Cautionary Note Regarding Forward-Looking Statements This Quarterly Report on Form 10-Q contains forward-looking statements that involve substantial risks and uncertainties. "Forward-looking statements," as that term is defined in the Private Securities Litigation Reform Act of 1995, Section 27A of the Securities Act of 1933, as amended (the "Securities Act") and Section 21E of the Securities Exchange Act of 1934, as amended (the "Exchange Act"), are statements that are not historical facts and involve a number of risks and uncertainties. Words herein such as "may," "will," "should," "could," "would," "expects," "plans," "anticipates," "believes," "estimates," "projects," "predicts," "intends," "potential," "continues," and similar expressions (as well as other words or expressions referencing future events, conditions or circumstances) identify forward-looking statements. Forward-looking statements are based on our current expectations and beliefs, and involve known and unknown risks, uncertainties and other factors, which may cause our actual results, performance and achievements and the timing of certain events to differ materially from the results, performance, achievements or timing discussed, projected, anticipated or indicated in any forward-looking statements. Such risks, uncertainties and other factors include, among others, the following: •failure to continue to successfully commercialize ARIKAYCE in the US, Europe or Japan (amikacin liposome inhalation suspension, Liposomal 590 mg Nebuliser Dispersion, and amikacin sulfate inhalation drug product, respectively) or failure to successfully commercialize BRINSUPRI in the US or Europe, or to maintain US, European or Japanese approval for ARIKAYCE or US or European approval for BRINSUPRI; •our inability to obtain full approval of ARIKAYCE from the FDA, or our failure to obtain regulatory approval to expand ARIKAYCE’s indication to a broader patient population; •failure to obtain, or delays in obtaining, regulatory approvals for our product candidates in the US, Europe or Japan, for ARIKAYCE outside of the US, Europe and Japan, including separate regulatory approval for Lamira in each market and for each usage, or for BRINSUPRI outside of the US and Europe; •failure to successfully commercialize our product candidates, if approved by applicable regulatory authorities, or to maintain applicable regulatory approvals for such product candidates, if approved; •uncertainties or changes in the degree of market acceptance of our marketed products or, if approved, our product candidates, by physicians, patients, third-party payors and others in the healthcare community; •our inability to obtain and maintain adequate reimbursement from government or third-party payors for our marketed products or, if approved, our product candidates, or acceptable prices for our marketed products or, if approved, our product candidates; •inaccuracies in our estimates of the size of the potential markets for our marketed products and our product candidates or in data we have used to identify physicians, expected rates of patient uptake, duration of expected treatment, or expected patient adherence or discontinuation rates; •failure of third parties on which we are dependent to manufacture sufficient quantities of our marketed products and our product candidates for commercial or clinical needs, as applicable, to conduct our clinical trials, or to comply with our agreements or laws and regulations that impact our business; •risks and uncertainties associated with, and the perceived benefits of, our senior secured loan with certain funds managed by Pharmakon and our royalty financing with OrbiMed, including our ability to maintain compliance with the covenants in the agreements for the senior secured loan and royalty financing and the impact of the restrictions on our operations under these agreements; •our inability to create or maintain an effective direct sales and marketing infrastructure or to partner with third parties that offer such an infrastructure for distribution of our marketed products or any of our product candidates that are approved in the future; •failure to successfully conduct future clinical trials for our marketed products or our product candidates and our potential inability to enroll or retain sufficient patients to conduct and complete the trials or generate data necessary for regulatory approval of our product candidates; •development of unexpected safety or efficacy concerns related to our marketed products or our product candidates; •risks that our clinical studies will be delayed, that serious side effects will be identified during drug development, or that any protocol amendments submitted will be rejected; 24 Table of Contents •failure to successfully predict the time and cost of development, regulatory approval and commercialization for novel gene therapy products; •risk that interim, topline or preliminary data from our clinical trials that we announce or publish from time to time may change as more patient data become available or may be interpreted differently if additional data are disclosed, or that blinded data will not be predictive of unblinded data; •risk that our competitors may obtain orphan drug exclusivity for a product that is essentially the same as a product we are developing for a particular indication; •our inability to attract and retain key personnel or to effectively manage our growth; •our inability to successfully integrate our acquisitions and appropriately manage the amount of management’s time and attention devoted to integration