ORPHAZYME'S PHASE II/III TRIAL IN SPORADIC INCLUSION BODY MYOSITIS FULLY ENROLLED

Copenhagen, April 23, 2019 – Orphazyme A/S (ticker: ORPHA.CO), a biopharmaceutical company dedicated to developing treatments for patients living with rare diseases, today announced that its Phase II/III trial of arimoclomol for the treatment of sporadic Inclusion Body Myositis (sIBM) is fully enrolled. Performance of interim analysis and study completion expected in H1 2020 and by end 2020, respectively. Results are expected in H1 2021.

MAJOR SHAREHOLDER ANNOUNCEMENT

Copenhagen, April 17, 2019 – Orphazyme A/S (ticker: ORPHA.CO), a biopharmaceutical company dedicated to developing treatments for patients living with rare diseases, hereby announces the receipt of notification pursuant to Section 38 of the Danish Capital Markets Act from Danske Bank A/S that Danske Bank as of April 16, 2019 holds a total 1,000,395 shares in the Company, corresponding to 5.00% of the share capital and 7.69% of the voting rights in the Company.

SHARE CAPITAL AND VOTING RIGHTS IN ORPHAZYME A/S

Copenhagen, March 29, 2019 – Following a share capital increase in Orphazyme A/S (ticker: ORPHA.CO), CVR no. 32266355 (“Orphazyme”) in March 2019, the total nominal share capital of Orphazyme is DKK 19,984,799, divided into 19,984,799 shares each with a nominal value of DKK 1. Each share carries one vote at Orphazyme’s general meetings and accordingly the total number of voting rights in Orphazyme is 19,984,799.

ORPHAZYME A/S TO PRESENT AT COWEN & CO ANNUAL HEALTH CARE CONFERENCE

Copenhagen, Denmark, March 11, 2019 – Orphazyme A/S (ticker: ORPHA.CO), a biopharmaceutical company dedicated to developing treatments for patients living with rare diseases, today announced that Anders Hinsby, Chief Executive Officer at Orphazyme, will be presenting at the Cowen & Co Annual Health Care Conference in Boston, MA, USA on March 12, 2019.

REPORTING OF TRANSACTIONS IN ORPHAZYME'S SHARES MADE BY PERSONS DISCHARGING MANAGERIAL RESPONSIBILITIES

Copenhagen, Denmark, March 8, 2019 – Pursuant to the Market Abuse Regulation article 19, Orphazyme A/S, CVR no. 32266355 (“Orphazyme”), hereby notifies receipt of information of the following transactions in Orphazyme’s shares by persons discharging managerial responsibilities in Orphazyme:

REPORTING OF TRANSACTIONS IN ORPHAZYME'S SHARES MADE BY PERSONS DISCHARGING MANAGERIAL RESPONSIBILITIES

Copenhagen, Denmark, March 7, 2019 – Pursuant to the Market Abuse Regulation article 19, Orphazyme A/S, CVR no. 32266355 (“Orphazyme”), hereby notifies receipt of information of the following transactions in Orphazyme’s shares by persons discharging managerial responsibilities in Orphazyme:

REPORTING OF TRANSACTIONS IN ORPHAZYME'S SHARES MADE BY PERSONS DISCHARGING MANAGERIAL RESPONSIBILITIES

Copenhagen, Denmark, March 5, 2019 – Pursuant to the Market Abuse Regulation article 19, Orphazyme A/S, CVR no. 32266355 (“Orphazyme”), hereby notifies receipt of information of the following transactions in Orphazyme’s shares by closely associated persons to persons discharging managerial responsibilities in Orphazyme: