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Media Release

20 October 2016

Excellentoperational results–supported by outstandingUptravi launch – financial guidance upgraded

ALLSCHWIL/BASEL, SWITZERLAND – 20 October 2016– Actelion Ltd (SIX: ATLN) today announced its results for the first ninemonths of 2016.

OPERATINGHIGHLIGHTS

  • Strong Opsumit® (macitentan) trajectory andquarterly netnew patient gains sustained
  • Excellent US launchmomentum for Uptravi® (selexipag) – enhanced by launches in Germany and the Netherlands
  • Uptravi® (selexipag)approvals in Switzerland and Japan(by Nippon Shinyaku) in Q3
  • Firstall-oral combination therapy with ponesimod and dimethyl fumarate(Tecfidera®)to be evaluatedfor patients with relapsing multiple sclerosis

FINANCIAL HIGHLIGHTS

  • Salesgrowing to CHF 1,785 million(+14% at CER) – New PAH products surpass Tracleer for the first time
  • Opsumitsales grow to CHF 596million (+63% at CER)
  • Uptravi sales reachCHF 160millionafter just 9 months on the market
  • US GAAP operating income grows to CHF 660 million (+17% at CER)
  • Core operating incomegrows to CHF 781million(+14% at CER)
  • 2016 financial guidanceupgraded: core operating income growthin the mid-teen percentage range,at constant exchange rates and barring unforeseen events

% variance
in CHF million
(except for per share data) / 9M 2016 / 9M2015 / in CHF / at CER(1)
US GAAPresults
Net revenue / 1,791 / 1,525 / 17% / 14%
Operating income / 660 / 533 / 24% / 17%
Net income / 581 / 452 / 29% / 21%
Diluted EPS / 5.37 / 3.99 / 35% / 27%
Core performance(2)
Product sales / 1,785 / 1,522 / 17% / 14%
Core operating income / 781 / 651 / 20% / 14%
Core net income / 691 / 560 / 23% / 17%
Core diluted EPS / 6.38 / 4.94 / 29% / 23%
Cash flow / 9M 2016 / 9M 2015
Operating cash flow / 695 / 533
Capital expenditure / (45) / (17)
Free cash flow / 6 / (506)
Net cash position as of 30 September / 411 / 698

(1)CER percentage changes are calculated by reconsolidating both the 9M 2015and9M 2016 results at constant currencies (the average monthly exchange rates for 9M 2015).

(2)Actelion continues to measure, report and issue guidance on its core operating performance, which management believes more accurately reflects the underlying business performance. The Group believes that these non-GAAP financial measurements provide useful supplementary information to investors. These non-GAAP measures are reported in addition to, not as a substitute for, US GAAP financial performance.

Jean-Paul Clozel, MD, Chief Executive Officer,commented: “Once again, we have delivered a very strong performance across all areas of our business.I am particularly happy with the very positive feedback we receive from physicians prescribing Uptravi.Wehave also made considerable progress advancingour innovative pipeline, highlighted by the recent initiation of the combination study with ponesimod in relapsing multiple sclerosis. In summary, the transformation of the company is well under way.”

Otto Schwarz, Chief Operating Officer, commented: “We continue to be extremely pleased with the transformation of our PAH portfolio. For the first time, in the third quarter, more than 50% of our sales came from our new outcome-basedPAH portfolio. The strong uptake of Uptravi in the US continued during the third quarter,enhanced by a strong early trajectory in Germany, resulting in a total of over 1,800 patients benefiting from this novel oral therapy at the end of September 2016.”

André C. Muller, Chief Financial Officer, commented:“The excellent commercial performance has enabled us to increase our R&D effort to advance both the late and earlier stage pipeline, whilst continuing to deliver strong earnings growth. Our focus on the bottom line, coupled with strong salesenables us to increase guidance.Barring unforeseen events, we now expect 2016 core operating income growth in the mid-teen percentage range at constant exchange rates.”

SALES UPDATE

Actelion’s strongperformance continued during the third quarter of 2016 driven by the outstandingUptravi launch in the US and Opsumit’songoing growthdynamics. For the first time, during the third quarter of 2016, sales of the company’s outcome-based PAH portfolio, Opsumit,Uptravi and Veletri, exceeded 50% of total sales, demonstrating the extent of the transformation of the PAH franchise.