activities; •risks that our acquired technologies, products and product candidates will not be commercially successful; •inability to adapt to our highly competitive and changing environment; •inability to access, upgrade or expand our technology systems or difficulties in updating our existing technology or developing or implementing new technology; •risk that we are unable to maintain our significant customers; •risk that healthcare legislation or other government action materially adversely affects our business; •business or economic disruptions due to catastrophes or other events, including natural disasters or public health crises; •risk that our current and potential future use of AI and machine learning may not be successful; •deterioration in general economic conditions in the US, Europe, Japan and globally, including the effect of prolonged periods of inflation, affecting us, our suppliers, third-party service providers and potential partners; •risk that we could become involved in costly intellectual property disputes, be unable to adequately protect our intellectual property rights or prevent disclosure of our trade secrets and other proprietary information, and incur costs associated with litigation or other proceedings related to such matters; •restrictions or other obligations imposed on us by agreements related to our marketed products or our product candidates, including our license agreements with PARI and AstraZeneca, and failure to comply with our obligations under such agreements; •the cost and potential reputational damage resulting from litigation to which we are or may become a party, including product liability claims; •risk that our operations are subject to a material disruption in the event of a cybersecurity attack or issue; •changes in laws and regulations applicable to our business, including any pricing reform and laws that impact our ability to utilize certain third parties in the research, development or manufacture of our product candidates, and failure to comply with such laws and regulations; •our history of operating losses, and the possibility that we never achieve or maintain profitability; •goodwill impairment charges affecting our results of operations and financial condition; •inability to repay our existing indebtedness and uncertainties with respect to our ability to access future capital; and •delays in the execution of plans to build out an additional third-party manufacturing facility approved by the appropriate regulatory authorities and unexpected expenses associated with those plans. We caution readers not to place undue reliance on any such forward-looking statements, which speak only as of the date they are made. Any forward-looking statement is based on information current as of the date of this Quarterly Report on Form 10-Q and speaks only as of the date on which such statement is made. Actual events or results may differ materially from the results, plans, intentions or expectations anticipated in these forward-looking statements as a result of a variety of factors, many of which are beyond our control. More information on factors that could cause actual results to differ materially from those anticipated is included from time to time in our reports filed with the Securities and Exchange Commission (SEC), including, but not limited to, those described in the sections titled “Risk Factors” and “Management’s Discussion and Analysis of Financial Condition and Results of Operations” in this Quarterly Report on Form 10-Q and included in our Annual Report on Form 10-K for the fiscal year ended December 31, 2025. We disclaim any obligation, except as specifically required by law and the rules of the SEC, to publicly update or revise any such statements to reflect any change in our expectations or in events, 25 Table of Contents conditions or circumstances on which any such statements may be based, or that may affect the likelihood that actual results will differ from those set forth in the forward-looking statements. The following discussion should be read in conjunction with our consolidated financial statements and related notes thereto included elsewhere in this Quarterly Report on Form 10-Q and the consolidated financial statements and related notes thereto in our Annual Report on Form 10-K for the year ended December 31, 2025. OVERVIEW We are a people-first global biopharmaceutical company striving to deliver first- and best-in-class therapies to transform the lives of patients facing serious diseases. Our commercial portfolio and clinical pipeline are organized around three therapeutic areas: Respiratory, Immunology & Inflammation, and Neuro & Other Rare. To complement our internal research and development, we also actively evaluate in-licensing and acquisition opportunities for commercial products, product candidates, and technologies. For a more complete discussion of our business, strategy, products and pipeline, see Part I, Item 1, “Business,” of our Annual Report on Form 10-K for the year ended December 31, 2025. Our Marketed Products Our two commercial products, ARIKAYCE and BRINSUPRI, are both part of our Respiratory therapeutic area. ARIKAYCE is approved in the US as ARIKAYCE (amikacin liposome inhalation suspension), in Europe as ARIKAYCE Liposomal 590 mg Nebuliser Dispersion and in Japan as ARIKAYCE inhalation 590 mg (amikacin sulfate inhalation drug product). ARIKAYCE received accelerated approval in the US in September 2018 for the treatment of MAC lung disease as part of a combination antibacterial drug regimen for adult patients with limited or no alternative