Specialty Pipeline

<< Drug Pipeline Review

Last updated: 07.20.21

DRUG NAME/ MANUFACTURER PROPOSED USE COST ESTIMATE PER PATIENT HOW IT WORKS WHAT’S IMPORTANT
abrocitinib (Pfizer) atopic dermatitis (AD) $55,000/yr Janus kinase 1 (JAK1) inhibitor for the treatment of patients with moderate-to-severe atopic dermatitis (AD); inhibiting JAK 1 is thought to modulate pro-inflammatory cytokines which are key drivers in the pathophysiology of AD. Route of administration: Oral
Benefit coverage: Pharmacy
Anticipated FDA decision: July 2021
Impact: The American Academy of Dermatology estimates that between 10% and 20% of children and about 1% to 3% of adults are affected by AD.
adagrasib (Mirati Therapeutics) KRAS G12Cmutated
locally advanced or metastatic non-small cell lung cancer (NSCLC)
$215,000/yr KRAS is an oncogene.  When mutated, oncogenes can lead to normal cells becoming cancerous. A mutation at position 12 leads to improved tumor cell survival.  Adagrasib is a KRAS G12C inhibitor which is meant to inhibit growth and lead to destruction of tumor cells. Route of administration: Oral
Benefit coverage: Pharmacy
Anticipated FDA decision: 2022
Impact: Around 14% of NSCLC patients carry KRAS G12C mutations, and the cancer-linked gene is implicated in up to one-third of all human cancers. KRAS G12C mutations are frequently linked to negative outcomes. Each year in the U.S., approximately 25,000 patients with NSCLC with G12C mutations may be candidates for treatment with a KRAS inhibitor.
arimoclomol (Miplyffa - Orphazyme) Niemann-
Pick Disease Type C (NPC)
$500,000/yr Molecular chaperone activator that stimulates the normal cellular protein repair pathway for the treatment of Niemann- Pick Disease Type C (NPC). Route of administration: Oral
Benefit coverage: Pharmacy
Anticipated FDA decision: June 2021
Impact: At an estimated one case of NPC in 120,000 live births, fewer than 50 new cases are discovered in the U.S each year. The total U.S. patient population is believed to be about 200.
bardoxolone methyl (Reata) chronic kidney disease caused by Alport Syndrome $55,000/yr Antioxidant inflammation inhibitor that acts on Nuclear factor erythroid 2-related factor 2 (Nrf2) activator for the treatment of chronic kidney disease (CKD) due to Alport syndrome, a genetic condition that causes irreversible weakening and changes in the kidney that eventually lead to disease requiring transplant or dialysis. Route of administration: Oral
Benefit coverage: Pharmacy
Anticipated FDA decision: Feb 2022
Impact: There are approximately 30K-60K Americans with Alport syndrome. No drugs are currently approved for Alport syndrome.
belumosudil (Kadmon Pharmaceuticals) treatment of chronic graft-versus-host disease (cGVHD) $180,000/yr A selective rho-associated coiled-coil kinase 2 (ROCK2) inhibitor. Inhibiting this enzyme downregulates the ability of the immune system's T cells to secrete IL-21 and IL-17, thereby dampening the immune response. Route of administration: Oral
Benefit coverage: Pharmacy
Anticipated FDA decision: 3Q21
Impact: cGVHD is a common and often fatal complication following hematopoietic stem cell transplantation. In cGVHD, transplanted immune cells (graft) attack the patient's cells (host), leading to inflammation and fibrosis in multiple tissues. Approximately 14,000 patients in the U.S. currently live with cGVHD.
beta beglogene darolentivec
(Zynteglo - Bluebird Bio)
β thalassemia $2 million+ for one-time infusion Gene therapy for the treatment of transfusion-dependent β thalassemia. Upon extraction of the patients own CD34+ hematopoietic stem cells, the cells are genetically modified using a lentiviral gene vector to deliver the ß A-T87Q beta-globin gene to these cells. Once modified, they are returned to the patient. Route of administration: Intraveneous
Benefit coverage: Medical
Anticipated FDA decision: mid-2021
Impact: β-thalassemia is estimated to affect approximately one in 100,000 individuals in the general population.  In the U.S., thalassemia’s prevalence is approximately one in 272,000 or about 1,000 people.
