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Ruconest c1 esterase inhibitor recombinant 2100 U Injection, Powder, For Solution, 1 vial — NDC 71274-0350-02 package photo
Label image from the product's FDA listing (DailyMed) — may show a different pack size or an older label revision.

Ruconest c1 esterase inhibitor recombinant 2100 U Injection, Powder, For Solution, 1 vial — NDC 71274-350-02 (Billing 71274-0350-02)

by Pharming Healthcare Inc. · 1 VIAL, SINGLE-USE in 1 CARTON / 1 INJECTION, POWDER, FOR SOLUTION in 1 VIAL, SINGLE-USE

This is a package of 1 vial of Ruconest c1 esterase inhibitor recombinant 2100 U Injection, Powder, For Solution from Pharming Healthcare Inc., marketed since Apr 2018 and currently FDA-listed. It is this product's only package size.

NDC 71274-0350-02
🏷️ FDA NDC (as labeled) 71274-350-02 billing pads the product segment with a zero
Rx only Brand On market Non-controlled ⇄ Compare with another NDC
🗂️ FDA directory synced Oct 8, 2026 · this listing last changed Jul 24, 2026 · sources: openFDA · FDA label (DailyMed) · FDA Orange & Purple Book · First Databank · CMS NADAC, ASP, Medicare & Medicaid · RxNorm
📋 All sources & update times →

This page is curated, coded, and maintained by

Compiled from public FDA, CMS, DailyMed, NADAC, and related drug data sources. Editorial policy

NDC database record

One package, one record: these facts belong to NDC 71274-350-02 alone.

Record
FDA NDC Directory package listing · Human prescription drug
Code segments
71274 labeler · 350 product · 02 package
Package marketed since
Apr 1, 2018
Sample package
No — commercial package
Listing certified through
Dec 31, 2026
Billing quantity
1 EA per package
Barcode (UPC-A, from the NDC)
3 7127435002 4
Medicaid fills, this package
370 prescriptions in the last four reported quarters
FDA record last changed
Jul 24, 2026

Identity & classification

Regulatory identifiers FDA, NLM and CMS codes for this package

FDA NDC (as labeled) 71274-350-02
Product NDC 71274-350
11-digit billing NDC 71274035002
NCPDP billing unit EA — each (per item)
RxCUI 1599836, 1599841
UNII 5QS67N4551
Application # BLA125495
SPL Set ID 66d3fecb-32a4-7781-e053-2a91aa0a83ca
DEA schedule Non-controlled
Marketing category BLA
Marketing status On market
FDA listing status Listed (active directory)
Marketing start 2018-04-01
Route INTRAVENOUS
Dosage form INJECTION, POWDER, FOR SOLUTION
Substance CONESTAT ALFA
Biologic (Purple Book) 351(a)

Drug-database identifiers Medi-Span GPI and First Databank GCN / HICL / AHFS classification

GPI-14 85802022102130
GPI class Ruconest
GCN Seq No 067598
GCN 30182
HICL code 037766
Ingredient (HICL) C1 Esterase Inhibitor, Recomb
HIC1 code M
Therapeutic class — broad (HIC1) Blood
HIC2 code M0
Therapeutic class — intermediate (HIC2) Blood And Blood Replacement Preparations
HIC3 code M0N
Therapeutic class — specific (HIC3) C1 Esterase Inhibitors
AHFS code 92:32.00.00
AHFS class Complement Inhibitors (92:32)
FDB label name RUCONEST 2,100 UNIT VIAL
FDB brand name Ruconest
Legend status F — Federal legend — prescription drug or device
Quick answers
  • GSN (GCN sequence number): 067598
  • GCN: 30182
  • GPI-14 (Medi-Span): 85802022102130
  • HICL (First Databank): 037766
  • AHFS class code: 92:32.00.00
  • RxCUI (RxNorm): 1599836
Why two NDCs? The FDA registers this code as 71274-350-02 — a 5-3-2 layout, and that's what's printed on the package and shown on DailyMed. For insurance claims, every NDC is standardized to a uniform 11-digit 5-4-2 format by adding a zero to the product segment → 71274-0350-02. Same drug, same package — only the format differs.
Where does this data come from?
Identifiers from the FDA openFDA NDC Directory and Structured Product Labeling; RxCUI from RxNorm (NLM); GPI from Medi-Span; GCN / HIC / AHFS / legend from First Databank.

RxNorm drug class

This medicine belongs to the Human C1 Esterase Inhibitor class.

Pharmacologic class Human C1 Esterase Inhibitor
Drug family (ATC) Drugs used in hereditary angioedema
How it works Kallikrein Inhibitors
Where does this data come from?
Therapeutic classes from RxNorm RxClass (U.S. National Library of Medicine) — Established Pharmacologic Class (FDA), ATC drug family (WHO) and mechanism of action, matched by this product’s RxCUI.

Clinical

Label name RUCONEST 2,100 UNIT VIAL Ingredient C1 Esterase Inhibitor, Recomb
📗 Our plain-language guide HelloPharmacist
  • Ruconest is a lab-made version of a protein your body normally makes called C1-esterase inhibitor. If you have hereditary angioedema (HAE), your body doesn't produce enough of this...
  • What exactly is Ruconest and why has it been prescribed to me?
  • Yes, Ruconest is given as a slow injection directly into a vein (intravenously), over about 5 minutes. If your healthcare team has trained you properly, you can give it to yourself...
  • How do I actually take it — is it a shot I give myself?
📖 Read our full C1-Esterase Inhibitor (Recombinant) Injection guide →

Supplement & herbal interactions

🌿 Drug–supplement interaction checker →
Where does this data come from?
Plain-language summary from MedlinePlus (U.S. National Library of Medicine); supplement & herbal interactions and nutrient depletion data from the Natural Medicines database; our full guide is HelloPharmacist editorial content.

Pricing

A drug doesn't have one price. Each row is a different public payment system, and none is what you'd pay at the counter — that depends on your insurance. The ⓘ on each row explains what it measures.

Price systemPer mLPer package
Retail pharmacies payNADAC · weekly Not in the retail survey — common for institutional, discontinued, or low-volume packs.
Medicaid paysCMS SDUD · 12 mo $8,036.25 —
Medicare drug plans payPart D · Q2 2026 $8,585.98 —
Medicare Part B allowsASP · J0596 $37.832 / J0596 unit —
Where does this data come from?
NADAC (National Average Drug Acquisition Cost) is the CMS weekly pharmacy-acquisition-cost survey — what pharmacies pay. ASP (Average Sales Price) is the CMS Medicare Part B drug-payment file, published quarterly. Medicaid pays is computed by us from CMS State Drug Utilization Data (total reimbursed ÷ units, trailing 12 months) — gross of rebates and inclusive of dispensing fees, so it reflects what Medicaid paid, not an acquisition cost. Medicare drug plans pay is the median negotiated point-of-sale unit cost across plans listing this NDC in the CMS quarterly Prescription Drug Plan pricing files, before rebates. The VA pays is the federal contract price (FSS, and the statutory Big 4 ceiling where listed) from the VA National Acquisition Center pharmaceutical price file. All are free public government data; each measures a different payer, so the figures are not directly comparable.