treatment options in a refractory setting. In October 2020, the EC approved ARIKAYCE Liposomal for the treatment of NTM lung infections caused by MAC in adults with limited treatment options who do not have CF. In March 2021, Japan's MHLW approved ARIKAYCE for the treatment of patients with NTM lung disease caused by MAC who did not sufficiently respond to prior treatment with a multidrug regimen. NTM lung disease caused by MAC (which we refer to as MAC lung disease) is a rare and often chronic infection that can cause irreversible lung damage and can be fatal. We are not aware of any other approved inhaled therapies specifically indicated to treat MAC lung disease in North America, Europe, or Japan. BRINSUPRI (brensocatib 25 mg and 10 mg tablets), an oral, once-daily treatment for non-cystic fibrosis bronchiectasis (referred to as bronchiectasis or NCFB) in patients 12 years of age and older, was approved in the US in August 2025. In November 2025, the EC approved BRINSUPRI (brensocatib 25 mg tablets) for the treatment of NCFB in patients 12 years of age and older with two or more exacerbations in the prior 12 months. In February 2026, the MHRA granted a marketing authorisation for BRINSUPRI (brensocatib 25 mg tablets) to treat patients 12 years and older with NCFB who have experienced two or more flare-ups or worsening of symptoms in the past 12 months. Bronchiectasis is a serious, chronic lung disease in which the bronchi become permanently dilated due to a cycle of infection, inflammation, and lung tissue damage. We are not aware of any other approved therapies in the US, Europe, or Japan for the treatment of patients with bronchiectasis. Our Product Candidates & Research Our Respiratory therapeutic area also includes the clinical-stage programs TPIP and INS1148. TPIP is an inhaled dry powder formulation of the treprostinil prodrug treprostinil palmitil which may offer a differentiated product profile for PH-ILD, PAH, PPF, and IPF. INS1148 is a monoclonal antibody targeting SCF248, which we plan to initially develop for PPF and IPF. We are exploring additional opportunities utilizing our various technologies within the Respiratory therapeutic area. Our Immunology & Inflammation therapeutic area is exploring opportunities utilizing our various technologies. The clinical-stage programs in our Neuro & Other Rare therapeutic area are INS1201, an intrathecally delivered gene therapy for patients with DMD, and INS1202, an intrathecally delivered gene therapy for patients with ALS. We are exploring additional opportunities utilizing our various technologies within the Neuro & Other Rare therapeutic area. We are advancing a wide range of technologies and modalities, including gene therapy, AI-driven protein engineering, RNA end-joining, and synthetic rescue, in the pursuit of future pipeline candidates. 26 Table of Contents Our Strategy We plan to continue to develop, acquire, in-license, or co-promote other first- and best-in-class commercial products, product candidates, and technologies, including those that address serious diseases that currently have significant unmet needs. We are focused broadly on serious disease therapeutics and prioritizing those within our three therapeutic areas. Our key priorities are as follows: •Ensure successful US commercialization of BRINSUPRI; •Continue to provide ARIKAYCE to appropriate patients and expand our label; •Advance our pipeline and produce topline clinical data readouts in the near and long term; and •Control spending, prudently deploying capital to support the best return-generating opportunities. Prior to 2019, we had not generated significant revenue and, through June 30, 2026, we had an accumulated deficit of $5.8 billion. We have financed our operations primarily through the public offerings of our equity securities, debt financings and revenue interest financings. Although it is difficult to predict our future funding requirements, based upon our current operating plan, we anticipate that our cash and cash equivalents and marketable securities as of June 30, 2026 will enable us to fund our operations for at least the next 12 months. Our ability to reduce our operating loss and begin to generate positive cash flow from operations depends on the continued success in commercializing our marketed products and obtaining full approval of ARIKAYCE in the US. Our continued success also depends on bringing additional clinical stage products, such as TPIP, INS1148, INS1201, and INS1202, to market, and advancing our pre-clinical research programs. We expect to continue to incur substantial expenses related to our research and development activities as we conduct trials of TPIP in PH-ILD, PAH, PPF, and IPF, and fund development of our clinical and pre-clinical programs. We also expect to continue to incur significant costs related to the commercialization of our marketed products. Our financial results may fluctuate from quarter to quarter and will depend on, among other factors, the net sales of our marketed products; the scope and progress of our research and development efforts; and the timing of certain expenses. We cannot predict whether or when new products or new indications for marketed products will receive regulatory approval or, if any such approval is received, whether we will be able to successfully commercialize such products and whether or when we may become profitable. The information below summarizes our recent updates and anticipated near-term milestones for our marketed products and our product candidates. Respiratory BRINSUPRI •We continue to anticipate a