In the US, sales increased by 23% at CER, driven by the strong Uptravi launch, continued Opsumit momentum and ERA market share gains. European sales were at the same level as 2015,despite increased Opsumit uptake and Tracleer use in the digital ulcer indication,but mitigated bycontinued pricing pressure and market erosion from generics, particularly in Spain.Sales in Japan increased by 20% at CER, mostly driven by very strong sales ofOpsumit (launched in June 2015), Tracleer in digital ulcer indication, Veletri and Zavesca (Japanese trade name Brazaves).

Comparing average exchange rates for the first nine months of 2016 to the first nine months of 2015, the Swiss franc weakened, mostly against the US dollar, euro and Japanese yen, resulting in a positive currency variance of 54 million Swiss francs.

Sales by product – 9M 2016

% variance
in CHF millions / 9M2016 / 9M2015 / in CHF / at CER
Opsumit® / 596 / 354 / 68 / 63
Tracleer® / 790 / 934 / -15 / -18
Uptravi® / 160 / - / nm* / nm
Veletri® / 71 / 60 / 18 / 13
Ventavis® / 58 / 81 / -28 / -31
Valchlor® / 26 / 19 / 34 / 30
Zavesca® / 78 / 68 / 15 / 13
Others / 6 / 5 / 12 / 14
Total product sales / 1,785 / 1,522 / 17 / 14

*nm = not meaningful

Sales by product – Q3 2016

% variance
in CHF millions / Q32016 / Q32015 / in CHF / at CER
Opsumit® / 218 / 147 / 49 / 45
Tracleer® / 244 / 289 / -16 / -18
Uptravi® / 70 / - / nm / nm
Veletri® / 23 / 22 / 7 / 2
Ventavis® / 15 / 24 / -39 / -40
Valchlor® / 8 / 7 / 10 / 8
Zavesca® / 26 / 24 / 11 / 9
Others / 2 / 2 / 1 / -1
Total product sales / 606 / 514 / 18 / 15

Sales by region – 9M 2016

% variance
in CHF millions / 9M 2016 / 9M 2015 / in CHF / at CER
United States / 964 / 766 / 26 / 23
Europe* / 485 / 475 / 2 / 0
Japan / 182 / 132 / 38 / 20
Rest of the world / 155 / 149 / 4 / 6
Total product sales / 1,785 / 1,522 / 17 / 14

*Europe = EU28 and Switzerland

Sales by region – Q3 2016

% variance
in CHF millions / Q32016 / Q32015 / in CHF / at CER
United States / 325 / 271 / 20 / 18
Europe* / 163 / 157 / 3 / 3
Japan / 66 / 45 / 45 / 20
Rest of the world / 52 / 40 / 30 / 29
Total product sales / 606 / 514 / 18 / 15

*Europe = EU28 and Switzerland

PAH FRANCHISE

Opsumit®

Sales of Opsumit (macitentan) amounted to 596 million Swiss francs for the first nine months of 2016, an increase of 63% at CER compared to the first nine months of 2015. This increase continues to be driven by new patient starts with commercial availability in over 30 countries. The continuedincrease in patients benefitting from Opsumitis driven by referral of treatment-naïve patients together with increased use in combination with PDE-5 inhibitors, and some upgrades from Tracleer, notably in Japan.

Uptravi®

Sales of Uptravi (selexipag) amounted to 160 million Swiss francs for the first nine months of 2016. Since the US launch at the beginning of January 2016, patientdemand has continued to increasewith sales of 126 million Swiss francs (excluding initial inventory build of 30 million Swiss francs). For the third quarter, revenues from patient demand in the US amounted to 66 million Swiss francs compared to 45 million Swiss francs in the second quarter and 15 million Swiss francs in the first quarter of 2016. Uptravi was successfully launched in Germany in mid-June 2016 with sales reaching 3 million Swiss francs in an underdeveloped market in terms of prostacyclin usage. At the end of September, about 1,800 patients were using Uptraviglobally.

During the third quarter 2016, Uptraviwas approvedin Switzerland and Japan (where it will be co-promoted byNippon Shinyaku, who will record the sales) pending pricing and reimbursement approval.

