ACTELION S FINANCIAL PERFORMANCE



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ACTELION S Actelion delivered a strong performance during the first quarter of. Product sales rose to 589 million Swiss francs, an increase of 11% at constant exchange rates (CER)1. Actelion continues to measure, report and issue guidance on its core operating results, which more accurately reflects the underlying business performance. Core results exclude contract revenues, as well as costs related to employees stock compensation programs, depreciation, amortization, impairments and other items that management deems exceptional. Core operating income rose to 249 million Swiss francs, an increase of 8% at CER. Core net income rose by 10% at CER to 215 million Swiss francs, and core diluted earnings per share (EPS) rose by 17% at CER to 1.98 Swiss francs. US GAAP net income amounted to 178 million Swiss francs, and US GAAP diluted earnings per share amounted to 1.64 Swiss francs. CONTENTS Financial Overview Product Sales Core Operating Expenses Operating Income Diluted Core EPS Shares at a Glance 1 CER percentage changes are calculated by reconsolidating both the and results at constant currencies (the average monthly exchange rates for )

(except for per share data) Results Results % Variance in CHF % Variance at CER(1) Product sales 589 515 14 11 Core operating expenses 341 297 15 13 Core operating income 249 218 14 8 Diluted Core EPS 1.98 1.61 23 17 Diluted US GAAP EPS 1.64 1.38 19 11 No of shares in calculation 108.9 115.3 - - (million) The full financial statements can be found on www.actelion.com. 515 493 514 519 589 Actelion delivered an outstanding first quarter driven by the continued successful uptake of Opsumit, a very strong Uptravi launch, consistently strong recruitment of new PAH patients across markets, and ERA market expansion due to increased combination therapy with PDE-5 inhibitors. In the US, sales increased by 25% at CER, driven by the strong Uptravi launch, continued Opsumit momentum and ERA market share gains. European sales were flat at CER with increased Opsumit uptake and Tracleer use in the digital ulcer indication offset by continued pricing pressure and market erosion from generics. Sales in Japan increased by 11% at CER and were driven by Opsumit (launched in June ), Veletri, and Zavesca (Japanese trade name Brazaves). Sales in the rest of the world decreased by 14%, at CER. Comparing average exchange rates for the first three months of to the first three months of, the Swiss franc weakened, mostly against the US dollar and the Euro, resulting in a positive currency variance of 17 million Swiss francs. EPS

297 288 286 357 341 Core cost of sales decreased by 12% at CER to 46 million Swiss francs, despite an increase in sales of 11% at CER. Royalties in the first quarter were considerably lower, mainly due to ceased royalty obligations, following the patent expiry of Tracleer in the US (late November ) and Ventavis (mid- March ). Royalties were also lower due to a favorable product mix, with a low single-digit royalty rate paid on Opsumit sales compared to a high single-digit rate paid on Tracleer sales in markets where Tracleer is still under patent protection. Following the launch on January 4,, Actelion now pays to Nippon Shinyaku a mid-teen digit royalty rate on Uptravi net sales. Core R&D expenses amounted to 126 million Swiss francs, an increase of 29% at CER. This increase was driven by higher clinical trial expenses, mainly driven by the strong recruitment in the Phase III Optimum study (ponesimod in multiple sclerosis announced in April ) and the Phase III Impact study (Cadazolid in Clostridium difficile associated diarrhea) in order to complete enrollment before year-end. Core R&D expenditure represented 21% of product sales. Actelion will continue to focus on carefully balancing investments so as to ensure future growth and delivery of appropriate shareholder returns. Core SG&A expenses amounted to 169 million Swiss francs, an increase of 11% at CER. This increase was driven mostly by costs relating to launch activities of Uptravi in the United States as well as preparations for the anticipated European launches. Additionally, the company is continuing the roll-out of Opsumit and Veletri in various markets around the globe. G&A expenses increased by 3%. 163 218 205 228 249 Core operating income amounted to 249 million Swiss francs, an increase of 8% or 18 million Swiss francs at CER. The weakening of the Swiss franc against the major currencies affecting Actelion s performance (US dollar, euro and Japanese yen) had a positive impact of 13 million Swiss francs on core operating income. EPS in CHF 1.22 1.61 1.51 1.83 1.98 The increase in core EPS was driven by higher net income and the decrease in number of common shares. The average share count for diluted EPS decreased by 6.4 million shares, as the average number of dilutive instruments decreased by 0.4 million shares despite an increase in the average share price (135 Swiss francs per share compared to 109 Swiss francs in ). EPS

The registered shares of Actelion Ltd were listed on the SWX New Market on April 6, 2000 (symbol: ATLN). A total of 1,000,000 primary shares were placed at the company s Initial Public Offering (IPO), at a price of CHF 260 per share, raising CHF 246.6 million. On 8 July 2008, the Management Committee of the SIX Swiss Exchange added Actelion shares (ATLN, CH0010532478) to the Swiss Market Index SMI. Actelion shares have traded as part of the SMI since 22nd September 2008. On 5 December 2013, Actelion announced a new program to purchase up to 8.31% (up to 10 million shares) of the outstanding share capital over the next three years. The purchased registered shares will be used for the reactive servicing of existing employee option and share ownership programs. On 8 April, Actelion commenced the repurchase of up to 10 million shares of the company s common stock over the next three years via a second trading line on the SIX Swiss exchange. At subsequent Annual General Meetings, the Board of Directors will propose that the shares bought through this process be cancelled and the issued share capital reduced accordingly. Actelion Ltd is part of the following indices: SMI, SPI, SLI, SPIMLC, SPI20, SPISMC, SXXP, SXDP, SBC100, SNSPIX, SMHCAX, SLDI, SXI LIFE SCIENCES and SXI Bio+Medtech. Actelion is traded under the following symbols: Reuters ATLN.S/ Bloomberg ATLN. Key Share Data As of 31 March : Shares outstanding as of 31 March 114.1 million Closing share price as of 31 March 143.70 Market capitalization as of 31 March 16.4 billion 52-week high 146.20 52-week low 115.30 YTD price change 5% Average daily volume 436k Free float 91.7% Shareholdings As of 31 March : Number of registered shareholders 10,766 Major shareholders Management & Directors >3 % Actelion Ltd >5 % Rudolf Maag >3 % Blackrock >5 % Share price development over 12 months Actelion Ltd SMI May Jul. Sep. Nov. Jan. Mar. Stock charting tools available at www.actelion.com. EPS

Actelion Pharmaceuticals Ltd is a leading biopharmaceutical company focused on the discovery, development and commercialization of innovative drugs for diseases with significant unmet medical needs. The company is headquartered in Allschwil/Basel, Switzerland and is quoted on the SIX Swiss Exchange (tickersymbol: ATLN). All trademarks are legally protected. Disclaimer This fact sheet has the sole purpose to provide members of the public with general information about the activities of Actelion Ltd and its associated companies. The forward-looking statements in this fact sheet are based on current expectations and belief of company management, which are subject to numerous risks and uncertainties. Latest Update: April Copyright Actelion Pharmaceuticals Ltd Actelion Pharmaceuticals Ltd Gewerbestrasse 16 / CH-4123 Allschwil Switzerland / investor.relations@actelion.com / Phone +41 61 565 62 62 Fax +41 61 565 65 07 / www.actelion.com