regulatory decision for brensocatib for the treatment of NCFB in Japan in the second half of 2026. •We continue to evaluate the potential effect of evolving US policies which will then impact the timing for future potential international commercial launches. ARIKAYCE •In July 2026, we submitted a US supplemental new drug application (sNDA) for ARIKAYCE in newly diagnosed patients with MAC lung disease. We also plan to review the data with the Pharmaceuticals and Medical Devices Agency (PMDA) in the second half of 2026 to support potential label expansion in Japan. TPIP •PALM-ILD, our Phase 3 study of TPIP in patients with PH-ILD that we initiated in the fourth quarter of 2025, continues to enroll patients. •In April 2026, we initiated the Phase 3 PALM-PAH study of TPIP in patients with PAH and are actively enrolling patients. •In July 2026, we reported positive 12-month data from the ongoing open-label extension study of TPIP in patients with PAH (the OLE Study). See below for additional detail regarding the OLE Study. •We continue to anticipate initiating a Phase 3 study of TPIP in patients with PPF in the second half of 2026 and a Phase 3 study in patients with IPF in the first half of 2027. INS1148 27 Table of Contents •We continue to advance a Phase 2 development program for INS1148, initially targeting PPF and IPF, and we are exploring other diseases where inhibition of the inflammatory functions of SCF248 may be beneficial. Neuro & Other Rare INS1201 •We continue to enroll patients in the Phase 1 ASCEND clinical study of INS1201 for patients with DMD. INS1202 •We continue to enroll patients in the Phase 1 ARMOR clinical study of INS1202 for patients with ALS. Clinical Trial Developments The OLE Study The OLE Study is a non-placebo-controlled trial and was designed to evaluate the long-term safety, tolerability, and effectiveness of TPIP over 24 months in patients who completed the lead-in TPIP PAH studies. In July 2026, we reported positive 12-month data from the ongoing OLE Study. Data for the primary endpoint of safety and tolerability showed that once-daily TPIP therapy was generally well tolerated with no newly identified safety signals at doses up to 1,280 µg through month 12. Of the 91 patients in the OLE Study, treatment-emergent adverse events (TEAEs) occurred in 89.0% of patients; serious TEAEs were observed in 18.7% of patients; and severe TEAEs were observed in 16.5% of patients in the study. TEAEs leading to study discontinuation were experienced by 7.7% of patients. There were four deaths, none of which were considered related to TPIP treatment. The most common TEAEs through month 12 occurring in 5.0% or more of all patients were headache (28.6%), cough (15.4%), nasopharyngitis (14.3%), diarrhea (11.0%), upper respiratory tract infection (9.9%), bronchitis (7.7%), dizziness (6.6%), epistaxis (6.6%), nausea (6.6%), anemia (5.5%), influenza (5.5%), and pneumonia (5.5%). Data for secondary efficacy endpoints demonstrated sustained improvement with TPIP in six-minute walk distance (6MWD), N-terminal fragment pro-B-type natriuretic peptide (NT-proBNP) concentration, and World Health Organization (WHO) Functional Class, as well as a clinically meaningful improvement in REVEAL Lite 2.0 score at month 12. Patients in the Placebo Crossed group (n=31) showed similar outcomes to patients in the TPIP Continued group (n=60) across all efficacy measures at month 12. At month 12, mean improvement from baseline for 6MWD was +55.7 meters for the TPIP Continued group and +54.1 meters for the Placebo Crossed group; NT-proBNP concentration was reduced by approximately 60% in both groups with geometric mean ratios to baseline of 0.40 and 0.41 in TPIP Continued and Placebo Crossed, respectively; WHO Functional Class I or II was achieved in 78.3% of TPIP Continued group and 80.6% of Placebo Crossed group patients, and more than 25% of patients across both groups achieved WHO Functional Class I; and mean REVEAL Lite 2.0 score improved 2.0-points from baseline for the TPIP Continued group and 1.4-points from baseline for the Placebo Crossed group. Approximately 65% of all patients achieved Refined Low Risk status. KEY COMPONENTS OF OUR RESULTS OF OPERATIONS Product Revenues, Net Product revenues, net, consist of net sales of ARIKAYCE and BRINSUPRI. We recognize revenue for product received by our customers net of allowances for customer credits, including prompt pay discounts, service fees, estimated rebates, including government rebates, such as Medicaid rebates and Medicare Part D reimbursements in the US, chargebacks, and co-payment assistance. Cost of Product Revenues (Excluding Amortization of Intangible Assets) Cost of product revenues (excluding amortization of intangible assets) consist primarily of direct and indirect costs related to the manufacturing of ARIKAYCE and BRINSUPRI sold, including third-party manufacturing costs, packaging services, freight, and allocation of overhead costs, in addition to royalty expenses. Research and Development Expenses R&D expenses consist of salaries, benefits and other related costs, including stock-based compensation, for personnel serving in our research and development functions. R&D expenses also include other internal operating expenses, the cost of manufacturing product candidates, including the medical devices for drug delivery, for clinical study, the cost of conducting clinical studies, and the cost of conducting pre-clinical and research activities. In addition, R&D expenses include payments to third parties for the license rights to products in development (prior to