Billing & reimbursement

FDA NDC (as labeled)71274-350-02
11-digit billing NDC71274-0350-02
Format5-3-2 as registered → padded to 5-4-2 for billing (zero added to the product segment)
HCPCS J-codeJ0596
DescriptorINJECTION, C1 ESTERASE INHIBITOR (RECOMBINANT), RUCONEST, 10 UNITS
Billing units / pkg210 units
How the units are derivedThis package is 1 EA; the HCPCS unit is 10 U, so one package = 210 billing units.
Medicare Part B spend (2026 (Q1))$15,171 · 38 claims · $399.24 per claim (all NDCs under J0596)
Crosswalk sourcePDAC NDC-HCPCS crosswalk (DME MAC / DMEPOS)
Where does this data come from?
The HCPCS J-code crosswalk comes from the CMS ASP NDC-HCPCS crosswalk and the DMEPDAC (DME MAC) NDC-HCPCS crosswalk — free public CMS data. Billing units are derived from the code’s descriptor and the package amount.

Packaging — all sizes for this product

Package NDCDescription Marketing startMarketing endStatus
71274-0350-02 You're viewing this Main listing 1 VIAL, SINGLE-USE in 1 CARTON / 1 INJECTION, POWDER, FOR SOLUTION in 1 VIAL, SINGLE-USE 2018-04-01 — Active

Therapeutic equivalents

ProductLabelerPackNADAC/unitTEStatusPrice vs. this
Ruconest 2100 Uthis 71274-0350-02 Pharming 1 vial — — FDA listed —
About this product: this is a biologic. Biologics don't have small-molecule generics — competition comes from FDA-licensed biosimilars (shown above), not generics.
Where does this data come from?
Equivalents are other NDCs of the same ingredient, form and route from the openFDA NDC Directory, ranked least-expensive-first by NADAC. Therapeutic-equivalence (AB) ratings come from the FDA Orange Book; biologics use the FDA Purple Book for biosimilar & interchangeable status.

Availability & biosimilar status

🏛️
2014
First FDA approval
Jul 2014
📍
2026
Currently FDA-listed
12 years listed
🔓
·
Biosimilar pathway open
listed protections lapsed 2026
🧬Biologic — competition comes from biosimilars

Biologics have no small-molecule generics; biosimilar competition is tracked in the FDA Purple Book.

🛡️ Latest patent/protection date listed: The latest listed FDA patent/protection lapsed Jul 2026 — those protections no longer apply, though a biosimilar still needs FDA licensure and a manufacturer to market it.
📅 FDA approved Jul 16, 2014

Why the date isn’t exact: Biosimilar timing can change because patents may be challenged, settled, licensed, added or removed, and litigation can move the real date earlier or later.

Patents & exclusivity — FDA Purple Book
Exclusivity RefProduct
2014 2016 2018 2020 2022 2024 2026
Today
LOE
Biologic patent Exclusivity
🏛️Reference-product exclusivity
A flat 12 years of FDA market protection from first licensure. No biosimilar can be licensed before it ends — regardless of patents.
🧪Listed biologic patents
Patents the reference maker lists covering the molecule, formulation, or manufacturing. A biosimilar generally can’t launch until these resolve.
🔁Interchangeability
An interchangeable biosimilar may be substituted at the pharmacy (state laws vary). The first one can earn its own exclusivity period.
🛈 What do these terms mean?
Biologic patent
A patent the reference product’s maker has publicly listed. A biosimilar generally can’t launch until these expire — unless they’re invalidated or resolved in a settlement.
Reference-product exclusivity
A flat 12 years of FDA market protection from the biologic’s first licensure (the BPCIA). No biosimilar can be licensed before it ends, regardless of patents.
Interchangeable exclusivity
The first interchangeable biosimilar can earn a period as the only interchangeable version (pharmacists can substitute it without the prescriber).
Earliest biosimilar (LOE)
The latest of all the dates above — the soonest a biosimilar can realistically reach the market. Litigation and settlements can move it earlier.

Biologics have no small-molecule “generics” — competition comes from FDA-licensed biosimilars, tracked in the FDA Purple Book.

FDA exclusivity
CodeWhat it grantsExpires
RefProductReference-product exclusivity (12-year, BPCIA) — no biosimilar can be licensed before this dateJul 16, 2026
Common questions
Is there a biosimilar for RUCONEST 2,100 UNIT VIAL?
No FDA-licensed biosimilar is currently listed for this biologic in the FDA Purple Book.
Why do different websites show different biosimilar dates?
Biosimilar availability isn’t based on one single date. Some sources use the reference-product exclusivity, some use the last listed patent, and patent litigation, settlements, and licenses can all change the real-world launch date. This page shows the underlying Purple Book dates so you can see why estimates differ.
Can a biosimilar launch before the last patent expires?
Sometimes. A biosimilar maker may settle with the reference manufacturer or receive a license to launch earlier. In other cases, the last listed protection delays competition.
What does “current Purple Book estimate” mean?
It means we’re using the latest patent and exclusivity dates currently listed in the FDA Purple Book. It is not a guaranteed launch date.
What does “FDA listed” mean?
It means the product appears in the FDA’s official directory. That’s a good sign a product exists for the U.S. market, but on its own it does not confirm a pharmacy can get it today. Where we have recent retail pricing data, we label it “Availability likely” instead.
What does a patent or protection date mean here?
It’s the latest date currently listed in the FDA Purple Book for a patent or exclusivity on the reference biologic. It can affect when a biosimilar becomes widely available — but it is not a guaranteed launch date. Settlements and licenses can move the real date earlier or later.
Built from the FDA Purple Book Patent List (patents the reference-product sponsor has publicly listed under the BPCIA) plus reference-product exclusivity. Biosimilars cannot launch until these clear; patent litigation and settlements can shift the real date. Biologics have no small-molecule generics — competition comes from FDA-licensed biosimilars, not the Orange Book.
Where does this data come from?
Patents and exclusivity from the FDA Purple Book (biologics), refreshed from public FDA data. Biosimilar launch timing is an estimate, not a guarantee.

What it looks like

Color white
One label can cover several strengths, so colors may be combined — always confirm a loose pill against the dispensed prescription label or a pharmacist.
Where does this data come from?
Physical description (imprint, shape, color, scoring, coating) from this product’s FDA Structured Product Labeling (SPL), mirrored from DailyMed / openFDA.

Inactive Ingredients / Excipients

Inactive ingredients, also called excipients, are components of the drug product other than the active ingredient. They may include fillers, dyes, coatings, preservatives, flavors, or other formulation ingredients.

🧪 Avoiding an ingredient? See C1-Esterase Inhibitor (Recombinant) Injection inactive ingredients by manufacturer: every current product's list side by side, so you can ask your pharmacy for the version that does not list it.

💡 Tap an ingredient (hover on desktop) to see what it is and why it’s used.

  • UNII 2968PHW8QP
    A weak organic acid derived from citrus fruits or made through fermentation. It works as a buffer to control pH, a preservative to extend shelf life, and a flavoring agent in medications.
  • UNII 1Q73Q2JULR
    Sodium citrate is a salt derived from citric acid. It works as a buffer to maintain the medicine's pH level and may also help improve taste or act as a preservative in the formulation.
  • UNII C151H8M554
    A natural sugar derived from sugar cane or sugar beets. It's used as a sweetener, filler, and binder to improve taste, add bulk, and help hold tablet or capsule ingredients together.

3 inactive ingredients listed in the exact product block matched to this NDC.

