Full Press Release Details
Akari Therapeutics Announces Positive Initial Phase II Clinical
Data in Orphan Skin Disease Bullous Pemphigoid
NEW YORK and LONDON, April 23, 2019 - 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
Prior to treatment with Nomacopan (Coversin), two out of the
three patients were already on topical corticosteroids (mometasone) while a third was na ve 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."
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.
Cautionary Note Regarding Forward-Looking Statements
Certain statements in this press release constitute "forward-looking
statements" within the meaning of the Private Securities Litigation Reform Act of 1995. These forward-looking statements
reflect our current views about our plans, intentions, expectations, strategies and prospects, which are based on the information
currently available to us and on assumptions we have made. Although we believe that our plans, intentions, expectations, strategies
and prospects as reflected in or suggested by those forward-looking statements are reasonable, we can give no assurance that the
plans, intentions, expectations or strategies will be attained or achieved. Furthermore, actual results may differ materially from
those described in the forward-looking statements and will be affected by a variety of risks and factors that are beyond our control.
Such risks and uncertainties for our company include, but are not limited to: needs for additional capital to fund our operations,
our ability to continue as a going concern; uncertainties of cash flows and inability to meet working capital needs; an inability
or delay in obtaining required regulatory approvals for Nomacopan (Coversin) and any other product candidates, which may result
in unexpected cost expenditures; our ability to obtain orphan drug designation in additional indications; risks inherent in drug
development in general; uncertainties in obtaining successful clinical results for Nomacopan (Coversin) and any other product candidates
and unexpected costs that may result therefrom; difficulties enrolling patients in our clinical trials; failure to realize any
value of Nomacopan (Coversin) and any other product candidates developed and being developed in light of inherent risks and difficulties
involved in successfully bringing product candidates to market; inability to develop new product candidates and support existing
product candidates; the approval by the FDA and EMA and any other similar foreign regulatory authorities of other competing or
superior products brought to market; risks resulting from unforeseen side effects; risk that the market for Nomacopan (Coversin)
may not be as large as expected; risks associated with the departure of our former Chief Executive Officers and other executive
officers; risks associated with the SEC investigation; inability to obtain, maintain and enforce patents and other intellectual
property rights or the unexpected costs associated with such enforcement or litigation; inability to obtain and maintain commercial
manufacturing arrangements with third party manufacturers or establish commercial scale manufacturing capabilities; the inability
to timely source adequate supply of our active pharmaceutical ingredients from third party manufacturers on whom the company depends;
unexpected cost increases and pricing pressures and risks and other risk factors detailed in our public filings with the U.S. Securities
and Exchange Commission, including our most recently filed Annual Report on Form 20-F filed with the SEC. Except as otherwise noted,
these forward-looking statements speak only as of the date of this press release and we undertake no obligation to update or revise
any of these statements to reflect events or circumstances occurring after this press release. We caution investors not to place
considerable reliance on the forward-looking statements contained in this press release.
For more information
Sukaina Virji / Nicholas Brown / Lizzy Seeley
Consilium Strategic Communications