marketing approval), and may include the cost of asset 28 Table of Contents acquisitions. Our R&D expenses related to manufacturing our product candidates and medical devices for clinical study are primarily related to activities at CMOs that manufacture our product candidates and early-stage research activities. Our R&D expenses related to clinical trials are primarily related to activities at contract research organizations (CROs) that conduct and manage clinical trials on our behalf. These contracts with CROs set forth the scope of work to be completed at a fixed fee or billed at a per-unit cost, and increase proportionally to the volume of services rendered. Payments under these contracts with CROs primarily depend on performance criteria such as the successful enrollment of patients or the completion of clinical trial milestones as well as time-based fees. Expenses are accrued based on contracted amounts applied to the level of patient enrollment and to activity according to the clinical trial protocol. Deposits for goods or services that will be used or rendered for future research and development activities are deferred and capitalized. Such amounts are then recognized as an expense as the related goods are delivered or the services are performed. Selling, General and Administrative (SG&A) Expenses SG&A expenses consist of salaries, benefits and other related costs, including stock-based compensation, for our non-employee directors and personnel serving in our executive, finance and accounting, legal and compliance, commercial and pre-commercial, corporate development, field sales, information technology and human resource functions. SG&A expenses also include professional fees for legal services, advertising costs, consulting services, including commercial activities, insurance, board of director fees, tax and accounting services. Amortization of Intangible Assets Upon regulatory approval of each of ARIKAYCE and BRINSUPRI, the related intangible assets began to be amortized over their estimated useful lives. The fair values assigned to our intangible assets are based on estimates and assumptions we believe are reasonable based on available facts and circumstances. Unanticipated events or circumstances may occur that require us to review the assets for impairment. Change in Fair Value of Contingent Consideration In connection with the Business Acquisition, we recorded contingent consideration liabilities related to potential future milestone payments. Adjustments to the fair value are due to changes in the probability of achieving milestones, our stock price, or certain other estimated assumptions. The change in fair value of contingent consideration is calculated quarterly with gains and losses recorded in the consolidated statements of comprehensive loss. Investment Income and Interest Expense Investment income consists of interest and dividend income earned on our cash and cash equivalents and marketable securities. Interest expense consists primarily of contractual interest costs, Royalty Financing Agreement non-cash interest expense and the amortization of debt issuance costs. Debt issuance costs are amortized to interest expense using the effective interest rate method over the term of the debt. Our consolidated balance sheets reflect debt, net of the debt issuance costs paid to the lender, and other third-party costs. 29 Table of Contents RESULTS OF OPERATIONS Comparison of the Three Months Ended June 30, 2026 and 2025 Product Revenues, Net Product revenues, net, consists of net sales of ARIKAYCE and BRINSUPRI. The following table summarizes revenue by product and geography for the three months ended June 30, 2026 and 2025 (in thousands): Three Months Ended June 30, Increase (decrease) 2026 2025 $ % ARIKAYCE US $ 70,186 $ 68,683 $ 1,503 2% International 46,130 38,732 7,398 19% Total $ 116,316 $ 107,415 $ 8,901 8% BRINSUPRI US $ 308,555 $ — $ 308,555 NA International 615 — 615 NA Total $ 309,170 $ — $ 309,170 NA Total US $ 378,741 $ 68,683 $ 310,058 451% International 46,745 38,732 8,013 21% Total product revenues, net $ 425,486 $ 107,415 $ 318,071 296% Product revenues, net, for the three months ended June 30, 2026 were $425.5 million as compared to $107.4 million for the same period in 2025, an increase of $318.1 million, or 296%. This increase was a result of $308.6 million of US commercial sales of BRINSUPRI following approval in August 2025 and an $8.9 million growth in sales of ARIKAYCE, primarily driven by growth in international sales. In the first quarter of 2026, we began recognizing international BRINSUPRI revenue related to EAPs in Europe, consisting of sales to the French National Agency for Medicines and Health Products Safety, which has granted BRINSUPRI a Compassionate Access Authorisation (Autorisation d'accès compassionnel or AAC) and sales through the NPP in other countries. Cost of Product Revenues (excluding amortization of intangible assets) Cost of product revenues (excluding amortization of intangible assets) for the three months ended June 30, 2026 and 2025 were as follows (in thousands): Three Months Ended June 30, Increase (decrease) 2026 2025 $ % Cost of product revenues (excluding amortization of intangible assets) $ 67,212 $ 28,075 $ 39,137 139% Cost of product revenues, as % of revenues 15.8 % 26.1 % Cost of product revenues (excluding amortization of intangible assets) were $67.2 million for the three months ended June 30, 2026 as compared to $28.1 million for the same period in 2025, an increase of $39.1 million, or 139%. This increase was primarily attributable to the increase in total product revenues discussed above. Cost of product revenues as a percent of revenues decreased in the current period due to sales of BRINSUPRI, which has lower manufacturing costs than ARIKAYCE. All product costs for BRINSUPRI incurred prior to FDA approval on August 12, 2025 were expensed as R&D expenses. We expect this to benefit our cost of product revenues (excluding amortization of intangible assets) in 2026 and beyond as we sell through inventory that was expensed prior to FDA approval of BRINSUPRI. 