Where does this data come from?
Data sourced from official FDA Structured Product Labeling (SPL) via DailyMed — ingredient classCode="IACT" elements from the exact product block matched by this NDC. Label-section narrative from DailyMed / the openFDA label index is shown separately when available.

Inactive ingredient FAQ

Are inactive ingredients the same for every manufacturer?
No. Inactive ingredients can differ by manufacturer, dosage form, strength, and package / product version.
Why might an inactive ingredient be missing?
Some SPLs do not provide a complete structured inactive-ingredient list, and older or unusual labels may only include the information in narrative text.
Can inactive ingredients matter?
Yes. They can matter for allergies, intolerances, dyes, gluten / lactose concerns, preservatives, and formulation differences — but confirm with a pharmacist or the manufacturer when it’s clinically important.

Manufacturer & labeler

LabelerPharming Healthcare Inc.
FDA applicationBLA125495 (BLA)
Labeler code71274
First marketedApr 2018
Product typeHuman Prescription Drug
Portfolio2 products on file

More NDCs from Pharming Healthcare Inc. labeler code 71274

The labeler markets the product; the application holder owns the FDA approval. They’re often the same company but can differ (e.g. a repackager or an authorized generic). A mailing address / phone appears here when the manufacturer includes it in the product’s FDA label (not all do).
Where does this data come from?
Labeler, application holder and registered establishment from the FDA openFDA NDC Directory and Drugs@FDA; address/contact from the product’s FDA label.

Full prescribing information FDA SPL

The complete FDA label for this product — the official prescribing information, verbatim, section by section. Very long sections are excerpted here and marked; the full text is on DailyMed (linked in the sources below). Jump with a chip, search within the label, or expand everything.
🎯 Indications and Usage ⏱️ Dosage and Administration 💊 Dosage Forms and Strengths ⛔ Contraindications ⚠️ Warnings and Cautions 🤒 Adverse Reactions 👥 Use in Specific Populations 🤰 Pregnancy 🧒 Pediatric Use 🧓 Geriatric Use 🧬 Clinical Pharmacology 📦 How Supplied / Storage and Handling 📋 Description 💬 Information for Patients 🔬 Clinical Studies 🧪 Nonclinical Toxicology 📄 Carcinogenesis, Mutagenesis, Impairment of Fertility 📚 References 📄 Patient Package Insert 📄 Package Label / Principal Display Panel
🎯 Indications and Usage 79 words ▾

1 INDICATIONS AND USAGE RUCONEST is a C1 esterase inhibitor [recombinant] indicated for the treatment of acute attacks in adult and adolescent patients with hereditary angioedema (HAE). Limitation of Use: Effectiveness was not established in HAE patients with laryngeal attacks. RUCONEST is a C1 esterase inhibitor [recombinant] indicated for the treatment of acute attacks in adult and adolescent patients with hereditary angioedema (HAE).

( 1 ) Limitation of Use: Effectiveness was not established in HAE patients with laryngeal attacks.

⏱️ Dosage and Administration ~3 min read ▾

2 DOSAGE AND ADMINISTRATION For intravenous use after reconstitution only. For Intravenous Use Only. Reconstitute each vial (2100 U) by adding 14 mL sterile Water for Injection per vial to obtain a solution of 150 U per mL.

( 2.3 ) Administer the reconstituted solution at room temperature as a slow intravenous injection over approximately 5 minutes. ( 2.4 ) Appropriately trained patients may self-administer upon recognition of an HAE attack. ( 2.4 ) Recommended dose of RUCONEST for an acute attack Body weight RUCONEST Dose for Intravenous Injection Volume (mL) of Reconstituted Solution (150 U/mL) to be Administered < 84 kg 50 U per kg Body weight in kg divided by 3 ≥ 84 kg 4200 U (2 vials) 28 mL If the attack symptoms persist, an additional (second) dose can be administered at the recommended dose level.

Do not exceed 4200 U per dose. No more than two doses should be administered within a 24-hour period.

2.1Recommended Dosing Training on how to administer RUCONEST should be conducted by a qualified healthcare professional experienced in the treatment of HAE. Appropriately trained patients may self-administer upon recognition of an HAE attack. The recommended dose of RUCONEST is 50 U per kg with a maximum of 4200 U to be administered as a slow intravenous injection over approximately 5 minutes.

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

Table 1. Recommended dose of RUCONEST for an acute attack Body Weight RUCONEST Dose for Intravenous Injection Volume (mL) of Reconstituted Solution (150 U/mL) to be Administered < 84 kg 50 U per kg Body weight in kg divided by 3 ≥ 84 kg 4200 U (2 vials) 28 mL

2.2Preparation and Handling Store RUCONEST in the original carton and protect from light prior to reconstitution. Do not use after expiration date on the product vial label. Water for Injection is not included in the RUCONEST package.

Use aseptic technique to reconstitute, mix the solution, and to combine the reconstituted solution from more than one vial ( see Reconstitution [ 2.3 ] and Administration [ 2.4 ]). Do not mix or administer RUCONEST with other medicinal products or solutions. Discard all partially used vials after treatment.

2.3Reconstitution Each package contains one single-use vial of RUCONEST. To reconstitute, the following are also required: Sterile Water for Injection (diluent) - At least 14 mL per vial of RUCONEST requiring reconstitution. Antiseptic wipe Syringe Commercially available vial adapter and syringe luer lock or large bore needle.

If using a syringe with vial adapter, use a new vial adapter for each vial of RUCONEST and diluent. The procedures below are provided as general guidelines for the reconstitution and administration of RUCONEST. Ensure that the RUCONEST vial and diluent vial are at room temperature.

Remove the flip caps from the RUCONEST and diluent vials. Treat the vial stoppers with the antiseptic wipe and allow to dry. Using the syringe/needle or syringe/vial adapter, withdraw 14 mL of sterile water for injection from the diluent vial.

Remove the syringe and transfer the diluent to the RUCONEST vial. Add the diluent slowly to avoid forceful impact on the powder. Swirl the vial slowly to mix and avoid foaming.

Repeat this procedure using another 14 mL of diluent and a second vial of RUCONEST. If the same patient is to receive more than one vial, the contents of multiple vials may be pooled into a single administration device (i.e., syringe). Inspect RUCONEST visually for particulate matter and discoloration after reconstitution and prior to administration.

The reconstituted solution should be colorless, clear, and free from visible particles. Do not use if the solution is cloudy, colored, or contains particulates. RUCONEST vial is for single-use only.

Use the reconstituted product immediately, or within 8 hours stored at 36ºF - 46… [Excerpted — this section continues on DailyMed.]

💊 Dosage Forms and Strengths 45 words ▾

3 DOSAGE FORMS AND STRENGTHS RUCONEST is available as a lyophilized powder for reconstitution for injection in a single-use 25 mL glass vial. Each vial contains 2100 U of rhC1INH. 2100 U Lyophilized powder for reconstitution for injection in a single-use vial. ( 3 )

⛔ Contraindications 64 words ▾

4 CONTRAINDICATIONS RUCONEST is contraindicated in patients with a history of allergy to rabbits or rabbit-derived products. RUCONEST is contraindicated in patients with a history of life-threatening immediate hypersensitivity reactions to C1 esterase inhibitor preparations, including anaphylaxis. Known or suspected allergy to rabbits and rabbit-derived products.

