News Releases

--ARIKAYCE Plus Guideline-Based Therapy (GBT) Associated with Significantly Higher Rates of Sustained Culture Conversion on Therapy and Durable Culture Conversion Three Months Post-Treatment Compared with GBT Alone--

-- ARIKAYCE® (amikacin liposome inhalation suspension) U.S. Net Product Sales $21.0 Million for the First Quarter of 2019 --

--Patent is 10th Issued in the U.S. for ARIKAYCE and Second Extending Patent Protection into 2035--

--ARIKAYCE Preliminary U.S. Net Product Sales Approximately $21.0 Million for the First Quarter of 2019--

--Sustainability and Durability Data from Phase 3 CONVERT Study of ARIKAYCE to be Presented in Late-Breaking Oral Session--

--ARIKAYCE® (amikacin liposome inhalation suspension) U.S. Net Product Sales of $9.2 Million for the Fourth Quarter and Full Year 2018--

-ARIKAYCE Preliminary Unaudited U.S. Net Product Sales Approximately $9.2 Million for the Fourth Quarter of 2018-

—Seasoned Pharmaceutical Executive Elizabeth McKee Anderson Appointed as New Director—

—Quarter Highlighted by U.S. FDA Approval of ARIKAYCE® (amikacin liposome inhalation suspension), Followed by Immediate Launch—

New Patent Adds 7 ½ Years of Market Exclusivity in Japan

Commercial availability expected in early Q4 2018

As previously reported, study met primary endpoint of culture conversion (p<0.0001)

Advisory committee supports surrogate endpoint of sputum culture conversion

PDUFA (Prescription Drug User Fee Act) Action Date for ALIS (Amikacin Liposome Inhalation Suspension) Set for September 28, 2018

Meeting planned for August 7, 2018

Data support the benefit of treating patients with a combination of ALIS (Amikacin Liposome Inhalation Suspension) and guideline-based therapy (GBT) for the treatment of nontuberculous mycobacterial (NTM) lung disease caused by Mycobacterium avium complex (MAC)

PDUFA Action Date Set for September 28, 2018

Submitted NDA to FDA for Amikacin Liposome Inhalation Suspension (ALIS) in NTM lung disease caused by MAC; anticipates priority review

ALIS previously designated as an orphan drug, breakthrough therapy and Qualified Infectious Disease Product (QIDP)

On track to file NDA before the end of March and anticipate a priority review

Interim results from INS-312 show that 28% of patients who previously received 6 months of guideline based therapy (GBT) alone achieved culture conversion following addition of ALIS to therapy, consistent with data from the INS-212 study