Berinert (C1 esterase inhibitor [human]) — HCPCS J0597
CareCost Estimate · Billing Cheat Sheet
CSL Behring
500 IU single-dose lyophilized vial
IV push ~5 min (≤ 4 mL/min)
HAE acute attack — on-demand only
Reviewed: May 2, 2026
ASP: Q2 2026
70 kg dose
140 units
1,400 IU (20 IU/kg)
Modifier
JZ / JW
Single-dose vial; waste common
Admin CPT
96365
~5-min IV push (clinic)
Medicare ASP+6%
$76.051
/10 IU · ~$7.61/IU · ~$10,647 / 70 kg dose
BILLER ERROR TRAP — J0597 IS PER 10 IU, NOT PER IU. A 70 kg patient at 20 IU/kg
= 1,400 IU bills as 140 units, not 1,400 units. ALWAYS divide total IU by 10.
Billing IU as units overstates the claim by 10× and triggers automatic payer takeback.
LARYNGEAL ATTACKS: ALWAYS TREAT, regardless of severity. Asphyxiation risk — US HAEA
and WAO/EAACI guidelines mandate on-demand C1-INH for every laryngeal attack at first sign of involvement.
Do NOT wait for stridor or oxygen desaturation. Document attack site (laryngeal) on the claim narrative.
On-demand only — NOT prophylaxis. Same molecule as Cinryze (J0598) but different
FDA-labeled indication and different J-code. Prophy = Cinryze (J0598 IV) / Haegarda (J0599 SC) /
Takhzyro (J0593 SC). Billing J0597 for routine prophylaxis = automatic denial.
Codes & vial
| HCPCS | J0597 — "Inj, C-1 esterase inhibitor (human), Berinert, 10 units" (permanent) |
|---|
| NDC (11-digit) | 00053-7708-01 — CSL Behring labeler 0053; verify per shipment |
|---|
| Vial | 500 IU single-dose lyophilized; 10 mL sterile water diluent → 50 IU/mL |
|---|
| Storage | 2–8°C; or up to 25°C for ≤6 months prior to expiry |
|---|
| Use post-recon | Within 8 hours; do NOT refrigerate after reconstitution |
|---|
| Benefit | Medical OR pharmacy benefit per plan; specialty pharmacy commonly dispenses for home use |
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Dosing — 20 IU/kg by weight
| Weight | Total IU | J0597 units | 500 IU vials | Waste |
|---|
| 25 kg | 500 IU | 50 | 1 | 0 (none) |
| 40 kg | 800 IU | 80 | 2 | 200 IU = 20 (JW) |
| 50 kg | 1,000 IU | 100 | 2 | 0 (none) |
| 70 kg | 1,400 IU | 140 | 3 | 100 IU = 10 (JW) |
| 85 kg | 1,700 IU | 170 | 4 | 300 IU = 30 (JW) |
| 100 kg | 2,000 IU | 200 | 4 | 0 (none) |
| 120 kg | 2,400 IU | 240 | 5 | 100 IU = 10 (JW) |
Vial waste is the rule, not exception. Bill JZ on the administered units; JW on a separate line for wasted units (in 10 IU billing units).