( 4 ) History of immediate hypersensitivity reactions, including anaphylaxis, to C1 esterase inhibitor preparations. ( 4 )

⚠️ Warnings and Cautions 202 words ▾

5 WARNINGS AND PRECAUTIONS Hypersensitivity reactions, including anaphylaxis may occur. Should symptoms occur, discontinue RUCONEST and administer appropriate treatment. ( 5.1 ) Serious arterial and venous thromboembolic (TE) events have been reported at the recommended dose of plasma derived C1 esterase inhibitor products in patients with risk factors. Monitor patients with known risk factors for TE events during and after RUCONEST administration. ( 5.2 )

5.1Hypersensitivity Severe hypersensitivity reactions may occur . The signs and symptoms of hypersensitivity reactions may include hives, generalized urticaria, tightness of the chest, wheezing, hypotension, and/or anaphylaxis during or after injection of RUCONEST. Should symptoms occur, discontinue RUCONEST and institute appropriate treatment.

Because hypersensitivity reactions may have symptoms similar to HAE attacks, treatment methods should be carefully considered. [see Patient Counseling Information (17) ]

5.2Thromboembolic Events Serious arterial and venous thromboembolic (TE) events have been reported at the recommended dose of plasma derived C1 esterase inhibitor products in patients with risk factors. Risk factors may include the presence of an indwelling venous catheter/access device, prior history of thrombosis, underlying atherosclerosis, use of oral contraceptives or certain androgens, morbid obesity, and immobility. Monitor patients with known risk factors for TE events during and after RUCONEST administration.

🤒 Adverse Reactions ~3 min read ▾

6 ADVERSE REACTIONS The serious adverse reaction in clinical studies of RUCONEST was anaphylaxis. The most common adverse reactions (≥ 2%) reported in all clinical trials were headache, nausea, and diarrhea. The serious adverse reaction reported in clinical trials was anaphylactic reaction.

( 6 ) The common adverse reactions (≥ 2%) reported in clinical trials were headache, nausea, and diarrhea. ( 6 ) To report SUSPECTED ADVERSE REACTIONS, contact Pharming Healthcare Inc. at 1-800-930-5221 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.

6.1Clinical Trials Experience Because clinical trials are conducted under widely varying conditions, the adverse reaction rates observed in the clinical trials of a drug cannot be directly compared to rates in the clinical trials of another drug and may not reflect the rates observed in clinical practice. The RUCONEST clinical development program evaluated a combined total of 940 administrations in 236 subjects (symptomatic and non-symptomatic). In clinical studies, a total of 205 symptomatic HAE patients received treatment with RUCONEST for a combined total of 650 acute angioedema attacks.

Among these HAE patients, 83 were treated for a single HAE attack and 122 were treated for multiple attacks. Three randomized, placebo-controlled clinical trials (RCTs) were conducted in which 137 patients experiencing acute HAE attacks received RUCONEST (either an initial 50 U/kg or 100 U/kg body weight dose) or placebo (saline solution). Table 2 shows all adverse reactions (ARs) in the RCTs, compared with the placebo group.

Table 2. Adverse reactions occurring In ≥ 2% of subjects in the three RCT studies MedDRA Preferred Term RUCONEST 100 U/kg (N=29) n (%) RUCONEST 50 U/kg* (N=66) n (%) Placebo** (N=47) n (%) Total number of patients with adverse reactions 4 (14%) 6 (9%) 13 (28%) Headache 3 (10%) 0 2 (4%) Sneezing 0 1 (2%) 0 Angioedema 1 (3%) 0 0 Erythema marginatum 0 1 (2%) 0 Skin burning sensation 0 1 (2%) 0 Back pain 0 2 (3%) 0 C-reactive protein increased 0 1 (2%) 0 Fibrin D-dimer increased 0 1 (2%) 0 Vertigo 1 (3%) 0 0 Procedural headache 0 1 (2%) 0 Lipoma 0 1 (2%) 0 * Includes 5 patients who received an additional 50 U/kg dose MedDRA: Medical Dictionary for Regulatory Activities, version 15.0. ** Events only occurring in placebo patients are not listed.

Integrated RCT and Open-Label Extension (OLE) Studies In a total of seven RCT and OLE studies, 205 patients experiencing acute HAE attacks were treated with RUCONEST for a total of 650 HAE attacks. Included in this population were 124 patients who were treated at the 50 U/kg dosage strength for one or more attacks. Table 3 shows adverse reactions in ≥ 2% of patients in any RUCONEST group for the integrated dataset combining all seven RCT and OLE studies in patients experiencing acute HAE attacks.

Table 3. Adverse reactions in the seven RCT and OLE studies occurring ≥ 2% of RUCONEST-treated patients (all doses), irrespective of causality MedDRA Preferred Term All RUCONEST doses* N=205 n (%) Headache 19 (9%) Nausea 5 (2%) Diarrhea 5 (2%) * RUCONEST doses: doses up to 100 U/kg MedDRA: Medical Dictionary for Regulatory Activities, version 15.0. Immunogenicity As with all therapeutic proteins, there is potential for immunogenicity.

Pre- and post-exposure samples from 205 HAE patients treated for 650 acute attacks with RUCONEST were tested for the antibodies against plasma-derived C1INH or rhC1INH and for antibodies against host-related impurities (HRI). Testing was performed prior to and after treatment of a first attack and subsequent repeated attacks at 7, 22 or 28, and 90 days after RUCONEST treatment. Prior to the first exposure to RUCONEST, the frequency of anti-C1INH antibodies varied from 1.2% to 1.6% of samples.

After the first exposure, the frequency of anti-C1INH antibodies varied from 0.6% to 1.0% of samples tested. After repeated exposures, the frequency of anti-C1INH antibodies varied from 0.5 to 2.2% of samples tested. The frequency of ant… [Excerpted — this section continues on DailyMed.]

👥 Use in Specific Populations ~2 min read ▾

8 USE IN SPECIFIC POPULATIONS

8.1Pregnancy Risk Summary There are no adequate and well-controlled studies of RUCONEST in pregnant women. In the U.S. general population, regardless of drug exposure, available data suggest that major birth defects occur in 2 to 4% of the general population and miscarriage occurs in 15 to 20% of clinically recognized pregnancies. Data Human Data Limited available postmarketing safety data with RUCONEST use in pregnant women do not indicate any adverse effects in this population.

In a retrospective case collection study, 14 pregnant women aged 17 to 37 years with HAE received RUCONEST at 50 U/kg (max 4200 U) via intravenous administration for treatment of acute atttacks or for prophylaxis during pregnancy. For all 14 women in this study, delivery at full term with the births of healthy babies occurred without complications. No adverse events related to RUCONEST treatment were reported during the pregnancy period.

1,2 However these data cannot definitely establish the absence of any risk because of the small sample size and non-randomized study design. Animal Data Embryo toxicity with 625 U/kg of RUCONEST (12.5 times the human dose) was tested after intravenous dosing of pregnant rats in a segment II (embryofetal toxicity) study design. There were no significant outcomes from this study.

Dosing of pregnant rabbits with 625 U/kg during organogenesis showed no drug-related adverse effects on either the developing fetuses or mothers [see Animal Toxicology and/or Pharmacology (13.2)].

8.2Lactation Risk Summary There are no data on the presence of RUCONEST in human milk, its effects on the breastfed infant, or its effects on milk production. The developmental and health benefits of breastfeeding should be considered along with the mother's clinical need for RUCONEST and any potential adverse effects on the breastfed child from RUCONEST or from the underlying maternal condition.