30 Table of Contents R&D Expenses R&D expenses for the three months ended June 30, 2026 and 2025 were comprised of the following (in thousands): Three Months Ended June 30, Increase (decrease) 2026 2025 $ % External Expenses Clinical development and research $ 55,897 $ 41,251 $ 14,646 36% Manufacturing 37,841 41,608 (3,767) (9)% Regulatory, quality assurance, and medical affairs 14,090 10,798 3,292 30% Subtotal—external expenses $ 107,828 $ 93,657 $ 14,171 15% Internal Expenses Compensation and benefit-related expenses $ 69,080 $ 56,389 $ 12,691 23% Stock-based compensation 20,292 15,970 4,322 27% Other internal operating expenses 12,834 11,174 1,660 15% Subtotal—internal expenses $ 102,206 $ 83,533 $ 18,673 22% Total R&D expenses $ 210,034 $ 177,190 $ 32,844 19% R&D expenses were $210.0 million for the three months ended June 30, 2026 as compared to $177.2 million for the same period in 2025, an increase of $32.8 million, or 19%. This increase was primarily due to a $17.0 million increase in compensation and benefit-related expenses and stock-based compensation costs due to an increase in headcount, and a $14.6 million increase in clinical development and research costs primarily related to TPIP. External R&D expenses by product for the three months ended June 30, 2026 and 2025 were comprised of the following (in thousands): Three Months Ended June 30, Increase (decrease) 2026 2025 $ % TPIP external R&D expenses $ 38,935 $ 20,649 $ 18,286 89% Brensocatib external R&D expenses 13,829 33,560 (19,731) (59)% ARIKAYCE external R&D expenses 6,228 11,003 (4,775) (43)% Other external R&D expenses 48,836 28,445 20,391 72% Total external R&D expenses $ 107,828 $ 93,657 $ 14,171 15% SG&A Expenses SG&A expenses for the three months ended June 30, 2026 and 2025 were comprised of the following (in thousands): Three Months Ended June 30, Increase (decrease) 2026 2025 $ % Compensation and benefit-related expenses $ 70,520 $ 51,320 $ 19,200 37% Stock-based compensation 20,962 27,006 (6,044) (22)% Professional fees and other external expenses 133,267 56,805 76,462 135% Facility related and other internal expenses 22,718 19,632 3,086 16% Total SG&A expenses $ 247,467 $ 154,763 $ 92,704 60% SG&A expenses were $247.5 million for the three months ended June 30, 2026 as compared to $154.8 million for the same period in 2025, an increase of $92.7 million, or 60%. This increase was primarily due to a $76.5 million increase in professional fees and other external expenses and a $19.2 million increase in compensation and benefit-related expenses due to an increase in headcount, both driven by commercial activities for BRINSUPRI. Amortization of Intangible Assets Amortization of intangible assets was $2.1 million for the three months ended June 30, 2026 as compared to $1.3 million for the same period in 2025, an increase of $0.8 million. This increase was due to amortization of the AstraZeneca milestones achieved upon FDA and EC approvals of BRINSUPRI in August 2025 and November 2025, respectively. 31 Table of Contents Change in Fair Value of Contingent Consideration The change in fair value of contingent consideration for the three months ended June 30, 2026 was $99.8 million and was primarily due to the decrease in our share price and to a lesser extent, the change in probabilities of success. Contingent consideration is the potential future consideration to be paid to former equityholders of the businesses we acquired. Investment Income Investment income was $11.0 million for the three months ended June 30, 2026 as compared to $13.2 million for the same period in 2025, a decrease of $2.2 million, or 17%. This decrease was due to lower average cash and marketable securities balances and lower interest rates in 2026 relative to the same period in 2025. Interest Expense Interest expense for the three months ended June 30, 2026 was $20.3 million as compared to $21.2 million for the same period in 2025, a decrease of $1.0 million, or 5%. This decrease was primarily due to the redemption of the Company's outstanding 0.75% Convertible Senior Notes due 2028 in the second quarter of 2025. See Note 10 - Debt and Note 11 - Royalty Financing Agreement in this Quarterly Report on Form 10-Q for further details. RESULTS OF OPERATIONS Comparison of the Six Months Ended June 30, 2026 and 2025 Product Revenues, Net Product revenues, net, consists of net sales of ARIKAYCE and BRINSUPRI. The following table summarizes revenue by product and geography for the six months ended June 30, 2026 and 2025 (in thousands): Six Months Ended June 30, Increase (decrease) 2026 2025 $ % ARIKAYCE US $ 133,070 $ 132,958 $ 112 <1% International 81,356 67,280 14,076 21% Total $ 214,426 $ 200,238 $ 14,188 7% BRINSUPRI US $ 515,737 $ — $ 515,737 NA International 1,287 — 1,287 NA Total $ 517,024 $ — $ 517,024 NA Total US $ 648,807 $ 132,958 $ 515,849 388% International 82,643 67,280 15,363 23% Total product revenues, net $ 731,450 $ 200,238 $ 531,212 265% Product revenues, net, for the six months ended June 30, 2026 were $731.5 million as compared to $200.2 million for