Tracleer®

Sales of Tracleer (bosentan) amounted to 790 million Swiss francs for the first nine months of 2016, a decrease of 18% at CER compared to the first nine months of 2015, equally driven by lower underlying volume and average price. The decrease is mainly due to lower referrals of new patients as well as switches in countries where Opsumit is available, increased generic competition, notably in Spain, continued pricing pressure in Europe and lower inventories in the US.

Tracleer sales were supported by the digital ulcer indication in Europe and Japan and continued solid demand in markets where Opsumit is not yet available.

Following the Pediatric Investigation Plan (PIP) compliance statement from the European Committee for Medicinal Products for Human Use (CHMP), applications for extension of the Supplementary Protection Certificate (SPC) were granted in 19 EU countries until the end of August 2017.

Veletri®

Sales of Veletri (epoprostenol for injection) amounted to 71 million Swiss francs for the first nine months of 2016, an increase of 13% at CER compared to the first nine months of 2015. This increase was mostly driven by successfulmarket penetration, notably in France, and also Italy, Spain and UK. Demand in Japan remained solid, but sales were impacted by a 12% price cut effective from 01 March 2016.

Ventavis®

Sales of Ventavis (iloprost) amounted to 58 million Swiss francs for the first nine months of 2016, a decrease of 31% at CER, compared to the first nine months of 2015. This is due to the competitive environment, including the availability of Uptravi. Underlying units decreased by 18%.

SPECIALTY PRODUCTS

Valchlor®

Sales of Valchlor (mechlorethamine) amounted to 26 million Swiss francs for the first nine months of 2016, an increase of 30% at CER, compared to the first nine months of 2015. In the US, the company is continuing its efforts to establish Valchlor as an option in the treatment algorithm for early-stage mycosis fungoides, a type of Cutaneous T-Cell Lymphoma (MF-CTCL).In France, patients benefited from the drug under a temporary nominative authorization for use (“ATU”) program initiated during the second half of 2014.

The regulatory dossier is currently under review with the European Medicines Agency (under the trade name Ledaga®).

Zavesca®

Sales of Zavesca (miglustat) amounted to 78 million Swiss francs for the first nine months of 2016, an increase of 13% at CER compared to the first nine months of 2015.Sales in the US were strong due to a relatively low prior year base as a consequence of last year’s inventory adjustment.In Europe, sales decreased by 2% mainly due to the launch of generic miglustat (approved for the type 1 Gaucher disease indication only). Sales in Japan were 16% higher, driven by increased patient demand in the Niemann-Pick type C indication.

The global number of patients receiving therapy grew by 7% compared to the first nine months of 2015, driven by a 16% increase in Niemann-Pick type C demand.

CLINICAL DEVELOPMENT PIPELINE

The pipeline continued to strengthen withsubstantial progress made with several compounds:

  • In the third quarter, Actelion announced the initiation of the Phase III POINT study, which investigates the use of combination therapy with ponesimod, an orally active, selective sphingosine-1-phosphate receptor1 (S1P1) immunomodulator, and dimethyl fumarate (Tecfidera®) for patients with relapsing multiple sclerosis (RMS). The POINT study - which will be conducted under a Special Protocol Assessment (SPA) agreement with the FDA - is the first to assess the concurrent administration of two oral therapies in MS with the objective to improve disease control in this progressive, debilitating neurological disorder. Ponesimod is also being studied in the double blind long-term extension of the Phase II study as well as in thePhase III study OPTIMUM to compare the efficacy and safety of ponesimod with teriflunomide in patients with RMS. Enrollment for the OPTIMUM study is on target to be completed around the end of 2016.
  • Also in the third quarter, the company advanced its new dual orexin receptor antagonist (DORA) into Phase II development in patients with insomnia. The Phase II program consists of two studies, one in adult and one in elderly patients, and is designed to evaluate the effect of Actelion's DORA versus placebo on sleep maintenance and sleep initiation, as well as next-day residual effect and next-day performance. The study in adults also includes a zolpidem reference arm. The decision to move into a Phase II program was based on excellent data collected from the preclinical and Phase I clinical program, as well as a thorough understanding of the potential of dual orexin receptor antagonism on sleep efficacy and architecture.
  • With macitentan (Opsumit), we are entering into a pediatric study, TOMORROW, to evaluate the effect of macitentan on delaying disease progression in children with PAH using a pediatric formulation of macitentan.The global trial design has received endorsement from the US FDA (through a pediatric Written Request) and in the EU (through a Paediatric Investigation Plan). Enrollment is expected to start around the end of 2016.
  • A Phase II study, MERIT, assesses the efficacy, safety and tolerability of macitentan in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). The study is on schedule to deliver results by the end of the year.
  • Enrollment of participants in the ongoing Phase III program IMPACT investigating cadazolid treatment in patients suffering from Clostridium difficile-associated diarrhea is progressing well and is on target to be completed around the end of 2016.

