There are various treatments for hereditary angioedema (HAE), which can cause life-threatening swelling of the airways or intestinal tract, but all of them are given by injection or intravenous infusion. Pharvaris is looking to change that—the Dutch company raised $66 million in series B financing to ramp up the clinical development of an oral drug for the rare disease.
Pharvaris drew the capital from a marquee group of investors, including Foresite Capital, Bain Capital Life Sciences, ven-Bio Partners and Venrock, with existing backers LSP, Kurma Partners and Idinvest Partners also chipping in.
HAE stems from the deficiency of a plasma protein called C1-esterase inhibitor. People with HAE suffer sudden attacks of edema, or rapid swelling, in the feet, limbs, face, gastrointestinal tract or airways. Several drugs have been approved to treat HAE, including CSL’s Haegarda and Shire’s Cinryze, both of which boost C1-esterase inhibitor activity. Firazyr (icatibant), developed at Jerini and now sold by Takeda, was approved in 2011. It is the only HAE med that treats all kinds of HAE by targeting the bradykinin B2 receptor.
"Patients with HAE are eager for effective oral therapies. The development of a novel, oral B2-receptor antagonist could represent a new standard of care for the treatment and prevention of HAE and other B2-receptor-mediated conditions,” said Pharvaris Chief Scientific Officer and Chief Operating Officer Jochen Knolle, Ph.D., in a statement. Knolle was one of the inventors of Firazyr and shepherded the drug through development and approval when he was CSO and R&D chief at Jerini.
Now, Knolle is working on a drug that hits the same target as Firazyr, but is given orally. He’s doing it with some of his Jerini colleagues—Pharvaris CEO Berndt Modig was CFO at Jerini—as well as former execs from Prosensa, a rare disease specialist scooped up by BioMarin in 2015.
The drug, PHA121, blocks signaling at the B2 receptor to inhibit bradykinin, an inflammatory protein. Pharvaris has started a phase 1 study in healthy volunteers and is planning an ascending-dose study for early 2020.
"Our experienced team is capitalizing on its deep knowledge of drug development and HAE as we progress in the clinic with PHA121, a new chemical entity targeting the same mechanism as icatibant, a leading therapy for the treatment of HAE attacks,” Modig said in the statement. “The demands for less invasive routes of drug administration, more convenient dosing regimens, and additional treatment options support the development of an oral therapy to improve the quality of life of patients with HAE."
Pharvaris isn’t the only company working on an oral HAE drug. In January, Attune Pharmaceuticals raised $23 million to bankroll a phase 2 study for its candidate, which targets the enzyme kallikrein. North Carolina-based BioCryst Pharma is aiming at the same target.