the same period in 2025, an increase of $531.2 million, or 265%. This increase was a result of $515.7 million of US commercial sales of BRINSUPRI following approval in August 2025 and a $14.2 million growth in sales of ARIKAYCE, primarily driven by growth in international sales. Cost of Product Revenues (excluding amortization of intangible assets) Cost of product revenues (excluding amortization of intangible assets) for the six months ended June 30, 2026 and 2025 were as follows (in thousands): Six Months Ended June 30, Increase (decrease) 2026 2025 $ % Cost of product revenues (excluding amortization of intangible assets) $ 114,632 $ 49,353 $ 65,279 132% Cost of product revenues, as % of revenues 15.7 % 24.6 % Cost of product revenues (excluding amortization of intangible assets) were $114.6 million for the six months ended June 30, 2026 as compared to $49.4 million for the same period in 2025, an increase of $65.3 million, or 132%. This increase 32 Table of Contents was primarily attributable to the increase in total product revenues discussed above. Cost of product revenues as a percent of revenues decreased in the current period due to sales of BRINSUPRI, which has lower manufacturing costs than ARIKAYCE. R&D Expenses R&D expenses for the six months ended June 30, 2026 and 2025 were comprised of the following (in thousands): Six Months Ended June 30, Increase (decrease) 2026 2025 $ % External Expenses Clinical development and research $ 103,853 $ 81,788 $ 22,065 27% Manufacturing 75,977 63,417 12,560 20% Regulatory, quality assurance, and medical affairs 25,123 18,470 6,653 36% Subtotal—external expenses $ 204,953 $ 163,675 $ 41,278 25% Internal Expenses Compensation and benefit-related expenses $ 141,220 $ 109,947 $ 31,273 28% Stock-based compensation 44,286 33,350 10,936 33% Other internal operating expenses 29,060 22,795 6,265 27% Subtotal—internal expenses $ 214,566 $ 166,092 $ 48,474 29% Total R&D expenses $ 419,519 $ 329,767 $ 89,752 27% R&D expenses were $419.5 million for the six months ended June 30, 2026 as compared to $329.8 million for the same period in 2025, an increase of $89.8 million, or 27%. This increase was primarily due to a $42.2 million increase in compensation and benefit-related expenses and stock-based compensation costs due to an increase in headcount, and a $22.1 million increase in clinical development and research costs and a $12.6 million increase in manufacturing costs, both primarily related to TPIP. External R&D expenses by product for the six months ended June 30, 2026 and 2025 were comprised of the following (in thousands): Six Months Ended June 30, Increase (decrease) 2026 2025 $ % TPIP external R&D expenses $ 76,276 $ 29,802 $ 46,474 156% Brensocatib external R&D expenses 32,313 54,221 (21,908) (40)% ARIKAYCE external R&D expenses 14,850 23,124 (8,274) (36)% Other external R&D expenses 81,514 56,528 24,986 44% Total external R&D expenses $ 204,953 $ 163,675 $ 41,278 25% SG&A Expenses SG&A expenses for the six months ended June 30, 2026 and 2025 were comprised of the following (in thousands): Six Months Ended June 30, Increase (decrease) 2026 2025 $ % Compensation and benefit-related expenses $ 145,313 $ 106,156 $ 39,157 37% Stock-based compensation 42,722 48,888 (6,166) (13)% Professional fees and other external expenses 258,231 107,366 150,865 141% Facility related and other internal expenses 48,460 39,898 8,562 21% Total SG&A expenses $ 494,726 $ 302,308 $ 192,418 64% SG&A expenses were $494.7 million for the six months ended June 30, 2026 as compared to $302.3 million for the same period in 2025, an increase of $192.4 million, or 64%. This increase was primarily due to a $150.9 million increase in professional fees and other external expenses and a $39.2 million increase in compensation and benefit-related expenses due to an increase in headcount, both driven by commercial activities for BRINSUPRI. 33 Table of Contents Amortization of Intangible Assets Amortization of intangible assets was $4.2 million for the six months ended June 30, 2026 as compared to $2.5 million for the same period in 2025, an increase of $1.6 million. This increase was due to amortization of the AstraZeneca milestones achieved upon FDA and EC approvals of BRINSUPRI in August 2025 and November 2025, respectively. Change in Fair Value of Contingent Consideration The change in fair value of contingent consideration for the six months ended June 30, 2026 was $146.7 million and was primarily due to the decrease in our share price and to a lesser extent, the change in probabilities of success, partially offset by an increase in the obligation associated with the reauthorization of the PRV program. Investment Income Investment income was $23.0 million for the six months ended June 30, 2026 as compared to $27.1 million for the same period in 2025, a decrease of $4.1 million, or 15%. This decrease was due to lower average cash and marketable securities balances and lower interest rates in 2026 relative to the same period in 2025. Interest Expense Interest expense for the six months ended June 30, 2026 was $40.4 million as compared to $42.8 million for the same period in 2025, a decrease of $2.5 million, or 6%. This decrease was primarily due to the redemption of the Company's outstanding 0.75% Convertible Senior Notes due 2028 in the second quarter of 2025. See Note 10 - Debt and Note 11 - Royalty Financing Agreement in this Quarterly Report on Form 10-Q for further details. LIQUIDITY AND CAPITAL RESOURCES Overview There is considerable time and cost associated with developing potential pharmaceutical products to the point of regulatory approval and commercialization. We commenced commercial shipments of ARIKAYCE in October 2018 and BRINSUPRI in August 2025. We expect to continue to incur consolidated operating losses, including losses at our US and certain international entities, as we plan to fund R&D for ARIKAYCE, TPIP, INS1148, INS1201, INS1202, and our other pipeline programs, continue commercialization and regulatory activities for ARIKAYCE and BRINSUPRI, and engage in other general and administrative activities. In June 2025, we completed an underwritten offering of 8,984,375 shares of our common stock at a public offering price of $96.00 per share. 