Compound / Indication / Study / Status
Phase III / Cadazolid / Clostridium difficile-associated diarrhea / IMPACT / Ongoing
Macitentan / Eisenmenger syndrome / MAESTRO / Ongoing
Macitentan / Pediatric PAH / TOMORROW / Initiating
Ponesimod / Multiple sclerosis / OPTIMUM / Ongoing
Ponesimod / Multiple sclerosis / POINT / Initiating
Phase II / Cenerimod / Systemic lupus erythematosus / - / Ongoing
Clazosentan / Reversal of vasospasm associated with aneurysmal subarachnoid hemorrhage / REVERSE / Ongoing
Dual Orexin Receptor Antagonist / Insomnia / - / Ongoing
Endothelin Receptor Antagonist / Specialty cardiovascular disorders / - / Ongoing
Macitentan / Chronic thromboembolic pulmonary hypertension / MERIT / Ongoing
Macitentan / Combined pre- and post-capillary pulmonary hypertension / MELODY / Complete
Ponesimod / Graft-versus-host disease / - / Ongoing
Phase Ib / Lucerastat / Fabry disease / - / Complete
Phase I / New Chemical Entity / Cardiovascular disorders / - / Ongoing
New Chemical Entity / Inflammatory disorders / - / Ongoing
Selective Orexin 1 Receptor Antagonist / Neurological disorders / - / Ongoing
T-type Calcium Channel Blocker / Neurological disorders / - / Ongoing

RESULTS DAY CENTER

Investor community: To make your job easier, we provide links to all relevant documentation, such as a full financial review, reconciliation US-GAAP to Core results and geographical breakdown by product, from the Results Day Center on our corporate website:

UPCOMING CLINICAL UPDATES

  • Cardiovascular pipeline update with MERIT results
  • Lucerastat future development
  • Cadazolid Phase III program update

UPCOMING FINANCIAL UPDATES

  • FY 2016 Financial Results reporting on 14 February 2017
  • Q1 2017 Financial Results reporting on 20 April 2017
  • HY 2017 Financial Results reporting on 20 July 2017

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NOTES TO THE EDITOR

ABOUT ACTELION LTD.

Actelion Ltd. is a leading biopharmaceutical company focused on the discovery, development and commercialization of innovative drugs for diseases with significant unmet medical needs.

Actelion is a leader in the field of pulmonary arterial hypertension (PAH). Our portfolio of PAH treatments covers the spectrum of disease, from WHO Functional Class (FC) II through to FC IV, with oral, inhaled and intravenous medications. Although not available in all countries, Actelion has treatments approved by health authorities for a number of specialist diseases including Type 1 Gaucher disease, Niemann-Pick type C disease, Digital Ulcers in patients suffering from systemic sclerosis, and mycosis fungoides type cutaneous T-cell lymphoma.

Founded in late 1997, with now over 2,500 dedicated professionals covering all key markets around the world including Europe, the US, Japan, China, Russia and Mexico, Actelion has its corporate headquarters in Allschwil / Basel, Switzerland.

Actelion shares are traded on the SIX Swiss Exchange (ticker symbol: ATLN) as part of the Swiss blue-chip index SMI (Swiss Market Index SMI®). All trademarks are legally protected by their respective owners.

For further information please contact:

Andrew Weiss

Senior Vice President, Head of Investor Relations & Corporate Communications

Actelion Pharmaceuticals Ltd, Gewerbestrasse 16, CH-4123 Allschwil

+41 61 565 62 62

- First Nine Months 2016 Financial Results -