Recently, researchers sought to examine how Nucala (mepolizumab) affected disease progression in patients with eosinophilic granulomatosis with polyangiitis (EGPA), especially when used in conjunction with corticosteroids. According to ANCA Vasculitis News, the research highlighted that the two therapies are safe, effective, and well-tolerated. Even more importantly, the combination of corticosteroids and Nucala improved remission and lowered symptoms and disease progression. Check out the full study findings published in Arthritis Research & Therapy.

Nucala

What is Nucala? This therapy, administered via injection, is a humanized monoclonal antibody that targets interleukin-5 (IL-5). Normally, IL-5 is located on eosinophil surfaces. Thus, targeting IL-5 helps to eliminate high levels of eosinophils from the blood.

Normally, corticosteroids are the standard of care for patients with EGPA. However, some patients’ have treatment-averse EGPA, meaning they need to be treated with immunosuppressant therapies. These are also used for patients whose symptoms are extremely severe. But without using corticosteroids, patients often relapse. Thus, a new therapeutic option was needed.

Researchers explored the combination of Nucala and corticosteroids. Altogether, 16 patients with relapsing or refractory EGPA enrolled. During the study, patients received 300mg Nucala monthly for a 12-month period. Researchers evaluated eosinophil levels, adverse reactions, treatment retention, disease activity and remission, and changes in corticosteroid or immunosuppressant usage. Additionally, they compared this data to that of the same patients from the prior year. Patients had an average age of 61.5 years, with an average diagnosis time of around 4.5 years. Of the patients, 11 (68.75%) had refractory EGPA; 1 (6.25%) was in remission; and 4 (25%) had relapsing EGPA.

Researchers determined that:

• In the year prior to treatment, most patients’ disease activity scores increased by around 1 point. After Nucala treatment, patients’ disease activity scores decreased by a mean of 0.5 points.
• 12.5% of patients achieved remission within 3 months following Nucala treatment. Although the rates fell over time, reintroduction of Nucala significantly improved remission rates to 75% following another year.
• Respiratory – and other – symptoms significantly improved following Nucala use. Additionally, eosinophil levels largely decreased.
• Altogether, no patients discontinued treatment due to adverse reactions.
• Those taking Nucala were able to achieve remission even when using lower doses of corticosteroids.

Eosinophilic Granulomatosis with Polyangiitis (EGPA)

Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is a type of ANCA-associated vasculitis. While it shares features with granulomatosis with polyangiitis (GPA), EGPA is characterized by high eosinophil levels in the blood. Eosinophils are a type of white blood cell. Typically, high levels suggest some sort of infection or allergic reaction. While the exact cause of EGPA is unknown, many consider it to be an autoimmune disorder.

As blood vessels become inflamed, less blood flows to the nervous system, gastrointestinal tract, heart, skin, and lungs. As a result, the organs are often damaged. Only an estimated 2-5 out of every 1 million people are diagnosed with EGPA yearly. Typically, symptoms appear between ages 35 to 50. Patients may experience a variety of symptoms, as EGPA occurs in 3 distinct phases: prodromal (allergic), eosinophilic, and vasculitic. However, not every patient will experience all three phases, nor do these phases always happen in this order.

In the allergic phase, patients may experience:

• Progressively worsening asthma
• Chronic sinus issues
• Hay fever
• Allergic reactions
• Nasal polyps

Next, in the eosinophilic phase, patients may experience:

• High white blood cell counts (eosinophils) in the skin, gastrointestinal tract, lung, and body tissues

Finally, in the vasculitic phase, patients experience:

• Narrowed blood vessels
• Intense blood vessel inflammation

Outside of these phases, common EGPA-associated symptoms include:

• Severe or chronic asthma
• Fatigue
• Fever
• High eosinophil levels
• Acute or chronic sinusitis
• Abdominal pain
• Diarrhea
• Gastrointestinal bleeding
• Neuropathy
• Difficulty breathing
• Coughing up blood
• Lesions or spots which appear to move on a chest x-ray
• Rashes or skin nodules
• Joint or muscle pain
• Glomerulonephritis