Boehringer Ingelheim. (3/7/19). "Press Release: Novel Antibody Shows Potential to Transform Treatment of Rare Form of Psoriasis". Ingelheim.

	Region	Washington, D.C.
	Country	United States (USA)
	Organisation	Boehringer Ingelheim (Group)
	Product	BI 655130 (IL-36R MAb, Boehringer)
	Product 2	clinical research
	Person	Poth, Jan (SciRhom 202205– Managing Director (CEO) before Boehringer 201712 Therapeutic Area Head CNS + Immunology)

> Published in The New England Journal of Medicine, new data show single dose BI 655130* rapidly cleared symptoms of a rare and potentially life-threatening form of pustular psoriasis1

> BI 655130 is an investigational, potential first-in-class treatment targeting interleukin-36 receptor (IL-36R)

> BI 655130 is being studied in generalised pustular psoriasis as the first of several potential indications, including inflammatory bowel diseases

The New England Journal of Medicine (NEJM) today published new data from a Phase I clinical trial showing BI 655130, a first-in-class investigational treatment, significantly improved symptoms of generalised pustular psoriasis (GPP), a rare form of psoriasis.1 BI 655130 is a monoclonal antibody that blocks the action of the interleukin-36 receptor (IL-36R), a signalling pathway within the immune system that may play a role in many inflammatory diseases.

The newly published clinical data, which were also presented at the recent American Academy of Dermatology (AAD) annual meeting in Washington D.C.,2 indicate that BI 655130 rapidly improved symptoms in seven patients with GPP, who were experiencing acute moderate or severe disease flares.1 Five of seven patients in the 20-week, Phase I clinical trial achieved clear or almost clear skin within the first week, following a single dose of treatment, and all patients achieved this outcome after four weeks.1 The average improvement in patients’ skin symptoms was close to 80 per cent at week four and was maintained until the end of the study (week 20).1

“The tailored targeting of the IL-36 pathway is one of the most exciting new areas in dermatology research, and progress in this mechanism has been eagerly anticipated by the scientific community,” commented the trial’s principal investigator, Professor Hervé Bachelez, Hôpital Saint-Louis, Paris, France. “This trial provides long-awaited clinical data that demonstrates the positive effect of blocking IL-36 action as a potential, novel treatment approach. The rapid improvement seen in patients from just a single dose of BI 655130 show strong potential for the future treatment of GPP.”

The rare skin disease, GPP, is a chronic condition that is distinct from the more common condition, plaque psoriasis.3 It has a considerable impact on people’s quality of life. The skin becomes red and erupts into numerous blisters of non-infectious pus (pustules), covering wide areas of the body. 4,5,6 People who develop GPP may experience an abrupt onset of fever, chills and painful skin lesions.4,5,6 GPP may be associated with life-threatening organ failure and infectious complications, and, therefore, should be considered a medical emergency.7 Available treatment options for GPP are extremely limited and lack profound and persistent efficacy. Therefore, there is a strong need for new treatment options for GPP with rapid, strong and persistent efficacy. 8

“BI 655130 is a novel antibody discovered by Boehringer Ingelheim and is being investigated for the treatment of multiple inflammatory diseases in the hope of transforming the care currently available for these patients,” said Dr Jan Poth, Therapeutic Area Head, CNS and Immunology at Boehringer Ingelheim.

“We are one of the first companies to focus on targeting IL-36 in dermatology, a reflection of our long-term commitment to researching and developing transformative medicines for patients where there is still high unmet need. Due to its potential, we are moving to the next phase of clinical trials, involving larger numbers of patients with GPP. Trials of BI 655130 are also underway in other immune related conditions, such as palmo-plantar pustulosis, ulcerative colitis, Crohn’s disease and atopic dermatitis,” continued Poth.

Study details

The Phase I trial is the first of its kind to include patients who were experiencing an acute, moderate-to-severe flare of GPP.1

Each of the seven patients received a single intravenous dose of BI 655130 and was monitored for 20 weeks.1 The severity of GPP was assessed using the Generalized Pustular Psoriasis Physician Global Assessment score and the Generalized Pustular Psoriasis Area and Severity Index score.