8.4Pediatric Use The safety and efficacy of RUCONEST were evaluated in 17 adolescent patients (13-17 years of age) treated for 52 HAE attacks. Eight out of 17 (47%) adolescent patients experienced adverse reactions. No serious adverse reactions were reported in these patients. The most common reactions (occurring in at least 2 patients) were: abdominal pain, headache, and oropharyngeal pain.

8.5Geriatric Use The clinical studies of RUCONEST included seven patients older than 65 years. The clinical studies included an insufficient number of patients in this age group to determine if they respond differently from younger patients.

🤰 Pregnancy ~1 min read ▾

8.1Pregnancy Risk Summary There are no adequate and well-controlled studies of RUCONEST in pregnant women. In the U.S. general population, regardless of drug exposure, available data suggest that major birth defects occur in 2 to 4% of the general population and miscarriage occurs in 15 to 20% of clinically recognized pregnancies. Data Human Data Limited available postmarketing safety data with RUCONEST use in pregnant women do not indicate any adverse effects in this population.

In a retrospective case collection study, 14 pregnant women aged 17 to 37 years with HAE received RUCONEST at 50 U/kg (max 4200 U) via intravenous administration for treatment of acute atttacks or for prophylaxis during pregnancy. For all 14 women in this study, delivery at full term with the births of healthy babies occurred without complications. No adverse events related to RUCONEST treatment were reported during the pregnancy period.

1,2 However these data cannot definitely establish the absence of any risk because of the small sample size and non-randomized study design. Animal Data Embryo toxicity with 625 U/kg of RUCONEST (12.5 times the human dose) was tested after intravenous dosing of pregnant rats in a segment II (embryofetal toxicity) study design. There were no significant outcomes from this study.

Dosing of pregnant rabbits with 625 U/kg during organogenesis showed no drug-related adverse effects on either the developing fetuses or mothers [see Animal Toxicology and/or Pharmacology (13.2)].

🧒 Pediatric Use 60 words ▾

8.4Pediatric Use The safety and efficacy of RUCONEST were evaluated in 17 adolescent patients (13-17 years of age) treated for 52 HAE attacks. Eight out of 17 (47%) adolescent patients experienced adverse reactions. No serious adverse reactions were reported in these patients. The most common reactions (occurring in at least 2 patients) were: abdominal pain, headache, and oropharyngeal pain.

🧓 Geriatric Use 37 words ▾

8.5Geriatric Use The clinical studies of RUCONEST included seven patients older than 65 years. The clinical studies included an insufficient number of patients in this age group to determine if they respond differently from younger patients.

🧬 Clinical Pharmacology ~2 min read ▾

12 CLINICAL PHARMACOLOGY

12.1Mechanism of Action C1 esterase inhibitor (C1INH) is a normal constituent of human blood and is one of the serine protease inhibitors (serpins). The primary function of C1INH is to regulate the activation of the complement and contact system pathways. Regulation of these systems is performed through the formation of complexes between the protease and the inhibitor, resulting in inactivation of both and consumption of the C1INH.

C1INH exerts its inhibitory effect by irreversibly binding several proteases (target proteases) of the contact and complement systems. The effect of RUCONEST on the following target proteases was assessed in vitro : activated C1s, kallikrein, factor XIIa and factor XIa. Inhibition kinetics were found to be comparable with those observed for plasma-derived human C1INH.

HAE patients have low levels of endogenous or functional C1INH. Although the events that induce attacks of angioedema in HAE patients are not well defined, it is thought that contact system activation, and resulting increased vascular permeability lead to the clinical manifestation of HAE attacks. Suppression of contact system activation by C1INH through the inactivation of plasma kallikrein and factor XIIa is thought to modulate vascular permeability by preventing the generation of bradykinin.

3 Administration of RUCONEST increases plasma levels of functional C1INH activity.

12.2Pharmacodynamics The complement component (protein) C4 is a substrate for activated C1. Patients with HAE have low levels of C4 in the circulation; RUCONEST shows a dose-dependent restoration of complement homeostasis of C4 in HAE patients. A dose of 50 U/kg of RUCONEST increases plasma C1INH activity levels to greater than

0.7U/mL (the lower limit of normal) in HAE patients.

12.3Pharmacokinetics The pharmacokinetics of RUCONEST was evaluated in a study of 12 asymptomatic HAE patients (dose ranged from

6.25U/kg to 100 U/kg). Pharmacokinetics evaluation was performed by non-compartmental analysis, using functional C1INH levels. Following administration of RUCONEST (50 U/kg) in asymptomatic HAE patients ( Table 5 ), the mean Cmax was

1.2U/mL, and the elimination half-life was approximately 2.5 hours. The clearance of RUCONEST was nonlinear (clearance decreased with increasing dose) over the dose range of 25-100 U/kg. Table 5. Baseline corrected pharmacokinetic parameters (Mean ± SD) of functional C1INH following administration of 50 U/kg and 100 U/kg RUCONEST to asymptomatic HAE patients Parameters 50 U/kg 100 U/kg C baseline (U/mL) 0.18 ± 0.12 0.14 ±

0.08C max (U/mL) 1.2 ± 0.2 2.3 ±

0.2T max (hours) 0.31 ± 0.10 0.31 ±

0.10AUC (U x hr/mL) 3.3 ± 1.0 10.6 ±

2.5CL (mL/hr) 1207 ± 414 781 ± 147 Half-life (hours) 2.4 ± 0.6 2.7 ±

0.3V ss (L) 3.0 ± 0.9 2.4 ±

0.5Studies have not been conducted to evaluate the PK of RUCONEST in special patient populations, identified by race, age (pediatric or geriatric), or the presence of renal or hepatic impairment.

📦 How Supplied / Storage and Handling 148 words ▾

16 HOW SUPPLIED/STORAGE AND HANDLING

16.1How Supplied RUCONEST is supplied in single-use 25 mL glass vials with a stopper (siliconized chlorobutyl rubber) and a flip-off seal (aluminum and colored plastic). Each carton contains one single-use vial. Each vial contains 2100 U rhC1INH lyophilized powder for reconstitution for injection.

16.2Storage and Handling Store in the original package in order to protect from light. Shelf life: 48 months when stored at 36ºF - 77ºF (2ºC - 25ºC). Do not freeze.

Each vial of RUCONEST should be reconstituted with 14 mL Water for Injection (not supplied). The reconstituted solution contains 150 U/mL rhC1INH and is clear and colorless. Each vial of RUCONEST is for single use only.

RUCONEST contains no preservative. Any product that has been reconstituted should be used immediately, or within 8 hours stored at 36ºF - 46ºF (2ºC - 8ºC). Discard partially used vials after treatment.

📋 Description ~2 min read ▾

11 DESCRIPTION RUCONEST is a recombinant analogue of human complement component 1 esterase inhibitor for intravenous injection. RUCONEST is purified from the milk of transgenic rabbits, and supplied as a sterile, preservative-free, white/off-white lyophilized powder for reconstitution for injection. One U of rhC1INH activity is defined as the equivalent of C1 esterase inhibiting activity present in 1 mL of pooled normal plasma.

RUCONEST is a soluble, single-chain glycoprotein containing 478 amino acids, with a molecular mass of 68 kDa, of which approximately 22% comprises oligosaccharide structures. The primary and secondary structures of the molecule and target protease selectivity are consistent with those of plasma-derived C1 esterase inhibitor. Each vial of RUCONEST contains 2100 U of rhC1INH, 937 mg of sucrose, 83.3 mg of sodium citrate dihydrate and 1.0 mg of citric acid monohydrate.

