CONSOLIDATED US GAAP FINANCIAL STATEMENTS FOR SEPTEMBER 30, 2016

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1 CONSOLIDATED US GAAP FINANCIAL STATEMENTS FOR SEPTEMBER 30, 2016 Contents: CONSOLIDATED INCOME STATEMENTS CONSOLIDATED STATEMENTS OF COMPREHENSIVE INCOME CONSOLIDATED BALANCE SHEETS CONSOLIDATED STATEMENTS OF CASH FLOWS CONSOLIDATED STATEMENTS OF CHANGES IN SHAREHOLDERS' EQUITY

2 CONSOLIDATED INCOME STATEMENTS Three months ended Nine months ended September 30, September 30, (in CHF thousands, except per share amounts) (unaudited) (unaudited) (unaudited) (unaudited) Net revenue Product sales 606' '298 1'785'317 1'522'181 Contract revenue 5' '680 3'126 Total net revenue 611' '701 1'790'997 1'525'307 Operating (expenses) 1 Cost of sales 2 (53'531) (42'940) (154'091) (130'665) Research and development (122'559) (104'675) (399'948) (331'178) Selling, general and administration (174'037) (164'654) (535'670) (490'353) Amortization of acquired intangible assets (13'652) (13'091) (41'164) (39'918) Total operating (expenses) (363'779) (325'360) (1'130'873) (992'114) Operating income 247' ' ' '193 Interest income (expense), net 147 (738) 605 (6'283) Other financial income (expense), net 1'109 (925) 3'233 (12'763) Total financial income (expenses) 1'256 (1'663) 3'838 (19'046) Income before income tax benefit (expense) 248' ' ' '147 Income tax benefit (expense) (28'603) (23'176) (83'851) (65'153) Net income 220' ' ' '994 Less: Net loss attributable to the noncontrolling interests '248 3'172 Net income attributable to Actelion's shareholders 220' ' ' '166 Basic net income per share attributable to Actelion's shareholders Weighted-average number of common shares (in thousands) 104' ' ' '194 Diluted net income per share attributable to Actelion's shareholders Weighted-average number of common shares (in thousands) 107' ' ' '406 1 Includes stock-based compensation as follows: Research and development (6'366) (6'018) (20'209) (17'605) Selling, general and administration (9'562) (8'801) (27'642) (24'191) Total stock-based compensation (15'928) (14'819) (47'851) (41'796) 2 Excludes amortization of intangible assets as presented separately.

3 CONSOLIDATED STATEMENTS OF COMPREHENSIVE INCOME Three months ended Nine months ended September 30, September 30, (in CHF thousands) (unaudited) (unaudited) (unaudited) (unaudited) Net income 220' ' ' '994 Other comprehensive income (loss), net of tax: Foreign currency translation adjustments 51 3'610 6'791 (25'023) Estimated effect of pension plans' merger (5'482) Amortization of components of net periodic benefit costs '193 1'419 Other comprehensive income (loss), net of tax 448 4'082 7'984 (29'086) Comprehensive income 220' ' ' '908 Less: Comprehensive loss attributable to noncontrolling interests '248 3'172 Comprehensive income attributable to Actelion's shareholders 221' ' ' '080

4 CONSOLIDATED BALANCE SHEETS September 30, December 31, (in CHF thousands, except number of shares) (unaudited) (audited) Assets Current assets Cash and cash equivalents 410' '892 Trade and other receivables, net 495' '223 Inventories 120'333 62'107 Other current assets 55'887 68'828 Total current assets 1'082' '050 Noncurrent assets Property, plant and equipment, net 343' '277 Intangible assets, net 395' '542 Goodwill 133' '494 Deferred tax assets 16'981 39'159 Other noncurrent assets 27'058 16'415 Total noncurrent assets 917' '887 Total assets 1'999'739 1'914'937 Liabilities Current liabilities Trade and other payables 101'828 83'878 Accrued expenses 367' '729 Other current liabilities 35'524 34'375 Total current liabilities 504' '982 Noncurrent liabilities Pension liability 66'565 67'204 Contingent considerations 86'428 83'759 Other noncurrent liabilities 32'832 27'979 Total noncurrent liabilities 185' '942 Total liabilities 690' '924 Equity Actelion's shareholders' equity Common shares (par value CHF 0.50 per share; authorized 147,753,077 and 154,120,627; issued 107,761,427 and 114,128,427 shares in 2016 and 2015, respectively) 53'881 57'064 Accumulated profit 2'084'262 2'636'931 Treasury shares, at cost (593'756) (1'137'399) Accumulated other comprehensive income (loss) (230'811) (238'795) Total Actelion's shareholders' equity 1'313'576 1'317'801 Noncontrolling interests Equity attributable to noncontrolling interests (4'036) (2'788) Total equity 1'309'540 1'315'013 Total liabilities and equity 1'999'739 1'914'937