Administration & modifiers
| Code | When |
|---|
96365 | Therapeutic IV (~5-min push fits "up to 1 hour") — non-chemo, clinic only |
96374 | IV push <15 min — verify MAC; 96365 generally preferred |
96413/96415 | NOT appropriate — Berinert is non-chemo |
JZ | Single-dose vial, no waste (required since 7/1/2023) |
JW | Partial-vial waste — common with weight-based 20 IU/kg dosing |
| Self-admin at home: specialty pharmacy bills dispense; provider does NOT bill J0597 + 96365. |
HAE class — on-demand vs prophylaxis
| Drug | HCPCS | Mech | Route | Use |
|---|
| Berinert | J0597 | pdC1-INH | IV push | On-demand 20 IU/kg |
| Ruconest | J0596 | recC1-INH | IV push | On-demand 50 IU/kg |
| Firazyr/Sajazir | J1744 | B2 antag | SC | On-demand 30 mg |
| Kalbitor | J1290 | kallikrein | SC | On-demand 30 mg |
| Cinryze | J0598 | pdC1-INH (same mol) | IV | Prophy 1,000 IU q3-4d |
| Haegarda | J0599 | pdC1-INH | SC | Prophy 60 IU/kg 2×/wk |
| Takhzyro | J0593 | anti-kallikrein mAb | SC | Prophy 300 mg q2-4wk |
| Orladeyo | NDC (PO) | kallikrein | oral | Prophy 150 mg PO daily |
ICD-10 — HAE acute attack
| Code | For |
|---|
D84.1 | Defects in complement system — primary HAE Type I/II Dx |
T78.3XXA | Angioneurotic edema, initial encounter — the active attack being treated |
T78.3XXD | Angioneurotic edema, subsequent encounter (repeat dose) |
D84.81 | Other complement deficiency — verify payer |
D84.9 | AVOID — too non-specific for PA approval |
Standard combo: D84.1 + T78.3XXA + attack site narrative (abdominal/facial/laryngeal).
Laryngeal attack callout
ALWAYS TREAT every laryngeal attack regardless of severity. Pre-on-demand-era case-fatality 15–40% from asphyxiation.
- Triggers to treat: voice change, throat tightness, dysphagia, anterior neck swelling
- Disposition: Even with home self-admin — instruct patient to proceed to ED or 911 simultaneously
- Document: "laryngeal" attack site, urgency rationale, citing US HAEA/WAO/EAACI
- Payer: UHC HAE LCD, Aetna CPB 0758 recognize laryngeal as automatic medical necessity
Payer requirements (May 2026)
| Payer | PA | Key criteria |
|---|
| UnitedHealthcare | Yes | HAE Type I/II confirmation (C4 + C1-INH antigenic/functional); on-demand-only enforcement; laryngeal = auto-MN |
| Aetna | Yes | CPB 0758 — HAE Type I/II; on-demand split from prophy |
| Cigna | Yes | Coverage Policy 0500 — quantity limits per attack/month; QL exception for high frequency |
| BCBS | Yes | Plan-specific; align with FDA label |
| Medicare (MAC LCD) | Generally not | Coverage per FDA label + ICD-10 D84.1+T78.3xxA; MA plans often apply commercial-style PA |
Medicare reimbursement (Q2 2026)
| Field | Value |
|---|
| ASP + 6% | $76.051 / 10 IU unit (eff. 4/1 – 6/30/2026) |
| Per IU | ~$7.61 |
| 25 kg pediatric (50 units) | ~$3,802.55 |
| 70 kg adult (140 units) | ~$10,647.14 |
| 100 kg adult (200 units) | ~$15,210.20 |
| Annual @ 10 attacks/yr (70 kg) | ~$106,471 drug only |
Site of care & POS
| Setting | POS | Notes |
|---|
| Patient home (self-admin) | 12 | Dominant — specialty pharmacy dispenses; provider does NOT bill admin |
| Allergy/immuno office | 11 | Common during diagnosis, training, or in-clinic attack |
| AIC | 49 | Less common (push is brief) |
| ED | 23 | Severe attacks, especially laryngeal, when no home product |
Patient assistance — CSL Behring
- Berinert direct line: 1-855-999-3273
- CSL Behring AssureSupport: 1-877-355-IGIV (1-877-355-4448) — benefits invest., PA, appeals
- Berinert Co-pay Program: commercial $0 first dose + ongoing copay support (excludes Medicare/federal)
- PAP: free product for uninsured/underinsured
- Foundations (Medicare): US HAEA, PAN, HealthWell, PAF — verify open HAE funds quarterly
- US HAEA advocacy: 1-866-798-5598
Pending SME review. Page is staff-authored from FDA label, CMS, CSL Behring AssureSupport, and payer policy documents. Verify each claim for high-stakes use; final SME review by the CareCost editorial team in progress.