After reconstitution with 14 mL of sterile Water for Injection, each vial of RUCONEST contains 150 U of rhC1INH per 1 mL in a 20 mM sodium citrate buffer with a pH of 6.8. RUCONEST does not contain preservatives and each vial is for single use only. RUCONEST is purified from the milk of transgenic rabbits.

The rabbits are maintained in a closed colony that is controlled and routinely monitored for specific pathogens. The skimmed milk is screened for adventitious contaminants prior to further manufacture. The manufacturing process has been validated to demonstrate adequate capacity for removal and/or inactivation of viruses ( Table 4 ).

RUCONEST contains less than 0.002% of host-related impurities. Table 4. Viral reduction capacity of the rhC1INH manufacturing process a MLV: Murine leukemia virus; REO-3: Reovirus type 3; ORF: Scab-mouth ORF virus; FCV: Feline calicivirus; PPV: Porcine parvovirus; b SP BB: SP Sepharose BB; SD: Solvent/detergent; Q HP: Q Sepharose HP; Zn FF: Zinc Chelating Sepharose FF; c Not added to the total reduction factor since independence of clearance mechanism has not been experimentally verified; d Not applicable since indicated model virus is not enveloped; e Not tested because SD chemicals are present in the starting material.

Virus Reduction Factor (Log 10 ) Step MLV a REO 3 ORF FCV PPV SP BB chromatography b 1.8 2.2 c 1.5 c 2.3 1.5 b SD incubation b ≥

5.8 NA d

3.7 NA NA Q HP chromatography b NT e

4.8NT 0.8 c

2.2Zn FF chromatography b 1.1 c 3.2 c 3.3 1.9 c 0.4 c Nanofiltration ≥ 5.5 ≥ 6.5 ≥ 5.8 ≥ 6.9

5.8Total reduction factor ≥ 13.1 ≥ 11.3 ≥ 12.8 ≥ 9.2 8.0

💬 Information for Patients 195 words ▾

17 PATIENT COUNSELING INFORMATION Advise the patient to read the FDA-Approved patient labeling (Product Information and Instructions for Use). Patients being treated with RUCONEST should receive the following information and instructions. This information is intended to aid the patient in the safe and effective use of RUCONEST.

Advise female patients to notify their physician if they are pregnant or intend to become pregnant during the treatment of acute attacks of HAE with RUCONEST. Advise patients to notify their physician if they are breastfeeding or plan to breastfeed. Inform patients of the risks and benefits of RUCONEST before prescribing or administering it to the patient.

Advise patients to immediately report : Signs and symptoms of allergic hypersensitivity reactions, such as hives, urticaria, tightness of the chest, wheezing, hypotension and/or anaphylaxis experienced during or after injection of RUCONEST [ see WARNINGS AND PRECAUTIONS/Hypersensitivity ( 5 )]. Manufactured by: Pharming Americas B.V. Darwinweg 24 2333 CR Leiden The Netherlands RUCONEST ® is a registered trademark of Pharming Intellectual Property B.V.

Distributed by: Pharming Healthcare Inc. Warren NJ 07059 Product protected by U.S patent Nos. 7,067,713 and RE43691.

Please see www.ruconest.com for patent information. US License No. 2079 pharming

🔬 Clinical Studies ~3 min read ▾

14 CLINICAL STUDIES The safety and efficacy of RUCONEST for treatment of acute angioedema attacks in patients with HAE was established in Study 1, a double-blind, randomized, placebo-controlled trial (RCT) which included an open-label extension (OLE) phase; and supported by the results of 2 additional RCTs and 2 additional OLE studies. Randomized, Controlled Trials The safety and efficacy of RUCONEST in the treatment of acute attacks in patients with hereditary angioedema were demonstrated in a placebo-controlled, double-blind, randomized study (Study 1).

Supportive evidence of effectiveness is provided by two double-blind, randomized, placebo-controlled studies (Studies 2 and 3). Evidence for the efficacy of repeat treatment of HAE attacks is provided from the open-label extensions (OLE) of each of the three randomized studies. Study 1 was a randomized, double-blind, placebo-controlled trial that included an open-label extension (OLE) phase to assess the efficacy and safety of RUCONEST 50 U/kg in the treatment of acute attacks in patients with HAE.

Seventy-five (75) adults and adolescent patients were randomized (3:2) to receive RUCONEST 50 U/kg (N = 44) or placebo (N = 31). Patients ranged in age from 17 to 69 years of age; 63% were female and 37% were male; 96% were Caucasian. The primary efficacy endpoint was the time to beginning of relief of symptoms, assessed using patient-reported responses to two questions from a Treatment Effect Questionnaire (TEQ).

The TEQ required patients to assess the severity of their attack symptoms at each affected anatomic location, using a seven-point scale (“much worse” to “much better” [TEQ Question 1]), and whether their symptoms had begun to decrease notably since receiving the study medication (“yes” or “no” [TEQ Question 2]). To achieve the primary endpoint, a patient had to have a positive response to both questions along with persistence of improvement at the next assessment time (i.e., the same or better response). Rescue treatment with RUCONEST was available for patients who did not experience the beginning of relief at 4 hours after study drug administration, or earlier to patients who experienced life-threatening oropharyngeal-laryngeal angioedema symptoms.

If a patient received a medication which could have impacted the efficacy evaluation or open-label RUCONEST as rescue medication, prior to achieving beginning of relief of symptoms, the time to beginning of relief of symptoms was censored at the last assessed time prior to medication use. In the RCT phase, the median time to beginning of relief of symptoms was statistically significantly shorter in patients treated with RUCONEST 50 U/kg compared with patients treated with placebo as assessed by the TEQ; ( Table 6 , Figure 1).

Table 6. Time to Beginning of Relief of Symptoms based on questionnaire based on TEQ (Study 1, RCT Phase) Time to Beginning of Relief of Symptoms, minutes RUCONEST 50 U/kg N=44 Placebo N=31 Values that are not estimable are displayed as ‘- ’. Median 90 152 95% CI (61, 150) (93, -) Log-rank p-value 0.031 Figure 1.

Kaplan-Meier Plot of Time to Beginning of Relief of Symptoms (Study 1, RCT Phase) Among several planned subgroup analyses, descriptive statistics showed that in US patients a median time to beginning of relief of symptoms with persistence at the primary attack location (based on TEQ) was 98 minutes [95% CI:(45, 240); n=22] for those receiving RUCONEST and 90 minutes [95% CI: (50, -); n=16] for those receiving placebo. The hazard ratio for time to the beginning of relief of symptoms in this subpopulation was 1.20 [95% CI: (0.48, 3.01)] for patients receiving RUCONEST as compared with patients receiving placebo.

Non-US patients receiving RUCONEST had a median time to beginning of relief of 90 minutes [95% CI: (63, 120); n=22] and non-US patients receiving placebo had a median time to beginning of relief of 334 minutes [95% CI: (150, -); n=15]. The hazard ratio for the non-US subgroup was 4.82 [95%… [Excerpted — this section continues on DailyMed.]

