Managing Antineutrophil Cytoplasmic Antibody (ANCA) Vasculitis

According to an article in Healio, Dr. Anisha Dua recently addressed attendees at the ACR Clinical Symposium on the subject of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Dr. Dua is the director of Northwestern University’s  Vasculitis Center. She reminded attendees at the symposium that patients with ANCA often do not attain remission. She offered the following definition of ANCA:

The short definition of ANCA is that the small blood vessels are damaged. However, there is an array of other manifestations such as the involvement of the skin, heart, sinuses, eyes, kidneys, ears, and nervous system.

Dr. Dua stressed that it is the severity of the involvement of these organs that will dictate how the disease will be managed.

In the past, cyclophosphamide and corticosteroids were the treatments of choice. But in the previous ten years treatments have evolved that minimize toxicity and prevent relapse.

A Case Presentation

Dr. Dua described an interview with a twenty-eight-year-old woman who received a diagnosis of microscopic polyangiitis. She explained her own reaction to the examination as watching the patient deteriorate right before her eyes.

Her immediate reaction was to try each and every treatment currently available.

Treatment Options

In cases such as this, pulse steroids immediately come to mind. Dr. Dua said that doctors often use pulse steroids even before they receive the results from the lab.

There are, however, certain risks connected to the use of steroids. The ACR/Vasculitis guidelines suggest a reduction in glucocorticoids when treating microscopic polyangiitis.

The next option doctors may use is cyclophosphamide, otherwise known as rituximab (Rituxan by Genentech). These decisions are based on results from the RITUXIVAST and RAVE clinical trials. Note that additionally rituximab may be used as a maintenance treatment over a four-year period at a dose of 500 mg every six months.

Other Options

Moving on to other cases and options for the various types of ANCA vasculitis, doctors should consider plasmapheresis. This is a method that withdraws blood, then converts it into cells and plasma, and infuses it back into the blood. It is also called plasma exchange.

But again, the cautionary ACR/VF guidelines come into play by advising against the exchange of blood plasma to or from blood circulation.

Complement Cascade

The next option introduced by Dr. Dua is the use of the complement cascade in ANCA. The complement system consists of proteins working together that destroy invaders such as viruses and bacteria. It must be regulated to be certain that it does not attack healthy cells.

Avacopan is an orally administered drug that inhibits the complement C5a receptor. It is being developed to treat autoimmune and inflammatory diseases. It blocks C5a activity, which is a component of the complement system.

Dr. Dua comments that as the role of complement increases, the medical field will be using fewer steroids for ANCA.

About Mepolizumab

Mepolizumab (Nucala by GlaxoSmithKline) is yet another treatment under consideration. It is an interleukinIL-5 inhibitor that has been effective in eosinophilic granulomatosis and polyangiitis (EPGA). It has shown to be effective in the upper respiratory and upper airway issues of EPGA. It is another agent that, if successful, will reduce the need for steroids.

Where There is a Need

Dr. Dua concluded her presentation by saying that in spite of so much progress, still, about thirty percent of patients are not able to achieve remission within six months.

Rose Duesterwald

Rose Duesterwald

Rose became acquainted with Patient Worthy after her husband was diagnosed with Acute Myeloid Leukemia (AML) six years ago. During this period of partial remission, Rose researched investigational drugs to be prepared in the event of a relapse. Her husband died February 12, 2021 with a rare and unexplained occurrence of liver cancer possibly unrelated to AML.

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