5 CONSOLIDATED STATEMENTS OF CASH FLOWS Three months ended Nine months ended September 30, September 30, (in CHF thousands) (unaudited) (unaudited) (unaudited) (unaudited) Cash flow from operating activities Net income 220' ' ' '994 Adjustments to reconcile net income to net cash provided from operating activities: Depreciation and amortization 21'460 21'887 65'165 67'064 Stock-based compensation, incl. treasury shares to members of Board of Directors 16'159 15'065 48'560 42'506 Excess tax benefits from share-based payment arrangements (993) (697) (12'526) (11'235) Deferred taxes 9'314 19'073 37'668 50'734 Deferred revenue (670) (Gains) Losses on derivative instruments (4'316) 14'660 (6'615) (24'569) Interest expense on bonds - 3'010-8'997 Accretion expense (benefit) on contingent considerations 1'089 3'434 9'606 (3'224) Changes in operating assets and liabilities: Trade and other receivables (48'128) 40'160 (59'191) (18'216) Inventories 625 (4'115) (58'305) 1'838 Trade and other payables 29'409 6'454 23'863 (3'242) Accrued expenses 16'292 (22'054) 70'267 (38'888) Changes in other operating cash flow items 13'489 (6'185) (4'021) 12'805 Net cash flow provided by (used in) operating activities 275' ' ' '894 Cash flow from investing activities Purchase of property, plant and equipment (10'153) (3'377) (18'277) (11'680) Purchase of intangible assets (637) (671) (23'342) (2'893) Acquisition of a business, incl. contingent consideration payments (1'210) (960) (3'246) (2'055) Net cash flow provided by (used in) investing activities (12'000) (5'008) (44'865) (16'628) Cash flow from financing activities Dividend payment (3) - (158'513) (142'429) Payments on capital leases (3) Proceeds from exercise of stock options, net of expense 5'743 6'161 25'046 66'937 Purchase of treasury shares (276'719) (231'845) (524'738) (946'184) Excess tax benefits from share-based payment arrangements '526 11'235 Contributions from noncontrolling interests' owners '136 Net cash flow provided by (used in) financing activities (269'986) (224'987) (645'679) (1'009'308) Net effect of exchange rates on cash and cash equivalents (740) 8'420 1'748 (13'427) Net change in cash and cash equivalents (7'696) 33'727 5'818 (506'469) Cash and cash equivalents at beginning of period 418' ' '892 1'204'958 Cash and cash equivalents at end of period 410' ' ' '489

6 CONSOLIDATED STATEMENTS OF CHANGES IN SHAREHOLDERS' EQUITY Actelion's shareholders Noncontrolling interests (in CHF thousands, Additional Accum. other Equity attrib. to except number of shares) Common shares paid-in Accumulated Treasury comprehensive noncontrolling Total Shares Amount capital profit shares income (loss) interests equity At January 1, '128'427 57'064-2'359'573 (287'701) (208'513) - 1'920'423 Comprehensive income (loss) 1) : Net income 452'166 (3'172) 448'994 Other comprehensive income (loss) (29'086) (29'086) Comprehensive income (loss) 1) 419'908 Excess tax benefits from share-based payments 11'235 11'235 Transactions in treasury shares (5'166'161) 89'656 (261'488) (720'623) (892'455) Stock-based compensation expense 41'538 41'538 Dividend payment (142'429) (142'429) Contributions from owners 1'136 1'136 At September 30, 2015 (unaudited) 105'962'266 57'064-2'550'251 (1'008'324) (237'599) - (2'036) 1'359'356 Comprehensive income (loss) 1) : Net income 99'693 (752) 98'941 Other comprehensive income (loss) (1'196) (1'196) Comprehensive income (loss) 1) 97'745 Excess tax benefits from share-based payments (1'170) (1'170) Transactions in treasury shares (893'349) (13'667) (13'013) (129'075) (155'755) Stock-based compensation expense 14'798 14'798 Dividend payment Contributions from owners - - At December 31, 2015 (audited) 105'068'917 57'064-2'636'931 (1'137'399) (238'795) (2'788) 1'315'013 Comprehensive income (loss) 1) : Net income 581'359 (1'248) 580'111 Other comprehensive income (loss) 7'984 7'984 Comprehensive income (loss) 1) 588'095 Excess tax benefits from share-based payments 8'151 8'151 Transactions in treasury shares (1'410'447) 102'956 (296'775) (296'793) (490'612) Stock-based compensation expense 47'406 47'406 Cancellation treasury shares (share repurchase program) (3'183) - (837'253) 840'436 - Dividend payment (158'513) (158'513) At September 30, 2016 (unaudited) 103'658'470 53'881-2'084'262 (593'756) (230'811) (4'036) 1'309'540 1) Comprehensive income (loss) is presented net of tax.

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