Leiden, the Netherlands – 23 January 2019

Pharming Group N.V. (“Pharming” or “the Company”) (Euronext Amsterdam: PHARM) announced today that two abstracts were presented at The Western Society of Allergy, Asthma and Immunology (WSAAI) in Maui, Hawaii, USA on January 22-24, 2019.

These presentations are:
•Impact of Hereditary Angioedema Prophylaxis With Recombinant Human C1 Esterase Inhibitor on Burden of Emergency Department Visits by Dr. Douglas Jones, Rocky Mountain Allergy, Asthma and Immunology.
?The authors report a patient on plasma-derived C1-INH prophylaxis who was still experiencing HAE attacks and required numerous visits to the emergency department (ED). The patient was switched to RUCONEST® prophylaxis, and the number of attacks requiring acute treatment decreased significantly (1 attack over a 15 month period) along with visits to the ED.
•Recombinant Human C1 Esterase Inhibitor as Routine Short-Term Prophylaxis for Hereditary Angioedema Inadequately Controlled With Long-Term Prophylaxis During Menses by Dr. Andrew Smith, Allergy Associates of Utah.
?The authors report a patient suboptimally controlled with plasma-derived C1-INH prophylaxis, initially delivered intravenously and then switched to the subcutaneous route of administration. Because of frequent breakthrough attacks especially during menses, RUCONEST® was added to her regimen as a short-term prophylactic therapy. This resulted in a substantial reduction of her HAE symptoms, and the patient did not require any acute therapy since beginning Ruconest.

“This real world evidence demonstrates that RUCONEST® continues to serve an important role in the management of HAE despite the availability of other therapeutic options.” said Bruno Giannetti, MD, PhD COO of Pharming.

These presentations will be made available following the congress on Pharming’s website www.pharming.com.

About HAE

Hereditary Angioedema (HAE) is a rare genetic disorder. It is characterized by spontaneous and recurrent episodes of swelling (edema attacks) of the skin in different parts of the body, as well as in the airways and internal organs. Edema of the skin usually affects the extremities, the face, and the genitals. Patients suffering from this kind of edema often withdraw from their social lives because of the disfiguration, discomfort and pain these symptoms may cause. Almost all HAE patients suffer from bouts of severe abdominal pain, nausea, vomiting and diarrhea caused by swelling of the intestinal wall.

Edema of the throat, nose or tongue can be particularly dangerous as this can lead to obstruction of the airway passages and be potentially life threatening. Although there is currently no known cure for HAE, it is possible to treat the symptoms associated with edema attacks. HAE affects about 1 in 10,000 to 1 in 50,000 people, worldwide experts believe that a lot of patients are still seeking the right diagnosis: although HAE is (in principle) easy to diagnose, it is frequently identified very late or not discovered at all. The reason HAE is often misdiagnosed is because the symptoms are similar to those of many other common conditions such as allergies or appendicitis by the time it is diagnosed correctly, the patient has often been through a long-lasting ordeal.

About RUCONEST®

US INDICATION

RUCONEST® (C1 esterase inhibitor [recombinant]) is indicated for the treatment of acute attacks in adult and adolescent patients with hereditary angioedema (“HAE”). Effectiveness in clinical studies was not established in HAE patients with laryngeal attacks.

IMPORTANT SAFETY INFORMATION
•RUCONEST® (C1 esterase inhibitor [recombinant]) is contraindicated in: ?Patients with a history of allergy to rabbits or rabbit-derived products.
?Patients with a history of life-threatening immediate hypersensitivity reactions to C1 esterase inhibitor preparations, including anaphylaxis.

