Santhera Pharmaceuticals Holding AG

Hohenrainstrasse 24, 4133 Pratteln, Switzerland

Phone: +41 61 906 89 50 | Fax: +41 61 906 89 51

www.santhera.com

Santhera's Shareholders Approve all Board Proposals at Today's Annual

General Meeting

Pratteln, Switzerland, May 28, 2019 - Santhera Pharmaceuticals (SIX: SANN) announces that a large majority of shareholders approved all proposals by the Board of Directors at the Annual General Meeting (AGM) held today. Participating shareholders represented a total of 4'109'097 shares or 36.8% of the share capital.

"I am very pleased about the consent of Santhera's shareholders at today's AGM," said Elmar Schnee, Chairman of the Board of Directors. "The Company has successfully expanded its clinical stage product pipeline with focus on neuromuscular and pulmonary diseases. Key value inflection points are expected next year with Puldysa® (idebenone) and vamorolone, both development products for the treatment of Duchenne muscular dystrophy. I take today's votes of our shareholders as a clear sign of their confidence in our strategy and we thank them for their continued support."

Approval of the 2018 Annual Report, results appropriation and allocation of reserves

The shareholders endorsed the Annual Report, the annual financial statements and the consolidated financial statements for 2018, the appropriation of the net result to new account as well as the allocation of reserves from capital contributions to free reserves. Furthermore, the shareholders granted discharge to the members of the Board of Directors and Executive Management for the financial year 2018.

Consent to capital structure supports financial and strategic flexibility

In two separate votes, the shareholders approved the increase of conditional capital from CHF 930'000 to CHF 2'500'000 (22% of share capital) and the creation of authorized capital of CHF 3,000,000 (27% of share capital). With their consent, the shareholders support the financial flexibility of the Company in view of its growth plans.

Opting-out clause cancelled in Articles of Incorporation

After the shareholders voted in favor of abandoning the current opting-out provision, Santhera reintroduces the statutory threshold of 33⅓% of shares held of the Company which, if exceeded, triggers the obligation of an offer to acquire all listed equity securities of the company.

Re-election of members of the Board of Directors

Shareholders re-elected Elmar Schnee, Martin Gertsch, Philipp Gutzwiller, Patrick Vink and Thomas Meier as members of the Board of Directors, each for a term of one year. In addition, shareholders reelected Elmar Schnee as Chairman of the Board of Directors. Furthermore, the shareholders confirmed Elmar Schnee and Patrick Vink as members of the Compensation Committee.

Santhera's Shareholders Approve all Board Proposals at Today's Annual General Meeting May 28, 2019 / Page 2 of 2

Compensation Report and remuneration for Board and Executive Management approved

In a consultative vote, the shareholders endorsed the Compensation Report 2018. In separate binding votes, Santhera's shareholders agreed to the compensation for members of the Board of Directors (maximum total amount of fixed covering the period from the 2019 AGM to the 2020 AGM) and for the members of the Executive Management (maximum total amount of fixed compensation 2020 and variable compensation 2018).

AGM documents

The invitation with proposals and explanations on the agenda items and the CEO presentation are available on the Company's website at www.santhera.com/investors-and-media/investor- toolbox/shareholder-meetings. From the end of next week, the minutes of the Annual General Meeting 2019 will also be available.

About Santhera

Santhera Pharmaceuticals (SIX: SANN) is a Swiss specialty pharmaceutical company focused on the development and commercialization of innovative medicines for rare and other diseases with high unmet medical needs. The portfolio comprises clinical stage and marketed treatments for neuro- ophthalmologic, neuromuscular and pulmonary diseases. Santhera's Raxone® (idebenone) is authorized in the European Union, Norway, Iceland, Liechtenstein, Israel and Serbia for the treatment of Leber's hereditary optic neuropathy (LHON) and is currently commercialized in more than 20 countries. For further information, please visit www.santhera.com.

Raxone® and Puldysa® are trademarks of Santhera Pharmaceuticals.

For further information please contact: public-relations@santhera.comor

Eva Kalias, Head External Communications Phone: +41 79 875 27 80 eva.kalias@santhera.com

For Investors:

investor-relations@santhera.comor

Christoph Rentsch, Chief Financial Officer

Hans Vitzthum, LifeSci Advisors

Europe: +41 61 906 89 65

US: +1 212 915 2568

christoph.rentsch@santhera.com

hans@lifesciadvisors.com

Disclaimer / Forward-looking statements

This communication does not constitute an offer or invitation to subscribe for or purchase any securities of Santhera Pharmaceuticals Holding AG. This publication may contain certain forward-looking statements concerning the Company and its business. Such statements involve certain risks, uncertainties and other factors which could cause the actual results, financial condition, performance or achievements of the Company to be materially different from those expressed or implied by such statements. Readers should therefore not place undue reliance on these statements, particularly not in connection with any contract or investment decision. The Company disclaims any obligation to update these forward-looking statements.

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Santhera Pharmaceuticals Holding AG published this content on 28 May 2019 and is solely responsible for the information contained herein. Distributed by Public, unedited and unaltered, on 28 May 2019 10:48:06 UTC