1,171,875 of the shares of common stock were issued pursuant to the exercise in full of the underwriters' option to purchase additional shares. Our net proceeds from the sale of the shares, after deducting the underwriting discounts and offering expenses of $39.2 million, were $823.3 million. Based on our current operating plan, we anticipate that our cash and cash equivalents and marketable securities as of June 30, 2026 will enable us to fund our operations. While we believe we currently have sufficient funds to meet our financial needs for at least the next 12 months, we may raise additional capital to fund future development of our product candidates, and to develop, acquire, in-license or co-promote other products or product candidates, including those that address serious diseases with significant unmet need. Our cash requirements for the next 12 months will be impacted by a number of factors, the most significant of which we expect to be expenses related to our commercialization efforts for ARIKAYCE and BRINSUPRI and development costs for our clinical-stage assets and, to a lesser extent, our pre-clinical research programs. Cash Flows As of June 30, 2026, we had cash and cash equivalents of $544.8 million, as compared to $510.4 million as of December 31, 2025. In addition, as of June 30, 2026, we had marketable securities of $615.5 million, as compared to $919.6 million as of December 31, 2025. The net decrease in cash and cash equivalents and marketable securities was primarily due to the cash used in operating activities and the $15.0 million milestone payment to AstraZeneca following EC approval of BRINSUPRI, partially offset by proceeds from the exercise of stock options. Our working capital was $1.2 billion and $1.3 billion as of June 30, 2026 and December 31, 2025, respectively. Net cash used in operating activities was $311.6 million and $467.7 million for the six months ended June 30, 2026 and 2025, respectively. The net cash used in operating activities during the six months ended June 30, 2026 and 2025 was primarily driven by commercial, clinical, and manufacturing activities related to ARIKAYCE, commercial and commercial readiness activities for BRINSUPRI, as well as other SG&A expenses, and clinical trial expenses related to brensocatib and TPIP. The decrease in cash used in operating activities for the six months ended June 30, 2026 as compared to the same period in 2025 was primarily due to the decrease in net loss, excluding the adjustments to reconcile net loss to net cash used in operating activities. 34 Table of Contents Net cash provided by investing activities was $290.0 million and $308.2 million for the six months ended June 30, 2026 and 2025, respectively. During the six months ended June 30, 2026, net cash provided by investing activities consisted primarily of maturities of marketable securities, partially offset by purchases of marketable securities and the $15.0 million milestone payment to AstraZeneca following EC approval of BRINSUPRI. During the six months ended June 30, 2025, net cash provided by investing activities consisted primarily of maturities of marketable securities, partially offset by purchases of marketable securities. Net cash provided by financing activities was $56.7 million and $886.8 million for the six months ended June 30, 2026 and 2025, respectively. During the six months ended June 30, 2026, net cash provided by financing activities consisted primarily of proceeds from the exercise of stock options and ESPP. During the six months ended June 30, 2025, net cash provided by financing activities consisted primarily of net proceeds from the issuance of common stock and proceeds from the exercise of stock options and ESPP. Contractual Obligations There were no material changes outside of the ordinary course of business in our contractual obligations during the six months ended June 30, 2026 from those disclosed in Item 7, “Management’s Discussion and Analysis of Financial Condition and Results of Operations—Liquidity and Capital Resources—Contractual Obligations” in our Annual Report on Form 10-K for the year ended December 31, 2025. Off-Balance Sheet Arrangements We do not have any off-balance sheet arrangements that have or are reasonably likely to have a current or future material effect on our financial condition, revenues or expenses, results of operations, liquidity, capital expenditures or capital resources. We do not have any interest in special purpose entities, structured finance entities or other variable interest entities. CRITICAL ACCOUNTING ESTIMATES There have been no material changes to our critical accounting policies and estimates as disclosed in our Annual Report on Form 10-K for the year ended December 31, 2025. For the required interim disclosure updates related to our accounting policies and estimates, see Note 2 - Summary of Significant Accounting Policies in this Quarterly Report on Form 10-Q.