Shire to Present New Data on the Treatment of Primary Immunodeficiency

• Research to be presented at IPIC 2017 Annual Scientific Meeting, reinforcing Shire’s commitment to help patients living with primary immunodeficiency

Zug, Switzerland – November 6, 2017 – Shire plc (LSE: SHP, NASDAQ: SHPG), the global leader in serving patients with rare diseases, announced today new data from the company’s primary immunodeficiency (PI) portfolio will be presented at the 2017 International Primary Immunodeficiency Congress in Dubai, U.A.E., November 8 - 10.

“The data presented at IPIC provide important information on the treatment of patients with PI, and help enhance the clinical understanding of patient experiences with different medications and different routes of administration over both the short- and long-term,” said Wolfram Nothaft, Head of Global Clinical Development ad interim, Shire. “Shire’s strong presence at IPIC is a reflection of our deep commitment to advancing research and treatment for patients with PI, a condition with the potential for debilitating impact.”

Primary Immunodeficiencies (PI) are a group of more than 300 disorders in which part of the body's immune system is missing or does not function properly. Normally, the immune system protects the body from pathogenic microorganisms like bacteria, viruses, protozoa and fungi, which can cause infectious diseases. When any part of a person's immune system is absent or dysfunctional, the individuals can be susceptible to infections, and it may take longer to recover from these infections.¹ When a defect in the immune system is inherited and genetically determined, it is called primary immune deficiency.¹ It is estimated that as many as six million children and adults may be affected by PI worldwide.²

Key Shire data presentations at IPIC 2017 include:

• PI Research
  • Analyses of efficacy and tolerability in patients with primary immunodeficiency diseases treated by different modes of administration of immunoglobulin therapy during three consecutive studies (Poster 25)
• Immune Globulin Infusion 10% (Human) with Recombinant Human Hyaluronidase Solution Research
  • Overall and pediatric patient preferences for recombinant human hyaluronidase (rhuph20)-facilitated subcutaneous infusion of immunoglobulin G (FSCIG) in patients with primary immunodeficiencies (Poster 24)
  • Long-term safety of hyaluronidase-facilitated subcutaneous immune globulin 10% in patients with primary immunodeficiencies in the United States: interim results of a post-authorization safety study (Poster 44)
  • SENEQA: study on the utilisation of Hyqvia®▼ (10% normal immunoglobulin and recombinant human hyaluronidase) in elderly patients (Poster 165)
• Immune Globulin Subcutaneous (Human), 20% Solution Research
  • Adverse events and tolerability of the human subcutaneous immunoglobulin 20% in pediatric patients (<18 years old) with primary immunodeficiency diseases (Poster 42)
  • Treatment preference on the subcutaneous immunoglobulin 20% treatment in patients with primary immunodeficiency diseases in Europe (Poster 71)
  • Treatment satisfaction during pivotal clinical trials with the subcutaneous immunoglobulin 20% in patients with primary immunodeficiency diseases previously treated with intravenous immunoglobulin (Poster 72)
  • The onboarding experience and tolerability of the 20% human immune globulin for subcutaneous administration in North America (Poster 80)

The poster presentations are intended for scientific discussion only.

Shire will also host booth #3 in the exhibition hall, as well as a symposium, “Expert Insights on Shared Decision-Making in the Treatment of Primary Immunodeficiency Diseases (PIDD),” on Friday, November 10th at 7:45 a.m. local time.

About the Immunology Franchise

Shire’s immunology franchise has a strong legacy in developing therapies for people living with hereditary angioedema and primary immunodeficiency. Our broad portfolio includes commercial products and investigational therapies. We are committed to serial innovation and rely on our expertise to help fulfill unmet treatment needs for patients living with these diseases. Beyond our focus on developing novel treatments, we provide specialized services and support offerings that help meet the needs of our patients.

SHIRE and the Shire Logo are registered trademarks of Shire Pharmaceutical Holdings Ireland Limited or its affiliates.

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NOTES TO EDITORS

About Shire

Shire is the global leader in serving patients with rare diseases. We strive to develop best-in-class therapies across a core of rare disease areas including hematology, immunology, genetic diseases, neuroscience, and internal medicine with growing therapeutic areas in ophthalmics and oncology. Our diversified capabilities enable us to reach patients in more than 100 countries who are struggling to live their lives to the fullest.

We feel a strong sense of urgency to address unmet medical needs and work tirelessly to improve people’s lives with medicines that have a meaningful impact on patients and all who support them on their journey.

www.shire.com

¹ Blaese RM, Bonilla FA, Stiehm ER, Younger ME, eds. Patient & Family Handbook for Primary Immunodeficiency Diseases. 5th ed. Towson, MD: Immune Deficiency Foundation; 2015.
² Bousfiha AA et al. Primary immunodeficiency diseases worldwide: more common than generally thought. J Clin Immunol. 2013 Jan;33(1):1-7.