bimekizumab (UCB) plaque psoriasis $65,000/yr Monoclonal antibody that blocks the effects of IL-17A and IL-17F for the treatment of moderate-to-severe plaque psoriasis. Route of administration: Subcutaneous
Benefit coverage: Pharmacy
Anticipated FDA decision: October 2021
Impact: Plaque psoriasis affects approximately 8M patients in the U.S.  Moderate-to-severe plaque psoriasis accounts for nearly 35% of psoriasis cases.
ciltacabtagene autoleucel (Janssen) multiple myeloma $475,000 B cell maturation antigen (BCMA)-directed chimeric antigen receptor T cell (CAR-T) therapy in previously treated patients with multiple myeloma. Multiple myeloma is an incurable blood cancer that affects a type of white blood cell called plasma cells, which are found in the bone marrow. Route of administration: Intraveneous
Benefit coverage: Medical
Anticipated FDA decision: Npvember 2021
Impact: It is estimated that 32,270 people were diagnosed and 12,830 died from the disease in the U.S. in 2020.
deucravacitinib (Bristol Myers Squibb) plaque psoriasis $45,000/yr Tyrosine kinase 2 (TYK2) inhibitor for use in patients with moderate to severe plaque psoriasis. It inhibits the interleukin (IL)-12, IL-23, and Type 1 interferon pathways, which are implicated in the pathogenesis of psoriasis and other immune-mediated diseases. Route of administration: Oral
Benefit coverage: Pharmacy
Anticipated FDA decision: 2021
Impact: Plaque psoriasis affects approximately 8M patients in the U.S. Moderate-to-severe plaque psoriasis accounts for nearly 35% of psoriasis cases.
efgartigimod (Argenx) myasthenia gravis (MG) $450,000/yr FcRn-targeting antibody fragment designed to deplete pathogenic IgGs for the treatment of MG.   MG is an autoimmune disease that is caused by poor nerve signaling resulting in the weakness of the voluntary muscles. Route of administration: Intraveneous
Benefit coverage: Medical
Anticipated FDA decision: December 2021
Impact: MG is thought to affect approximately 65,000 Americans, but prevalence is probably higher since it is often underdiagnosed.
eladocagene exuparvovec (PTC Therapeutics) aromatic-L-amino-acid decarboxylase (AADC) deficiency $4M for one-time infusion Recombinant, adeno-associated virus containing the human DNA to replace an enzyme, AADC, the  deficiency of which is a rare neurologic disease which may be fatal. Route of administration: Intracerebral infusion
Benefit coverage: Medical
Anticipated FDA decision: 2021
Impact: AACD deficiency is extremely rare; about 100 patients with this disease have been identified in the U.S.
inclisiran (Leqvio - Novartis) high LDL cholesterol $5,500/yr Proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitor that uses RNA interference to treat elevated levels of low-density cholesterol; treatment of adults with atherosclerotic cardiovascular disease (ASCVD) or heterozygous familial hypercholesterolemia who have elevated LDL-C while being on a maximum tolerated dose of a lipidlowering therapies.  Route of administration: Subcutaneous
Benefit coverage: Pharmacy
Anticipated FDA decision: May 2021
Impact: It’s estimated that there are more than 11M patients in the U.S. with familial
hypercholesterolemia and/or clinical ASCVD.
obeticholic acid (Intercept
Pharmaceuticals)
nonalcoholic steatohepatitis (NASH) $15K-$20K/yr Farnesoid X receptor (FXR) agonist for the treatment of NASH; Nonalcoholic fatty liver disease, a common form of chronic liver disease, is characterized by fat buildup in the liver.  Its more severe progressive form, NASH, damages liver cells and causes inflammation, potentially resulting in fibrosis, cirrhosis, end-stage liver disease, liver transplant, liver cancer and liver-related death. Route of administration: Oral
Benefit coverage: Pharmacy
Anticipated FDA decision: 2022
Impact: It’s estimated that there are around 3M NASH patients with advanced fibrosis (F3 or F3-like, without cirrhosis) in the US, of which around 500,000 patients are thought to be under the care of a hepatologist or gastroenterologist. These 500,000 patients are Intercept’s target patient population for obeticholic acid.
