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Santhera Completes Capital Increase For Financing Arrangements

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NOT FOR RELEASE, PUBLICATION OR DISTRIBUTION IN THE UNITED STATES OR ANY OTHER JURISDICTION IN WHICH IT WOULD BE UNLAWFUL TO DO SO.

Ad hoc announcement pursuant to Art. 53 LR

Pratteln, Switzerland, September   23 , 202 1 - Santhera Pharmaceuticals (SIX: SANN) announces completion of the ordinary capital increase resolved by its shareholders on June   22, 202 1 . Santhera's share capital currently amounts to CHF 46,470,235 .

On September 22, 2021, 9,972,502 shares were issued in an ordinary capital increase to investors who subscribed for Santhera shares in the private placement announced on September 20, 2021. The new shares will be listed as per September 23, 2021. In addition, Santhera updated its articles of association to reflect past share issuances out of the conditional capital for employee participation and out of the conditional capital for financings.

Santhera plans to issue the remaining shares subscribed by investors in the private placement on or around September 24, 2021.

About SantheraSanthera Pharmaceuticals (SIX: SANN) is a Swiss specialty pharmaceutical company focused on the development and commercialization of innovative medicines for rare neuromuscular and pulmonary diseases with high unmet medical need. Santhera has an exclusive license for all indications worldwide to vamorolone, a first-in-class dissociative steroid with novel mode of action, which was investigated in a pivotal study in patients with DMD as an alternative to standard corticosteroids. The clinical stage pipeline also includes lonodelestat (POL6014) to treat cystic fibrosis (CF) and other neutrophilic pulmonary diseases as well as an exploratory gene therapy approach targeting congenital muscular dystrophies. Santhera out-licensed rights to its first approved product, Raxone® (idebenone), outside North America and France for the treatment of Leber's hereditary optic neuropathy (LHON) to Chiesi Group. For further information, please visit www.santhera.com.

Raxone ® is a trademark of Santhera Pharmaceuticals.

For further information please contact: public-relations@santhera.com orEva Kalias, Head External CommunicationsPhone: +41 79 875 27 80 eva.kalias@santhera.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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