New recommendations for managing AAV released by EULAR

Alliance maintains updates will help 'streamline' disease management

Steve Bryson, PhD avatar

by Steve Bryson, PhD |

Share this article:

Share article via email
A doctor talks to a patient.

The European Alliance of Associations for Rheumatology (EULAR) has updated its recommendations on managing ANCA-associated vasculitis (AAV) based on new research and clinical data.

Four overarching principles were introduced — the importance of patient-physician joint decisions, disease education, routine screening for treatment side effects and additional medical conditions, and the need for a multidisciplinary healthcare team.

The experts also provided several treatment recommendations specific to AAV types in addition to clearly defining disease activity states and advising on diagnostic methods.

“EULAR believes that these recommendations will help to streamline the management of people with AAV – while they are not intended to be used as a one-size-fits-all strategy, and may need to be used alongside other recommendations and treatment algorithms, depending on each person’s disease, manifestations, and [simultaneous conditions],” EULAR stated in a press release.

Details of the update, “EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update,” were published in the Annals of the Rheumatic Diseases.

AAV is caused by the immune system’s abnormal production of self-reactive antibodies, called ANCAs, that lead to inflammation and damage to small blood vessels in several organs. Depending on certain features, such as the affected organs, it can be divided into three types — microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (EGPA).

AAV symptoms can vary widely between people, making treatment management challenging.

Recommendations for diagnosing and treating inflammation in small and medium blood vessels were first published in 2009. They were updated in 2016 with a focus on AAV.

A task force of 29 people, including 20 clinical experts from 15 European countries and the U.S., voted on the recommendations after defining key research questions addressing AAV management and performing a literature review.

The new release introduces four overarching principles and 17 recommendations for managing AAV. Separate recommendations for AAV types were also included.

Because definitions of AAV activity have differed across clinical trials, the experts proposed clear definitions for different activity states, namely active disease, remission, sustained remission, treatment response, relapse, and refractory (hard to treat) disease.

Recommended Reading
An illustration showing a line of hands giving the thumb's up sign.

Tavneos’ use by AAV patients prior to approval support its safety

Task force recommendations for AAV

Among the four main principles, the task force emphasized that people with AAV should be given the best care based on decisions shared by patients and physicians with regard to treatment safety, efficacy, and cost. They should be educated to understand the disease’s impact and identify key warning symptoms and treatment-related complications.

Routine screening for treatment-related side effects and coexisting conditions is also recommended, alongside advice to reduce these complications.

Lastly, because AAV is a rare, potentially life- and organ-threatening disease, patients should be followed by multidisciplinary teams at centers with expertise in treating vasculitis.

Managing GPA, MPA

The task force recommended biopsies to establish a new diagnosis and evaluate patients suspected of relapsing. High-quality ANCA testing is also needed for those with AAV signs and/or symptoms.

Among those with new-onset or relapsing GPA or MPA who also have organ- or life-threatening disease, a combination of high-dose glucocorticoids and either rituximab or cyclophosphamide is advised. In relapsing disease, rituximab — sold as Rituxan in the U.S. and MabThera in Europe, with biosimilars available — is preferred.

For those without organ- or non-life-threatening GPA or MPA, a combination of glucocorticoids and rituximab is recommended, with methotrexate or mycophenolate mofetil as alternatives to rituximab. Researchers also recommend tapering the glucocorticoid dose to 5 mg per day within four to five months after the initial high dose.

Tavneos (avacopan), an approved therapy for GPA and MPA, may be considered in combination with rituximab or cyclophosphamide to reduce glucocorticoid exposure.

Plasma exchange, a blood antibody-clearance approach, may induce remission in GPA and MPA patients with active kidney disease. However, routine PLEX is not recommended for treating lung bleeding.

Rituximab is recommended for remission maintenance in GPA and MPA, with azathioprine or methotrexate as alternatives.

Managing EGPA, the rarest type of AAV

For those with new-onset or relapsing EGPA, the rarest AAV type, and organ- or life-threatening symptoms, a combination of high-dose glucocorticoids and cyclophosphamide are preferred. High-dose glucocorticoids and rituximab are an alternative.

Glucocorticoids are recommended for new-onset or relapsing EGPA without organ- or life-threatening disease. Nucala (mepolizumab), an approved EGPA therapy, is advised for relapsing or refractory EGPA patients without these complications.

Nucala is also favored for maintaining EGPA remission in patients without such symptoms at the time of relapse. For remission maintenance after treatment for such complications, methotrexate, azathioprine, Nucala, or rituximab may be considered.

Due to rituximab’s depleting effect on antibody-producing B-cells, patients on it should be tested for overall antibody levels before each treatment course. Those treated with rituximab, cyclophosphamide, and/or high-dose glucocorticoids should be given regular antibiotics (trimethoprim and sulfamethoxazole) to prevent infections.

A structured clinical assessment should inform decisions on treatment changes rather than ANCA and B-cell tests alone. No changes were made to the recommendations for ongoing vaccination in adults with AAV.

“The level of agreement for each recommendation was consistently high among the task force members,” the researchers wrote. “We encourage clinicians to implement these recommendations into their clinical practice to effectively manage AAV and to improve the patients’ quality of care.”