odevixibat (Bylvay - Albireo) progressive familial intrahepatic cholestasis (PFIC) $500,000/yr Oral iIleal bile acid transport inhibitor (IBATi) for the treatment of progressive familial intrahepatic cholestasis (PFIC). PFIC is an inherited progressive liver disease that is caused by a disruption in bile acid flow and formation. Route of administration: Oral
Benefit coverage: Pharmacy
Anticipated FDA decision: 3Q21
Impact: PFIC is a rare disease that has an incidence between one out of 50,000 to 100,000 births, with an exact prevalence that is unknown. Children with PFIC appear to have a prevalence between 9%-13% among those suffering from other liver diseases. Of children who suffer from cholestasis or need a liver transplant, 10%-15% of cases are caused by PFIC
pegunigalsidase alfa (Protalix BioTherapeutics) Fabry disease $300,000/yr Plant cell-expressed, recombinant alpha-galactosidase-A enzyme for the treatment of Fabry disease (FD). FD is a rare genetic disease
caused by mutations in galactosidase alpha gene (GLA), resulting in little to no α-galactosidase-A (α-Gal-A), a lysosomal enzyme.
Route of administration: Intraveneous
Benefit coverage: Medical
Anticipated FDA decision: 2022
Impact: FD affects an estimated one in 40,000 to 60,000 males, while female prevalence remains unknown. There are about 7,000 patients in the U.S. with FD.
sutimlimab (Sanofi) primary cold agglutinin
disease (CAD)
$300,000+/yr C1s inhibitor. Sutimlimab works by inhibiting C1s, a complement-cascade protease, to block signals for inflammation and cell destruction; CAD is a type of nonhereditary autoimmune hemolytic anemia. Route of administration: Intraveneous
Benefit coverage: Medical
Anticipated FDA decision: 2022
Impact: At a prevalence of
around 16 per 1M people, an estimated 5,200 Individuals in the U.S. may be affected, but one-half or more of patients have mild forms of the condition.
teplizumab (Provention Bio) to delay or prevent the onset of type 1 diabetes in at-risk individuals $500,000 per patient over two cycles Humanized anti-CD-3 specific monoclonal antibody to prevent or delay clinical type one diabetes (T1D) in at-risk individuals. There is a genetic predisposition to inheriting T1D which, coupled with environmental triggers that are not fully known, leads to certain autoantibodies and destruction of the β-cells in the islets of Langerhans of the pancreas. Route of administration: Intravenous
Benefit coverage: Medical
Anticipated FDA decision: 2021-2022
Impact: Newly diagnosed T1D, also known as juvenile diabetes, is considered an orphan indication due to its high disease burden and unmet need in the U.S.  Provention Bio believes teplizumab could have a target population of 30,000 annually, representing about 15% of the 200,000 who have two or more autoantibodies, dysglycemia and would be genetic relatives at risk for TD1. The company also believes that awareness will lead to further screening of T1D relatives and could encourage broader testing globally with a total addressable patient population of 2.3M people.
tralokinumab (LEO Pharma) atopic dermatitis (AD) $40,000/yr Anti-IL-13 for the treatment of moderate to severe ADA.  Atopic dermatitis (AD) is a chronic skin disease characterized by inflammation of the skin and skin barrier defects. Route of administration: Subcutaneous
Benefit coverage: Pharmacy
Anticipated FDA decision: 2021 - 2022.
Impact: The American Academy of Dermatology (AAD) estimates that between 10% and 20% of children and about 1% to 3% of adults are affected by AD.
valoctocogene roxaparvovec
(Roctavian – BioMarin
Pharmaceuticals)
hemophilia A $2-3 million as one-time infusion Valoctocogene is an adeno-associated virus (AAV) vector gene therapy that can penetrate cells without harming them. It replaces the missing gene needed to produce factor VIII. Route of administration: Intraveneous
Benefit coverage: Medical
Anticipated FDA decision: 2022 - 2023
Impact: According to BioMarin, about 2,400 patients in the U.S. will be candidates for treatment with valoctocogene.
vosoritide (Voxzogo -BioMarin) achondroplasia $350,000/yr Analog of C-type Natriuretic Peptide (CNP) for the treatment of children with achondroplasia, the most common form of disproportionate short stature in humans. Achondroplasia results from mutations of FGFR3 genes. Route of administration: Subcutaneous
Benefit coverage: Pharmacy
Anticipated FDA decision: November 2021
Impact: Achondroplasia affects about one birth in 10,000 to 30,000; or around 100 to 400 babies per year in the U.S. Among the estimated 30,000 Americans living with the condition, roughly one-quarter of individuals are under the age of 18 – the years when growth plates in bones typically remain open, allowing for increases in height.

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