Akari Therapeutics Announces Positive Initial Phase II Clinical Data in Orphan Skin Disease Bullous Pemphigoid

Treatment with Nomacopan (Coversin) in three patients with mild-to-moderate bullous pemphigoid resulted in no drug-related adverse events and rapid reduction in Bullous Pemphigoid Disease Area Index (BPDAI) global score and blistering in ongoing Phase II clinical trial

NEW YORK, NY, USA and LONDON, UK I April 23, 2019 I Akari Therapeutics, Plc (Nasdaq: AKTX), a biopharmaceutical company focused on innovative therapeutics to treat orphan autoimmune and inflammatory diseases where the complement and/or leukotriene systems are implicated, announces positive initial Phase II clinical data from the first three of bullous pemphigoid (BP) patients in an ongoing clinical trial.

Bullous pemphigoid is a severe orphan inflammatory skin disease currently treated primarily with steroids and immunosuppressants which bring with them well known side effects. Treatment response and steroid potency varies significantly based on the severity of the disease, although flares and relapses frequently occur.

In patients with bullous pemphigoid there is evidence that both terminal complement activation (C5) and the lipid mediator leukotriene B4 (LTB4) have a central role in driving the disease. Ex vivo data, from a recent study at Lubeck University, in BP patients showed a pronounced accumulation of LTB4 and C5 and its activation products in the inflamed skin of bullous pemphigoid disease patients.

The Phase II trial for up to nine mild-to-moderate bullous pemphigoid patients is a six-week open-label single-arm study evaluating safety and with the main efficacy measure the Bullous Pemphigoid Disease Area Index (BPDAI) a frequently used evaluation of the extent and severity of the disease.

Initial results from the first three patients showed that Nomacopan (Coversin), dosed daily subcutaneously, was well tolerated in three elderly patients (>65 years), and that there were no drug-related adverse events.

Prior to treatment with Nomacopan (Coversin), two out of the three patients were already on topical corticosteroids (mometasone) while a third was naïve to steroid treatment. Steroids were reduced at weekly intervals so that by day 21 both patients were only treated with Nomacopan (Coversin). In the 7-11-day period prior to initiation on Nomacopan (Coversin), the two patients on steroids showed either no or minor improvement in their BPDAI global score (between 0% and 5%) and no improvement in blisters.

By Day 7, 21 and 42 of treatment with Nomacopan (Coversin), the BPDAI global score fell by a mean of 31%, 45% and 52%, respectively.

By Day 7, 21 and day 42 of treatment with Nomacopan (Coversin), blisters/erosions dropped by a mean of 45%, 75% and 87%, respectively.

“The initial Phase II data in BP patients treated with Nomacopan (Coversin) is very encouraging, indicating that BP can potentially be resolved without the adverse issues caused by current steroidal treatments,” said Professor Detlef Zillikens and Professor Christian Sadick – lead investigators – Department of Dermatology, University of Lubeck, Germany. “We are impressed by the rapidity of the improvement in patients’ BPDAI and blister score which is predictive of overall response. This initial data supports the idea that the combination of C5 and LTB4 provides a potential new treatment option for patients suffering from this debilitating disease.”

A graph accompanying this announcement is available at http://www.globenewswire.com/NewsRoom/AttachmentNg/9b0b8fe9-681d-48b5-a02f-801559113cf4

Clive Richardson, interim CEO of Akari Therapeutics, said, “As a result of this encouraging data, we plan to expand the trial to include additional severe patients by way of an amendment. We believe this promising data helps validate our strategy of focusing on those poorly treated orphan diseases where both C5 and LTB4 are implicated.”

About Akari Therapeutics

Akari is a biopharmaceutical company focused on developing inhibitors of acute and chronic inflammation, specifically for the treatment of rare and orphan diseases, in particular those where the complement (C5) or leukotriene (LTB4) systems, or both complement and leukotrienes together, play a primary role in disease progression. Akari's lead drug candidate, Nomacopan (Coversin), is a C5 complement inhibitor that also independently and specifically inhibits leukotriene B4 (LTB4) activity. Nomacopan (Coversin) is currently being clinically evaluated in four indications: bullous pemphigoid (BP), atopic keratoconjunctivitis (AKC), thrombotic microangiopathy (TMA), and paroxysmal nocturnal hemoglobinuria (PNH). Akari believes that the dual action of Nomacopan (Coversin) on both C5 and LTB4 may be beneficial in AKC and BP. Akari is also developing other tick derived proteins, including longer acting versions.

SOURCE: Akari Therapeutics

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