Insmed Reports Fourth Quarter and Full

Insmed Reports Fourth Quarter and Full

Year 2019 Financial Results and Provides Business Update

liposome inhalation suspension) Total Revenue of $45.7 Million for the Fourth Quarter 2019 and $136.5 Million for the Full Year

-Company Provides Full-Year

2020 ARIKAYCE Revenue Guidance of $180 Million to $220 Million-

-Phase 2 WILLOW Study

of INS1007 in Patients with Non-Cystic Fibrosis Bronchiectasis Achieves Primary and Key Secondary Endpoint-

BRIDGEWATER, N.J., February 25, 2020 /PRNewswire/ - Insmed

Incorporated (Nasdaq:INSM), a global biopharmaceutical company on a mission to transform the lives of patients with serious and

rare diseases, today reported financial results for the fourth quarter and full year ended December 31, 2019 and provided a business

"2019 was a transformative year for Insmed as we evolved

into a commercial-stage organization and advanced our global infrastructure. I am incredibly proud of our team's performance

in the first full year of the ARIKAYCE U.S. launch, and we look forward to serving even more patients with the same level of dedication

as we prepare for a potential approval and commercial launch in Europe and regulatory filings in Japan," commented Will

Lewis, Chairman and Chief Executive Officer of Insmed. "With our recent announcement of positive top-line Phase 2 data for

INS1007 in non-cystic fibrosis bronchiectasis, in addition to other meaningful advancements in our pipeline, we are well on our

way toward building a robust portfolio of therapies that address the unmet needs of small patient populations experiencing big

Fourth Quarter and Full-Year 2019 Financial Results

Recent Corporate Developments & Program Highlights

Positive Top-Line Results from WILLOW Study

On February 3, 2020, Insmed announced positive top-line results

from the Phase 2 WILLOW study evaluating the efficacy, safety, and pharmacokinetics of INS1007 in adults with non-cystic fibrosis

bronchiectasis. The study met both its primary endpoint of time to first pulmonary exacerbation over the 24-week treatment period

as well as a key secondary endpoint of reduction in the frequency of pulmonary exacerbations versus placebo. The Company plans

to advance INS1007 to Phase 3 development.

ARIKAYCE Lifecycle Management

Insmed continues to advance the post-approval confirmatory

clinical trial for ARIKAYCE in a front-line setting of patients with Mycobacterium avium complex (MAC) lung disease as

well as the development of an appropriate patient reported outcome (PRO) tool that will enable the assessment of therapies for

the treatment of nontuberculous mycobacterial (NTM) lung disease. Insmed plans to initiate both the confirmatory study and a study

to validate the PRO in the second half of 2020 and to run these studies in parallel, pending alignment with the FDA. The Company

also plans to advance into a separate registrational Phase 3 study in patients with NTM lung disease caused by Mycobacterium

Insmed is advancing INS1009, a dry powder, inhaled treprostinil

prodrug formulation, for the potential treatment of pulmonary arterial hypertension. The Company plans to file an Investigational

New Drug application and initiate a Phase 1 study of INS1009 this year.

Pending the approval of our marketing authorization application

in Europe, Insmed anticipates a potential launch of ARIKAYCE in Germany by the end of 2020, followed shortly thereafter by the

UK. The Company plans to file for approval of ARIKAYCE in Japan in the first quarter of 2020. In addition, Insmed has engaged

DKSH Korea Ltd. to provide services to support an expanded access program on a named patient basis in South Korea.

Recent senior leadership appointments include Fred Zussa, Senior

Vice President, Business Development, and Anjan Chatterjee, MD, MBA, MPH, Senior Vice President, Medical Affairs. Fred joins the

Company from Celgene Corporation, where he was a senior member of the Corporate Strategy and Development group. He is responsible

for sourcing, evaluating, and transacting business development activities for Insmed. Anjan joins Insmed from Boehringer

Ingelheim, where he most recently served as Corporate Vice President. He is responsible for expanding and leading the global Medical

Affairs function for ARIKAYCE as well as for our pipeline candidates as they advance toward late-stage development.

Financial Guidance and Balance Sheet

As of December 31, 2019, Insmed had cash and cash

equivalents of $487.4 million. The Company's total operating expenses for the fourth quarter of 2019 were $84.1 million and

for the full year of 2019 were $347.5 million. Adjusted operating expenses, as defined below, for the fourth quarter of 2019 were

$75.0 million and for the full year 2019 were $300.1 million.