> At the end of week one, clear or almost clear skin was achieved in five of seven patients, and in all study participants by week four.1

> At the end of week two, a 73.2 per cent average improvement in skin symptoms was observed in all study participants.1

> Overall, BI 655130 was well tolerated with no drug-related severe or serious adverse events or safety signals.1

BI 655130 is the second compound developed by Boehringer Ingelheim’s immunology division; the first, risankizumab, an IL-23 blocker is part of a collaboration between Boehringer Ingelheim and AbbVie, with AbbVie leading future development and commercialisation of risankizumab globally.

About Boehringer Ingelheim

Improving the health and quality of life of patients is the goal of the research-driven pharmaceutical company Boehringer Ingelheim. The focus in doing so is on diseases for which no satisfactory treatment option exists to date. The company, therefore, concentrates on developing innovative therapies that can extend patients’ lives. In animal health, Boehringer Ingelheim stands for advanced prevention.

Family-owned since it was established in 1885, Boehringer Ingelheim is one of the pharmaceutical industry’s top 20 companies. Some 50,000 employees create value through innovation daily for the three business areas human pharmaceuticals, animal health and biopharmaceuticals. In 2017, Boehringer Ingelheim achieved net sales of nearly 18.1 billion euros. R&D expenditure, exceeding three billion euros, corresponded to 17.0 per cent of net sales.

As a family-owned company, Boehringer Ingelheim plans in generations and focuses on long-term success. The company, therefore, aims at organic growth from its own resources with simultaneous openness to partnerships and strategic alliances in research. In everything it does, Boehringer Ingelheim naturally adopts responsibility towards mankind and the environment.

More information about Boehringer Ingelheim can be found on www.boehringer-ingelheim.com or in our annual report: http://annualreport.boehringer-ingelheim.com.

Intended audiences:

This press release is issued from our Corporate Headquarters in Ingelheim, Germany and is intended to provide information about our global business. Please be aware that information relating to the approval status and labels of approved products may vary from country to country, and a country-specific press release on this topic may have been issued in the countries where we do business.

*BI 655130 is an investigational compound. Its safety and efficacy have not been established.

References

1 Bachelez H, Choon SE, Marrakchi S, et al. Inhibition of the IL-36 Pathway for the Treatment of Generalized Pustular Psoriasis. N Engl J Med 2019; 380:(10).
2 Krueger JG, Bachelez H, Choon SE, et al.The therapeutic efficacy of BI 655130, an anti-interleukin-36 receptor antibody, in patients with acute generalized pustular psoriasis is associated with the downregulation of inflammatory and tissue remodeling genes in lesional skin’
Oral presentation, annual meeting of the American Academy of Dermatology, March 1-5 2019, Washington D.C.
3 Navarini AA, Burden AD, Capon F, et al. European consensus statement on phenotypes of pustular psoriasis. J Eur Acad Dermatol Venereol. 2017;31:1792-1799.
4 Umezawa Y, Ozawa A, Kawasima T, et al. Therapeutic guidelines for the treatment of generalized pustular psoriasis (GPP) based on a proposed classification of disease severity. Arch Dermatol Res. 2003;295 Suppl 1:S43-54.
5 Viguier M, Allez M, Zagdanski AM, et al. High frequency of cholestasis in generalized pustular psoriasis: Evidence for neutrophilic involvement of the biliary tract. Hepatology. 2004;40(2):452-458.
6 Augey F, Renaudier P, Nicolas JF. Generalized pustular psoriasis (Zumbusch): a French epidemiological survey. Eur J Dermatol. 2006;16(6):669-673.
7 Practical Dermatology. Addressing Localized and Generalized Pustular Psoriasis—A Potential Medical Emergency. Available at: http://practicaldermatology.com/2010/11/addressing-localized-and-generalized-pustular-psoriasisa-potential-medical-emergency (link is external) [last accessed February 2019].
8 Robinson A, Van Voorhees AS, Hsu S, et al. Treatment of pustular psoriasis: from the Medical Board of the National Psoriasis Foundation. J Am Acad Dermatol. 2012; 67(2):279-288.

Record changed: 2023-06-05