Principia Announces Expanded Development Program to Broaden BTK Footprint in Immune-Mediated Diseases

Company’s lead candidate PRN1008 receives generic name -- rilzabrutinib

Rilzabrutinib to enter clinical trial in IgG4-Related Disease

SOUTH SAN FRANCISCO, Calif., Jan. 07, 2020 (GLOBE NEWSWIRE) -- Principia Biopharma Inc. (Nasdaq: PRNB), a late-stage biopharmaceutical company focused on developing treatments for immune-mediated diseases, today announced an expansion in the development of rilzabrutinib (PRN1008) into IgG4-Related Disease (RD). This clinical program broadens the company’s Bruton tyrosine kinase (BTK) therapeutic areas and supports the company’s commitment to innovate and solve high unmet needs.

“Given BTK is critical in multiple signaling pathways across a wide range of immune-mediated diseases, we are expanding our clinical focus to advance the utility of BTK inhibition. Rilzabrutinib has now demonstrated rapid onset and proof of concept in two diverse therapeutic areas, pemphigus (dermatology) and immune thrombocytopenia (hematology) and our new clinical program in IgG4-RD (rheumatology) will allow us to further demonstrate the breadth of our lead clinical candidate,” said Martin Babler, Principia’s president and chief executive officer. 

About IgG4 Related Diseases
IgG4-RD is an immune-mediated disease of chronic inflammation and fibrosis that, if left untreated, can lead to severe morbidity including organ dysfunction and organ failure, which can be fatal. IgG4-related disease typically manifests with multiple organ involvement including but not limited to exocrine glands, GI tract organs (liver, pancreas), and kidneys. Principia believes rilzabrutinib can potentially lead to positive outcomes in IgG4-RD by impacting many of the driving features of the disease, including inflammation, allergic components (IgE and eosinophils), monocytes, macrophages (involved in fibrosis), and B cells implicated in initiation and maintenance of disease.

“There is a tremendous need for new treatment options for patients with IgG4-RD. A clinical trial with rilzabrutinib is very exciting for the rheumatology community as it has the potential to stop the inflammation, allergic components and fibrosis without chronic steroids or immune depleting agents. Rilzabrutinib has the potential to be a single oral agent that rapidly gets to the heart of the disease,” said John Stone, MD, MPH, professor of medicine in Boston and principal investigator.