The Company expects full-year 2020 revenues for ARIKAYCE to

be in the range of $180 million to $220 million.

The Company plans to invest in the following key activities

As a result of these activities, Insmed expects adjusted

operating expenses to be in the range of $340 million to $360 million for 2020.

Insmed will host a conference call beginning

today at 8:30 AM Eastern Time. Shareholders and other interested parties may participate in the conference call by dialing

(888) 317-6003 (domestic) or (412) 317-6061 (international) and referencing conference ID number 3317351. The call will also be

webcast live on the company's website at www.insmed.com.

of the conference call will be accessible approximately one hour after its completion through March 3, 2020 by

dialing (877) 344-7529 (domestic) or (412) 317-0088 (international) and referencing replay access code 10139238. A webcast of

the call will also be archived for 90 days under the Investors section of the Company's website at www.insmed.com.

to the U.S. generally accepted accounting principles (GAAP) results, this earnings release includes adjusted operating

expenses, a non-GAAP financial measure, which Insmed defines as total operating expenses less stock-based compensation

expense, depreciation, amortization of intangibles and certain milestones related to ARIKAYCE, which is payable under our

amended agreements with CFFT. A reconciliation of this non-GAAP financial measure to its most directly

comparable GAAP financial measure is presented in the table attached to this press release.

that this non-GAAP financial measure is useful to both management and investors in analyzing our ongoing business and operating

performance. Management believes that providing this non-GAAP information to investors, in addition to the GAAP results, allows

investors to view our financial results in the way that management views financial results. Management does not intend the

presentation of this non-GAAP financial measure to be considered in isolation or as a substitute for results prepared in accordance

with GAAP. In addition, this non-GAAP financial measure may differ from similarly named measures used by other companies.

About ARIKAYCE (amikacin liposome inhalation

ARIKAYCE is the first and only FDA-approved therapy indicated

for the treatment of Mycobacterium avium complex (MAC) lung disease as part of a combination antibacterial drug

regimen for adult patients with limited or no alternative treatment options. ARIKAYCE is a novel, inhaled, once-daily formulation

of amikacin, an established antibiotic that was historically administered intravenously and associated with severe toxicity to

hearing, balance, and kidney function. Insmed's proprietary PULMOVANCE liposomal technology enables the delivery of amikacin

directly to the lungs, where liposomal amikacin is taken up by lung macrophages where the infection resides. This approach prolongs

the release of amikacin in the lungs while limiting systemic exposure. ARIKAYCE is administered once daily using the Lamira Nebulizer

System manufactured by PARI Pharma GmbH (PARI).

About PARI Pharma and the Lamira Nebulizer

liposome inhalation suspension) is delivered by a novel inhalation device, the Lamira Nebulizer System,

developed by PARI. Lamira is a quiet, portable nebulizer that enables efficient aerosolization of liquid

medications, including liposomal formulations such as ARIKAYCE, via a vibrating, perforated membrane. Based on PARI's 100-year

history working with aerosols, PARI is dedicated to advancing inhalation therapies by developing innovative delivery platforms

and new pharmaceutical formulations that work together to improve patient care.

INS1007 is a small molecule, oral, reversible inhibitor of

dipeptidyl peptidase I (DPP1) being developed by Insmed for the treatment of patients with bronchiectasis. DPP1 is an enzyme responsible

for activating neutrophil serine proteases (NSPs), such as neutrophil elastase, in neutrophils when they are formed in the bone

marrow. Neutrophils are the most common type of white blood cell and play an essential role in pathogen destruction and inflammatory

In chronic inflammatory lung diseases, neutrophils accumulate

in the airways and result in excessive active NSPs that cause lung destruction and inflammation. INS1007 may decrease the damaging

effects of inflammatory diseases such as bronchiectasis by inhibiting DPP1 and its activation of NSPs.

IMPORTANT SAFETY INFORMATION FOR

Hypersensitivity Pneumonitis has

been reported with the use of ARIKAYCE in the clinical trials. Hypersensitivity pneumonitis (reported as allergic alveolitis,

pneumonitis, interstitial lung disease, allergic reaction to ARIKAYCE) was reported at a higher frequency in patients treated

with ARIKAYCE plus background regimen (3.1%) compared to patients treated with a background regimen alone (0%). Most patients