•Hypersensitivity: Severe hypersensitivity reactions may occur. Should symptoms occur, discontinue RUCONEST and administer appropriate treatment. Because hypersensitivity reactions may have symptoms similar to HAE attacks, treatment methods should be carefully considered.
•Thromboembolic Events: Serious arterial and venous thromboembolic (TE) events have been reported at the recommended dose of plasma-derived C1 esterase inhibitor products in patients with risk factors. Risk factors may include the presence of an indwelling venous catheter/access device, prior history of thrombosis, underlying atherosclerosis, use of oral contraceptives or certain androgens, morbid obesity, and immobility. Monitor patients with known risk factors for TE events during and after RUCONEST administration.
•Intravenous Use: RUCONEST is for intravenous use after reconstitution only. No more than 2 doses should be administered within a 24-hour period.
•Pregnancy and Nursing: RUCONEST has not been studied in pregnant women; therefore, it should only be used during pregnancy if clearly needed. Advise patients to notify their physician if they are breastfeeding or plan to breastfeed.
•Adverse reactions: The serious adverse reaction in clinical studies of RUCONEST was anaphylaxis.
•Common adverse reactions: The most common adverse reactions (incidence ?2%) were headache, nausea, and diarrhoea.

Please see Full Prescribing Information for RUCONEST® as applicable for various jurisdictions:

FDA: RUCONEST®[1] / EMA: RUCONEST®[2]

About Pharming Group N.V.

Pharming is a specialty pharmaceutical company developing innovative products for the safe, effective treatment of rare diseases and unmet medical needs. Pharming’s lead product, RUCONEST® (conestat alfa) is a recombinant human C1 esterase inhibitor approved for the treatment of acute Hereditary Angioedema (“HAE”) attacks in patients in Europe, the US, Israel and South Korea. The product is available on a named-patient basis in other territories where it has not yet obtained marketing authorization.

RUCONEST® is distributed by Pharming in Austria, France, Germany, Luxembourg, the Netherlands, the United Kingdom and the United States of America. Pharming holds commercialisation rights in Algeria, Andorra, Bahrain, Belgium, Ireland, Jordan, Kuwait, Lebanon, Morocco, Oman, Portugal, Qatar, Syria, Spain, Switzerland, Tunisia, United Arab Emirates and Yemen. In some of these countries distribution is made in association with the HAEi Global Access Program (GAP).

RUCONEST® is distributed by Swedish Orphan Biovitrum AB (publ) (SS: SOBI) in the other EU countries, and in Azerbaijan, Belarus, Georgia, Iceland, Kazakhstan, Liechtenstein, Norway, Russia, Serbia and Ukraine.

RUCONEST® is distributed in Argentina, Colombia, Costa Rica, the Dominican Republic, Panama, and Venezuela by Cytobioteck, in South Korea by HyupJin Corporation and in Israel by Megapharm.

RUCONEST® is also being investigated in a Phase II clinical trial for the treatment of HAE in young children (2-13 years of age) and evaluated for various additional follow-on indications.

Pharming’s technology platform includes a unique, GMP-compliant, validated process for the production of pure recombinant human proteins that has proven capable of producing industrial quantities of high quality recombinant human proteins in a more economical and less immunogenetic way compared with current cell-line based methods. Leads for enzyme replacement therapy (“ERT”) for Pompé and Fabry’s diseases are being optimized at present, with additional programs not involving ERT also being explored at an early stage at present.

Pharming has a long-term partnership with the China State Institute of Pharmaceutical Industry (“CSIPI”), a Sinopharm company, for joint global development of new products, starting with recombinant human Factor VIII for the treatment of Haemophilia A. Pre-clinical development and manufacturing will take place to global standards at CSIPI and are funded by CSIPI. Clinical development will be shared between the partners with each partner taking the costs for their territories under the partnership.

Additional information is available on the Pharming website: www.pharming.com

Forward-looking Statements

This press release of Pharming Group N.V. and its subsidiaries (“Pharming”, the “Company” or the “Group”) may contain forward-looking statements including without limitation those regarding Pharming’s financial projections, market expectations, developments, partnerships, plans, strategies and capital expenditures. Etc. etc..