ORPHAZYME EXPECTS TO ANNOUNCE RESULTS OF FULL DATA SET FOR NIEMANN-PICK DISEASE TYPE C (NPC) PHASE II/III TRIAL IN Q1 2019

Copenhagen, December 10, 2018 – Orphazyme A/S, a biopharmaceutical company dedicated to developing treatments for patients living with rare diseases, today announced that the Company expects to announce the results of the full data set for the clinical Phase II/III trial in NPC in Q1 2019.

On September 28, 2018, Orphazyme announced top-line data showing a treatment benefit in favor of arimoclomol, even if statistical significance was not reached. A subsequent evaluation of the data has motivated a further thorough data review and Orphazyme has initiated a data verification process that will be performed before completing the final statistical analysis of the trial.

Thomas Blaettler, Chief Medical Officer, said, “In this pivotal trial every single data point counts, which has led us to conduct our analysis at the highest level of granularity.”

The comprehensive data verification process is on-going and we expect to communicate the results of the full data set in Q1 2019, instead of in Q4 2018 as previously communicated.

This company announcement does not impact the 2018 financial guidance published in the Annual Report 2017 on March 15, 2018.