Aquinox Pharmaceuticals Announces Presentation on FLAGSHIP Study at the 2014 American Thoracic Society International Conference

May 14, 2014 – Aquinox Pharmaceuticals Inc.

Vancouver, British Columbia, May 14, 2014 – Aquinox Pharmaceuticals, Inc. (“Aquinox”, NASDAQ: AQXP), a clini- cal stage pharmaceutical company discovering and developing targeted therapeutics for inflammatory disease and cancer, today announced that the company will present a “study in progress” poster on the FLAGSHIP trial of its lead develop- ment candidate, AQX-1125, at the 2014 American Thoracic Society (ATS) International Conference. The conference will be held May 16-21, 2014 in San Diego, CA.

AQX-1125, a SHIP1 activator, is currently being investigated in the Phase 2 FLAGSHIP trial, a randomized, double- blind, placebo controlled trial that is enrolling unstable, moderate to severe chronic obstructive pulmonary disease (COPD) patients who have experienced a recent exacerbation and at least two other exacerbations in the prior 18 months. FLAGSHIP’s novel trial design utilizes the recently developed EXAcerbations of Chronic pulmonary disease Tool (EXACT), a highly sensitive patient-reported outcome (PRO) measurement tool for evaluating the symptoms of COPD patients. Top line results are anticipated in the first quarter of 2015.

Presentation details: Publication Number A2889: “The Flagship Study: A 12-Week Phase II Study To Evaluate The Efficacy And Safety Of AQX-1125 Following Exacerbations In Patients With Chronic Obstructive Pulmonary Disease (COPD) By Tar- geting The SHIP1 Pathway,” S.B. Shrewsbury, et al. The poster will be presented Monday, May 19 from 10:45 A.M. to 12:30 P.M. in Area A (Hall B2-C, Ground Level) Poster Board Number A30. A pdf of the poster will be available at www.aqxpharma.com following the presentation.

About Aquinox Pharmaceuticals
Aquinox Pharmaceuticals, Inc. is a clinical stage pharmaceutical company discovering and developing novel, small molecule therapeutics targeting SH2-containing inositol-5’-phosphatase 1 (“SHIP1”) for the treatment of inflammatory disease and cancer. For more information, please visit www.aqxpharma.com.

Cautionary Note on Forward-looking Statements
Certain of the statements made in this press release are forward looking, such as those, among others, relating to the therapeutic potential of AQX-1125 and the timing of results of the FLAGSHIP trial. Actual results or developments may differ materially from those projected or implied in these forward-looking statements. More information about the risks and uncertainties faced by Aquinox is contained in the company’s Quarterly Report on Form 10-Q for the quarter ended March 31, 2014 filed with the Securities and Exchange Commission. Aquinox disclaims any intention or obligation to update or revise any forward-looking statements, whether as a result of new information, future events or otherwise.

Contact Info:

Brendan Payne, Senior Manager, Investor Relations
Aquinox Pharmaceuticals, Inc.
604.629.9223
ir@aqxpharma.com

Bianca Nery
MacDougall Biomedical Communications
650.339.7533
aquinox@macbiocom.com