Angioedema Program

Evaluation and Management of Angioedema (Swelling)

Angioedema refers to unexplained swelling that may involve any part of the body. Patients may experience angioedema alone or a combination of angioedema and hives (urticaria). There are a variety of different reasons for symptoms of swelling including:

• Hereditary angioedema with and without C1 inhibitor deficiency
• Acquired angioedema with C1 inhibitor deficiency
• Histamine and non-histamine mediated angioedema
• Associated with a known or allergic cause (e.g. drug allergy including ACE inhibitor, food allergy)
• Idiopathic (i.e. of unknown cause)

Every angioedema patient's case is unique, so we tailor our diagnostic and therapeutic approaches based on your symptoms and medical history. Our goal is to identify the underlying cause of your angioedema, including what triggers it (when possible), and help you avoid these triggers. In some cases, there is no identifiable trigger, and some forms of angioedema may require lifelong medical management but our team will support you in the best way possible.

What to Expect at Your Initial Appointment

Your first appointment will be with one of our expert allergists. We offer virtual consultations as well. Your allergist will conduct an in-depth consultation that includes an evaluation of your medical history, including exposure to potential triggers, response to treatments previously used, review of prior laboratory testing and a physical examination.

If hereditary angioedema is suspected, we will recommend blood tests for further evaluation. If food allergies are a possible cause of your angioedema, we will schedule food-allergy skin testing.

In many patients who are diagnosed with angioedema, the exact cause may not ultimately be identified. Patients with mild symptoms may not need any treatment. Those with more severe symptoms may benefit from medications for symptom relief. Your doctor will review the results of your blood tests (if applicable) and work with you to determine the most appropriate treatment regimen at your follow-up visit.

A Focus on Treating Hereditary Angioedema at Mass General

Hereditary angioedema, a rare and sometimes life-threatening condition, can be triggered by stress, trauma and certain medications. In many cases, however, the trigger is not clear. The condition does not occur with hives.

Hereditary angioedema is often misdiagnosed as appendicitis or another abdominal disorder. As a result, some patients are treated for the wrong condition—and sometimes undergo unnecessary medical procedures, such as an appendectomy or abdominal surgery.

Our clinical and research experience means we are skilled in:

• Recognizing hereditary angioedema and other forms of angioedema
• Recommending the appropriate treatment

If you have a diagnosis of hereditary angioedema, Dr. Aleena Banerji (one of the few angioedema specialists in the New England area and nationally recognized in the field) will play an important role in your care.

Acquired Angioedema with C1 inhibitor deficiency

Patients with acquired C1 inhibitor deficiency can present with recurrent episodes of swelling. Acquired C1 inhibitor deficiency is a very rare condition that can be associated with lymphoma or other autoimmune conditions. It can diagnosed by clinical history and blood tests. The main goal of therapy is to understand the cause of your symptoms and develop the best management plan

Idiopathic Angioedema and Non-Histaminergic Angioedema

Idiopathic angioedema and non-histaminergic angioedema represent a wide spectrum of disease with varying degrees of severity and chronicity. For patients suspected of having idiopathic angioedema, we will complete a thorough evaluation to identify a cause and try different therapies in search of an effective treatment plan. Antihistamines may provide symptom relief in some patients with idiopathic angioedema.

Non-histaminergic angioedema remains a very rare disease for which there are no available laboratory tests to clearly define the disorder. Therapy is often difficult and various treatment options have been used which will be discussed with you as part of your care.

About This Program

Expertise in Diagnosis & Treatment

The Angioedema Program, led by Aleena Baneriji, MD, offers a full range of services at our downtown Boston campus and at Mass General Brigham Healthcare Center (Waltham).

The treatment options for hereditary angioedema have improved significantly in the past decade. The Mass General Allergy Unit has participated in the study of several important novel therapies for treating hereditary angioedema, including:

• A bradykinin receptor antagonist
• C1-inhibitor replacement therapy
• Kallikrein inhibitor therapy

We continue to actively participate in ongoing clinical trials of novel therapies for patients with hereditary angioedema and enroll patients regularly. Our group's experience with these new medications leaves us uniquely skilled in determining the best way to manage your symptoms if you have a diagnosis of hereditary angioedema we focus on building a strong patient-physician relationship focused on individualized care with the goal of improving quality of life and decreasing burden of disease.

Leadership in the Angioedema Field

Aleena Banerji, MD, an Associate Professor in our group specializing in angioedema, is known nationally for her clinical and research expertise in this disorder. She is one of the few angioedema specialists in the Northeast and has been treating angioedema patients for 20+ years.

In addition, Dr. Banerji has been regularly involved in research studies of novel treatments for hereditary angioedema. Under her guidance, Mass General has participated in numerous clinical trials improving the care of patients with hereditary angioedema.

Dr. Banerji continues to lead groundbreaking research efforts and to incorporate the latest advances into her clinical practice whenever possible.