Top 10 ANCA-associated vasculitis stories of 2023

News about rituximab was of particular interest to our readers

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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At ANCA Vasculitis News, we have spent the last year bringing you coverage of the latest developments in research and treatment for ANCA-associated vasculitis (AAV).

Here, we’ve compiled a list of the top 10 most-read articles of 2023, with a brief description of each. We look forward to continuing to serve the AAV community in 2024.

No. 10 – Rituximab effective for inducing AAV remission in most patients

A study of nearly 80 Japanese AAV patients who were treated with rituximab found that more than 70% of them were in disease remission within the first six months of treatment, and all but three were still in remission after another six months of follow-up. The results supported rituximab’s efficacy for AAV, though researchers noted serious infections were frequent with the therapy, highlighting the need for precautionary measures.

Sold as Rituxan among other brand names, rituximab is an immunosuppressive therapy approved for granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), the two most common types of AAV. It works by reducing the number of B-cells, the immune cells that produce antibodies, including the self-reactive antibodies, called ANCAs, that typically drive AAV.

No. 9 – Immunosuppressive drugs can help prevent airway tightening

A review of data from more than 100 GPA patients in Italy found immunosuppressive medications can help reduce the risk of recurrence of subglottic stenosis, a GPA complication in which the airway becomes tight. The immunosuppressants rituximab and cyclophosphamide showed particularly good efficacy for preventing subglottic stenosis relapse after a standard procedure to widen the airway.

No. 8 – About 1 in 10 AAV patients don’t respond to rituximab

By analyzing data from more than 120 newly diagnosed or relapsing AAV patients treated with rituximab, researchers in France found more than 10% showed resistance to treatment, meaning rituximab didn’t trigger disease remission within a few months as is typical. Poor responders significantly more often had a localized form of AAV where symptoms mainly affect a particular body part. All of the non-responsive patients were eventually able to achieve remission with higher doses of their treatment and/or other immunosuppressants.

No. 7 – Maintenance rituximab bests azathioprine in Phase 3 clinical trial

Full results from a Phase 3 trial called RITAZAREM (NCT01697267) showed, after AAV remission is achieved with rituximab and glucocorticoids, rituximab is better than the oral therapy azathioprine at preventing future disease recurrence. In the trial, which involved 170 GPA and MPA patients, those given maintenance rituximab treatment had less than half the risk of relapse compared with those given azathioprine.

No. 6 – Argenx plans proof-of-concept trial of efgartigimod in AAV

In May, Argenx announced its plans to launch a proof-of-concept trial to evaluate whether its treatment, efgartigimod, results in potential benefits for people with AAV. Administered directly into the bloodstream, the therapy is designed to reduce levels of IgGs, the antibody class to which most self-reactive antibodies belong, including ANCAs. Efgartigimod is already approved to treat another disease, called generalized myasthenia gravis, that’s associated with self-reactive antibodies.

No. 5 – Rituximab may lessen efficacy of COVID-19 vaccines in AAV patients

An analysis of data from 35 people with AAV who were on rituximab showed 72% of them failed to mount an appropriate antibody-mediated immune response after receiving vaccinations for COVID-19. Still, many of these patients showed robust cell-mediated immune responses after vaccination, and infection rates were low. However, severe disease was reported in one-third of infected patients, being generally associated with no or low immune responses to the vaccines.

No. 4 – Low antibody levels with rituximab linked to high infection risk

While immunosuppressants are known to increase the risk of serious infections, an analysis of data from 121 adults with AAV or other autoimmune disorders indicated those with low antibody levels following rituximab treatment are especially vulnerable. Findings also highlighted that a pneumococcal vaccine, which helps prevent certain bacterial infections, reduced serious infection risk by threefold for rituximab-treated patients.

No. 3 – Relapses of ear, nose, and throat symptoms are common in AAV

Researchers systematically reviewed 31 studies in the scientific literature to assess the efficacy of therapeutic approaches for AAV patients with nose, ear, and/or throat symptoms. They found immunosuppressants are usually effective for inducing remission from these symptoms, but relapse rates ranged from 10%-51%.

No. 2 – Report of mother and son with GPA suggests AAV’s heritability

Scientists reported the case of a 62-year-old woman and her 32-year-old son who both developed GPA and showed similar disease manifestations including symptoms and levels of anti-PR3 ANCAs, the type of self-reactive antibody typically linked to GPA. This case adds to a growing body of data indicating that, at least in some cases, AAV development may be influenced by certain mutations that can be passed from parents to their biological children.

No. 1 – Study highlights impact of fatigue in AAV

In a study that included 52 people with inactive AAV, more than half met the criteria for chronic fatigue disorder, a condition defined by excessive and long-lasting fatigue. Also, those meeting the criteria showed significantly more accumulated organ damage, and were more likely to have anti-MPO ANCAs, another common type of AAV-driving antibodies. These findings highlight that substantial fatigue can be present even when AAV is in remission, and may be influenced by ANCA type. Based on the results, the researchers called on clinicians to be aware of the effects this symptom can have on the daily lives of patients.


At ANCA Vasculitis News, we hope these stories and the rest of our 2023 coverage helped to better inform and improve the lives of those affected by AAV. We look forward to serving the community further in 2024, seeking to provide ongoing support and information.

We want to wish all our readers a bright and happy 2024.