Learn about the updated recommendations for the treatment of vasculitis

Vasculitis includes a group of diseases characterized primarily by inflammation of the blood vessels.

Vasodilators can develop at practically any age and their source remains uncertain. Photo: shutterstock.

Currently and depending on the location of the affected vessels, differences between their sizes and the histological findings of biopsy, 11 different types of vessels have been identified. Vasculitis. In the 2022 review, aspects such as guidance on the use of new drugs, such as avacopan (Tavneos) and mepolizumab (Nucala), as well as revised recommendations on the use of rituximab and glucocorticosteroids, were presented and included.

Updated recommendations for treatment Vasculitis Associated with antibody (AAV) and cytoplasmic antigens (AMP) and specific recommendations for the treatment of eosinophilic granulomatosis with polyangiitis (EGPA), it was first separated from eosinophilic granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA).

“So far, EGPA has generally been treated in the same way as [las] Other diseases,” Dr. Bernhard Helmich, MD from the University of Tübingen, Germany, said in an interview prior to his presentation at the conference. But we now have specific data on each type, so there is good reason to make separate recommendations,” he added.

In fact, so much new data has been made available in recent years that only three recommendations remain unchanged from the previous iteration published in 2016.

Since then “several high-impact studies on AAVs have been published, and the results of these studies necessitate an update of existing recommendations,” Helmich said.

Developed in record time, only 7 months from start to finish, the AAV Recommendations update process followed EULAR Standard Operating Procedures.

An important step in this process is to conduct a systematic review of the literature. Perhaps decisively, and in contrast to the early guidelines of Vasculitis Recently published in 2021, the most recent literature search was able to include data on avacopan, which was approved for use in Europe in January as an add-on treatment for adults with severe active GPA and PMA.

The results of the literature review were reported separately at the EULAR 2022 conference, with separate presentations highlighting the data behind the modified treatment, prognosis and follow-up recommendations.

Highlights changes

The main change is the introduction of four general principles that were not included in the previous update, Helmich said, “We have moved some of the current recommendations with a low level of evidence into general principles,” noting that the first year was the principle that “best care should be based on Patient-physician joint decision making, taking into account efficacy, safety, and cost” for all patients.

The second principle states that patients should receive education covering the diagnosis and impact of AAV, including recognition of warning symptoms and treatment options.

The third focuses on screening for adverse effects and comorbidities, and recommends that patients receive appropriate preventative treatment and lifestyle advice, in order to “de-dope”.

In fact, “for patients who already have a high burden of glucocorticoid-related adverse effects, especially relapses, I think it would make sense to give Avacoban only and not steroids,” Helmich said.

Other new recommendations regarding induction of remission and maintenance therapy in recent emergence of EGPA.

With regard to the latter, the choice of treatment depends on whether there is a condition with organ or life risk, methotrexate, azathioprine, mepolizumab or rituximab is recommended, or if there is no case of organ or life risk, mepolizumab is preferred.

Revised and unchanged recommendations

Eight of the recommendations were reviewed, with rituximab ranking more prominently as a treatment in some.

To induce remission in GPA and MPA with life-threatening or organ disease, rituximab is now the preferred option for recurrent disease.

Rituximab has also replaced methotrexate as the preferred option to maintain remission, although methotrexate and azathioprine can still be considered as alternatives.

Another changed statement concerns the duration of maintenance therapy in GPA and MPA, which now calls for 1-2 years of treatment after remission has been achieved. Longer treatment may be required in cases of relapse, but benefits and risks must be carefully weighed and patient preferences taken into account.

Prevention of pneumonia and other infections is still recommended, and evidence-based guidelines state that patients receiving high doses of cyclophosphamide, rituximab, or steroids should be treated with trimethoprim-sulfamethoxazole (co-trimoxazole).

“There are retrospective data in the AAV group that taking co-trimoxazole reduces not only the incidence of pneumonia, but also other infections. Therefore, this is an important recommendation for clinical practice,” Helmich said.

“For a group of rare diseases, I think it’s very good progress,” Helmich said, but “there are still a lot of open questions, so we have a long research agenda.”

However, there is no meaningful recommendation on COVID-19, because “the circumstances that affect COVID outcomes are rapidly changing and any recommendations made now will likely become outdated soon; AAV recommendations are supposed to last at least two years.”

In a press release issued by the German Association for rheumatismwhich has nothing to do with Helmich’s speech, experts commented on the guidelines for Vasculitis In general, he notes, there has been a move towards the use of biological therapies such as rituximab and mepolizumab as the new standard of treatment.

Andreas Krause, head of the DGRh and chief medical officer at the Emmanuel Hospital in Berlin, noted that “cyclophosphamide, which was used in the past and which inhibits blood formation in the bone marrow and can cause infertility, can now be dispensed with…” frequently.

Julia Hall, MD, of Rheumazentrum Schleswig-Holstein Mitte in Neumünster, Germany, was also quoted in the press release as saying, “For patients, successful use of biopharmaceuticals and lowering the dose of glucocorticoids is a major advance.”

Hall co-developed the revised European Guidelines. She is also the lead author on Cure Vasculitis Based on the available evidence. Hellmich has acknowledged having relationships with several pharmaceutical companies, serving as a speaker or consultant or both with Abbvie, Amgen, AstraZeneca, Boehringer Ingelheim, Bristol-Myers Squibb, Chugai, GlaxoSmithKline, InflaRx, Merck Sharp & Dohme, Novartis, Pfizer, Roche and Vifor .

Source consult here.