🧪 Nonclinical Toxicology ~1 min read ▾

13 NONCLINICAL TOXICOLOGY

13.1Carcinogenesis, Mutagenesis, Impairment of Fertility No animal studies have been conducted to evaluate the effects of RUCONEST on carcinogenesis and mutagenesis. Fertility was not evaluated in animal studies.

13.2Animal Toxicology and/or Pharmacology Single- and repeat-dose studies of up to 14 days in rats, dogs, and cynomolgus monkeys with daily doses of RUCONEST up to 80 times the human dose (50 U/kg) were conducted. In a 14-day monkey toxicology study of intravenous doses up to 2000 U/kg twice daily, increases in AST and ALP were observed at doses of 500 U/kg and higher. No histopathological correlates were observed in the liver and the increases in liver enzymes were reversible.

Histologic findings included changes in the size of thymic cortex and medulla, and microvacuoles in the epithelial cells lining the renal tubules. The renal tubular vacuolation was dose dependent, but was not accompanied by other histological changes in the kidney. This finding was only partially reversible at the highest dose level of 2000 U/kg twice daily.

It was concluded that the NOAEL of RUCONEST was 1000 U/kg twice daily in this species. There were no adverse findings in a safety pharmacology study in dogs and a local tolerance study in rabbits. It is concluded that preclinical toxicology data for daily doses up to 40 times the proposed human dose of RUCONEST (50 U/kg) do not indicate a safety concern for the use of RUCONEST in humans.

Embryo-fetal studies have been conducted in rabbits and rats at a dose of RUCONEST 12.5 times the human dose of 50 U/kg. In rats, no malformed fetuses were observed. In rabbits, an increase in the incidence of fetal cardiac vessel defects was observed (1.12% [2 cases] in the treatment group compared to 0.03% in historical controls).

These defects are considered to be a chance finding, but a RUCONEST-related effect cannot be excluded.

📄 Carcinogenesis, Mutagenesis, Impairment of Fertility 29 words ▾

13.1Carcinogenesis, Mutagenesis, Impairment of Fertility No animal studies have been conducted to evaluate the effects of RUCONEST on carcinogenesis and mutagenesis. Fertility was not evaluated in animal studies.

📚 References 107 words ▾

15 REFERENCES Moldovan D, Bernstein JA, Hakl R, Porebski G, Poarch K, Lumry WR, Relan A. Safety of recombinant human C1 esterase inhibitor for hereditary angioedema attacks during pregnancy. J Allergy Clin Immunol Pract.

2019 Jun 3. pii: S2213-2198(19)30499-4. Hakl R, Kuklinek P, Krcmova I, Kralickova P, Freiberger T, Janku P, Vlkova M, Litzman J. Treatment of hereditary angioedema attacks with icatibant and recombinant C1 inhibitor during pregnancy.

J Clin Immunol. 2018 Oct;38(7):80-815. Caliezi C, Wuillemin WA, Zeerleder S, Redondo M, Eisele B, Hack CE.

C1-esterase inhibitor: an anti-inflammatory agent and its potential use in the treatment of diseases other than hereditary angioedema. Pharmacol Rev. 2000 Mar;52(1):91-112.

📄 Patient Package Insert ~3 min read ▾

FDA-Approved Patient Labeling - Patient Product Information (PPI) RUCONEST ® (ROO-Ko-nest) ( C1 esterase inhibitor [recombinant]) Lyophilized powder for reconstitution for injection in a single-use vial This leaflet summarizes important information about RUCONEST. Please read it carefully each time before using RUCONEST. There may be new information provided.

This information does not take the place of talking with your healthcare provider, and it does not include all of the important information about RUCONEST. If you have any questions after reading this, ask your healthcare provider. What is RUCONEST?

RUCONEST is an injectable medicine that is used to treat acute angioedema attacks in adult and adolescent patients with Hereditary Angioedema (HAE). HAE is caused by a shortage of a protein called C1 esterase inhibitor, that is present in your blood and helps control inflammation (swelling) and parts of the immune system. A shortage of C1 esterase inhibitor can lead to repeated attacks of swelling, pain in the abdomen, difficulty breathing and other symptoms.

RUCONEST contains C1 esterase inhibitor. Who should not use RUCONEST? You should not use RUCONEST if you have a known or suspected allergy (hypersensitivity) to rabbits or rabbit-derived products.

You should not use RUCONEST if you have experienced life-threatening immediate hypersensitivity reactions, including anaphylaxis, to RUCONEST or to any other C1 esterase inhibitor product. RUCONEST is not indicated for use in children under the age of 13 years. What should I tell my healthcare provider before using RUCONEST?

Tell your healthcare provider about all of your medical conditions, including if you: have an allergy to rabbits since this can put you at high risk of a serious allergic reaction with RUCONEST. These allergy symptoms could include runny nose, itchy nose, sneezing, coughing, wheezing, difficulty breathing or watery eyes when you are near rabbits. are pregnant or planning to become pregnant. It is not known if RUCONEST can harm your unborn baby. are breastfeeding or plan to breastfeed.

It is not known if RUCONEST passes to your milk and if it can harm your baby. Tell your healthcare provider and pharmacist about all of the medicines you take, including all prescription and non-prescription medicines such as over-the-counter medicines, supplements, or herbal remedies. How is RUCONEST given?

RUCONEST will be slowly injected into your vein (intravenous injection). Before injection, the RUCONEST powder must be dissolved using sterile Water for Injection. The dose will be determined based on your weight.

Most of the time a single dose of RUCONEST is enough to treat an attack, but a second dose may be needed. What are the possible side effects of RUCONEST? Like all medicines, RUCONEST can cause side effects, although not everybody gets them.

Allergic reactions may occur with RUCONEST. Call your healthcare provider or the emergency department immediately if you have any of the following symptoms after receiving RUCONEST: wheezing difficulty breathing chest tightness turning blue (look at lips and gums) fast heartbeat swelling faintness rash hives In clinical studies, the most severe side effect reported in a person who received RUCONEST was a severe allergic reaction in a subject who was allergic to rabbits. Other side effects patients experienced during clinical research studies include: headache nausea diarrhea These are not all the possible side effects of RUCONEST.

If any of the side effects get serious, or if you notice any side effects not listed in the leaflet, please inform your healthcare provider or pharmacist. You can also report side effects to the FDA at 1-800-FDA-1088 or www.fda.gov/medwatch or contact Pharming Healthcare Inc. at 1-800-930-5221. You can ask your healthcare provider for information about RUCONEST that is written for healthcare providers.

General Information about RUCONEST Do not use RUCONEST for a condition for which it is not prescribe… [Excerpted — this section continues on DailyMed.]

