Launay D, et al. Tranexamic acid as maintenance treatment for non-histaminergic angioedema: analysis of efficacy and safety in 37 patients. Clin Exp Immunol. 2014 Oct; 178(1): 112–117.
  16. Saule C, Boccon-Gibod I, Fain O, et al. Benefits of progestin contraception in non-allergic angioedema. Clin Exp Allergy. 2013 Apr;43(4):475-82.
  17. Frank MM, Sergent JS, Kane MA, et al. Epsilon aminocaproic acid therapy of hereditary angioneurotic edema; a double-blind study. N Engl J Med. 1972:286:808-812.
  18. Riedl MA, Bernstein JA, Li H, et al. Recombinant human C1-esterase inhibitor relieves symptoms of hereditary angioedema attacks: phase 3, randomized, placebo-controlled trial. Ann Allergy Asthma Immunol. 2014;112(2):163-169.e1.
  19. Betschel S, Badiou J, Binkley K, et al. The International/Canadian Hereditary Angioedema Guideline. Allergy Asthma Clin Immunol. 2019; 15: 72. Published online 2019 Nov 25.  doi: 10.1186/s13223-019-0376-8.

Appendix 1 – Covered Diagnosis Codes

ICD-10

ICD-10 Description

D84.1

 Defects in the complement system

Appendix 2 – Centers for Medicare and Medicaid Services (CMS)

Medicare coverage for outpatient (Part B) drugs is outlined in the Medicare Benefit Policy Manual (Pub. 100-2), Chapter 15, §50 Drugs and Biologicals. In addition, National Coverage Determination (NCD), Local Coverage Determinations (LCDs), and Local Coverage Articles (LCAs) may exist and compliance with these policies is required where applicable. They can be found at: http://www.cms.gov/medicare-coverage-database/search/advanced-search.aspx. Additional indications may be covered at the discretion of the health plan.

Medicare Part B Covered Diagnosis Codes (applicable to existing NCD/LCD/LCA): N/A

Medicare Part B Administrative Contractor (MAC) Jurisdictions

Jurisdiction

Applicable State/US Territory

Contractor

E (1)

CA, HI, NV, AS, GU, CNMI

Noridian Healthcare Solutions, LLC

F (2 & 3)

AK, WA, OR, ID, ND, SD, MT, WY, UT, AZ

Noridian Healthcare Solutions, LLC

5

KS, NE, IA, MO

Wisconsin Physicians Service Insurance Corp (WPS)

6

MN, WI, IL

National Government Services, Inc. (NGS)

H (4 & 7)

LA, AR, MS, TX, OK, CO, NM

Novitas Solutions, Inc.

8

MI, IN

Wisconsin Physicians Service Insurance Corp (WPS)

N (9)

FL, PR, VI

First Coast Service Options, Inc.

J (10)

TN, GA, AL

Palmetto GBA, LLC

M (11)

NC, SC, WV, VA (excluding below)

Palmetto GBA, LLC

L (12)

DE, MD, PA, NJ, DC (includes Arlington & Fairfax counties and the city of Alexandria in VA)

Novitas Solutions, Inc.

K (13 & 14)

NY, CT, MA, RI, VT, ME, NH

National Government Services, Inc. (NGS)

15

KY, OH

CGS Administrators, LLC

 

 

RUCONEST® (C1 Esterase Inhibitor [recombinant]) Prior Auth Criteria
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