📄 Package Label / Principal Display Panel 71 words ▾

Package Label/Principal Display Panel - 2100 U Vial Label RUCONEST® C1 esterase inhibitor (recombinant) 2100 U NDC 71274-350-01 For intravenous use only. For single patient use. See prescribing information for directions for use. Rx only Label

Package Label/Principal Display Panel - 2100 U Carton RUCONEST® C1 esterase inhibitor (recombinant) 2100 U NDC 71274-350-02 For intravenous use only. For single patient use. See prescribing information for directions for use. Rx only Carton

Source: FDA Structured Product Labeling, mirrored from DailyMed / openFDA. Prefer the government’s original formatting? View this label on DailyMed ↗

Medicaid utilization & spend

📍 This exact package only: Medicaid data is reported per full 11-digit NDC — labeler, product and pack size — so every number here is for this package alone, not the drug overall. Other pack sizes report separately.
💊 Pharmacy benefit only: These are Medicaid outpatient pharmacy claims, billed by NDC. They exclude the medical benefit — clinic- or hospital-administered drugs billed under HCPCS J-codes — so drugs used mostly that way (e.g. Avastin, Lucentis, Keytruda) can look low or missing here. That’s expected, not an error.
📅 Q1 2025 – Q1 2026 · 5 quarters of data
ⓘ The newest quarter is usually incomplete when first published; states restate recent quarters in later CMS releases, so the latest figures typically revise upward. State coverage-policy changes can also shift quarter-to-quarter totals.
Prescriptions last 4 qtrs
370
Units reimbursed last 4 qtrs
3.9K
Gross reimbursed last 4 qtrs
$31.74M
Avg / prescription
$85,770.66
Avg / unit
$8,035.79
Latest quarter Q1 2026
84Rx
Fee-for-service vs managed care ⓘ
86% FFS 14% MCO
Fee-for-service · 319 Rx Managed care · 51 Rx
State Medicaid map
Alaska: no data reported AK Maine: no data reported ME Washington: no data reported WA Idaho: 168 units · 8.6 per 100k residents ID Montana: no data reported MT North Dakota: no data reported ND Minnesota: no data reported MN Wisconsin: no data reported WI Michigan: no data reported MI New York: 152 units · 0.8 per 100k residents NY Vermont: no data reported VT New Hampshire: no data reported NH Oregon: no data reported OR Nevada: no data reported NV Wyoming: no data reported WY South Dakota: no data reported SD Iowa: no data reported IA Illinois: no data reported IL Indiana: no data reported IN Ohio: no data reported OH Pennsylvania: no data reported PA New Jersey: no data reported NJ Massachusetts: no data reported MA California: 286 units · 0.7 per 100k residents CA Utah: no data reported UT Colorado: 2,822 units · 48.0 per 100k residents CO Nebraska: no data reported NE Missouri: no data reported MO Kentucky: no data reported KY West Virginia: no data reported WV Virginia: no data reported VA Maryland: no data reported MD Connecticut: no data reported CT Rhode Island: no data reported RI Arizona: 276 units · 3.7 per 100k residents AZ New Mexico: no data reported NM Kansas: no data reported KS Arkansas: no data reported AR Tennessee: no data reported TN North Carolina: no data reported NC South Carolina: no data reported SC Delaware: no data reported DE Oklahoma: no data reported OK Louisiana: no data reported LA Mississippi: no data reported MS Alabama: no data reported AL Georgia: no data reported GA D.C.: no data reported DC Hawaii: no data reported HI Texas: no data reported TX Florida: no data reported FL
Units reimbursed · per 100k residents
0.748.0
gray = no data reported ⓘ
Colors are per 100,000 residents, so big states don’t automatically dominate. Tap or hover a state for its actual totals.
Tap or hover a state
…for its Medicaid breakdown
🏆 Top states by units · per 100k residents
1 Colorado 48.0 /100k
2 Idaho 8.6 /100k
3 Arizona 3.7 /100k
4 New York 0.8 /100k
5 California 0.7 /100k
National units — by quarter
💵 About the dollar figures: “reimbursed” is what Medicaid paid pharmacies before confidential manufacturer rebates, so the program’s real net cost is lower than these numbers. Fee-for-service and managed-care claims are combined unless split above. Source: CMS State Drug Utilization Data; per-100k rates use 2023 Census population estimates.

Medicare Part D spend CMS · PART D · 2026 (Q1)

Medicare Part D (outpatient prescription) spending for Ruconest — the program that covers self-administered drugs. 1 manufacturer.
⚠️ Drug-level data: CMS publishes Part D spending by drug, not by NDC — these figures combine every manufacturer, strength and package size sold under the name Ruconest. That’s a different level of aggregation than the Medicaid card above, which is specific to this exact 11-digit NDC (pack size included), so the two aren’t directly comparable.
Period
Total Part D spend
$18.2M
Claims incl. refills
145
Beneficiaries
67
Spend / beneficiary
$271,674.75
Spend / claim
$125,532.47
Trend by period
💵 About the dollar figures: spending is what Part D plans paid before confidential manufacturer rebates, so the program’s real net cost is lower. A blank patient count means fewer than 11 people — CMS hides counts that small to protect privacy. Source: CMS Medicare Quarterly Part D Spending by Drug (data.cms.gov), updated quarterly.

About this NDC listing & data coverage

Finished prescription product
What data is (and isn’t) available for this NDC — tap to expand
NDC identity (package / product / labeler codes) ✓ Available
Labeler ✓ Available
Product & package description ✓ Available
Marketing category & status ✓ Available
Active ingredient / dosage form / route ✓ Available
FDA label (SPL via DailyMed) ✓ Available
Package photos ✓ Available
Inactive ingredients (structured) ✓ Available
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Orange Book / therapeutic-equivalence data ✓ Available
HCPCS J-code billing crosswalk ✓ Available
Medicaid utilization (CMS SDUD) ✓ Available
“Not published” reflects what the public FDA / CMS / NLM sources provide for this exact package code — it is a property of the data feeds, not a judgment about the product.

Questions about this listing

Why is there no price listed?
The pricing shown on our NDC pages comes from CMS NADAC, a voluntary survey of retail community pharmacy invoices. CMS does not publish a NADAC for every NDC — packages outside the retail survey (institutional and hospital products, bulk packages, discontinued items, and many OTC items) may never receive one. A missing price reflects the survey's scope, not this product's actual cost, and does not mean the product is free or unavailable.
Is the NDC printed on the package the same as the 11-digit billing NDC?
Yes, they identify this exact package in different formats. The form printed on the packaging and shown on DailyMed is the one the FDA registered. Insurance claims use a fixed 11-digit 5-4-2 format, so the short segment is padded with a leading zero and the dashes are dropped. The Identity section at the top of this page lists each form of this code.
Is this package still being marketed?
Yes, per the latest FDA NDC Directory data on this page: this package is listed as actively marketed, with no marketing end date reported by Pharming Healthcare Inc.. Listing status can change — the directory data on this page refreshes weekly.
Who lists this product with the FDA?
Pharming Healthcare Inc. is the labeler of record for this NDC — the company under whose FDA-assigned code the package is listed. The labeler may be the manufacturer itself or a distributor marketing the product under its own code.
Do I need a prescription for this product?
This NDC is listed with FDA as a prescription product, so it is dispensed under a prescriber's order. Your pharmacist can tell you whether any over-the-counter forms of the same medication exist.
Does this product have a billing J-code?
Yes — this NDC cross-references HCPCS code J0596 for medical-claim billing (typically used when a product is administered in a clinical setting rather than dispensed at a retail pharmacy). See the Billing section on this page.
This page identifies an FDA-listed package (the NDC) and reports public regulatory and pricing data about the listing. It is reference information, not a medical recommendation — talk to your pharmacist or prescriber about your own medication.
Where does this data come from?
Listing facts (marketing category, packager status, marketing dates) from the FDA openFDA NDC Directory; label availability from DailyMed; pricing coverage from CMS NADAC; equivalence scope from the FDA Orange Book.
← 71274-0170-60 · Pharming 71277-0125-00 · Abhilasha →
For educational and professional reference only — not medical advice. Pricing reflects published NADAC and CMS ASP (free public data) and may differ from your acquisition cost; always verify before billing or dispensing.