EULAR: Assess for PR3-MPO-ANCA as primary testing method for ANCA-associated vasculitis
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Rheumatologists should assess for PR3- and MPO-ANCA using a “high-quality antigen-specific assay” as the primary testing method for patients suspected of ANCA-associated vasculitis, according to updated EULAR recommendations.
“The 2008 and 2016 EULAR recommendations for the management of [ANCA-associated vasculitis (AAV)] have provided guidance in clinical practice,” Bernhard Hellmich, MD, the chair of the department of internal medicine, rheumatology and immunology at the Medius Klinik Kirchheim in Germany, told attendees during EULAR 2022 Congress. “But since their publication, several high-impact studies in AAV have been published, and the results of these studies required an update of the existing recommendations.”
To provide a 2022 update to its recommendations for the management of ANCA-associated vasculitis, EULAR formed a task force that included rheumatologists, nephrologists and internists, as well as patient representatives, health care professionals, fellows and methodologists. Members followed the EULAR standardized operating procedure for developing new guidelines, which included meetings to discuss the goals and systematic literature reviews.
In all, the task force issued four overarching principles and 17 specific recommendations in the management of AAV. The overhaul includes six new recommendations, eight recommendations that have been revised since the last update in 2016, and three unchanged recommendations. Additionally, three old recommendations were transformed into new overarching principles.
The overarching principles included in the new set of recommendations include the idea that patients should be offered the best care possible, built on shared decision-making. Other principles argue that patients should have access to education discussing the impact of AAV and its prognosis, that they should “periodically” be screened for treatment-related adverse events and comorbidities, and that the heterogenous nature of AAV requires multidisciplinary management.
According to the recommendations, biopsies are supportive of new and relapsing vasculitis diagnoses. In patients showing signs of AAV, rheumatologists should test for PR3- and MPO-ANCA using a high-quality antigen-specific assay. To induce remission in patients with new-onset or relapsing granulomatosis with polyangiitis/microscopic polyangiitis (GPA/MPA), treatment with glucocorticoids and rituximab (Rituxan, Genentech) or cyclophosphamide are recommended.
To induce remission in non-organ, non-life-threatening GPA/MPA, EULAR recommends treatment with glucocorticoids and rituximab. For courses aiming for remission of GPA/MPAS, oral glucocorticoid doses of 50 mg to 75 mg of prednisolone per day is a good starting point, with the goal of achieving 5 mg prednisolone per day by 4 to 5 months, according to EULAR.
To reduce glucocorticoid exposure, rheumatologists can consider avacopan in combination with rituximab or cyclophosphamide. Routine use of plasma exchange for alveolar hemorrhage in GPA/MPA is not recommended, but may be considered to induce remission in patients with serum creatinine of more than 300 micro-mol per liter due to glomerulonephritis.
Patients with GPA/MPA who are experiencing disease refractory to therapy should have treatment courses re-examined, according to the recommendations. After achieving remission of GPA/MPA, treatment can be maintained with rituximab. Therapy to maintain remission for GPA/MPA should be maintained for 24 to 48 months. To induce remission in patients with eosinophilic granulomatosis with polyangiitis (EGPA), EULAR recommends therapy with a combination of high-dose glucocorticoids and cyclophosphamide.
Other recommendations include:
To induce remission in new-onset or relapsing EGPA with life-threatening involvement, rheumatologists should use combination treatment with high-dose glucocorticoids and cyclophosphamide;
Treatment with glucocorticoids should be used to induce remission for patients with EGPA without life-threatening involvement;
Mepolizumab should be used in patients with relapsing or refractory EGPA with no life-threatening disease;
To maintain remission in patients with EGPA, methotrexate, azathioprine, mepolizumab or rituximab are recommended.
“Structured clinical assessment” instead of ANCA or CD19 and B-cell testing should inform treatment decisions in patients with AAV;
Before each course of rituximab, serum immunoglobulin concentrations should be measured to detect immunodeficiency; and
Trimethoprim-sulfamethoxazole should be used as prophylaxis against pneumocystis jirovecii pneumonia in patients with AAV receiving rituximab, cyclophosphamide and/or high-dose courses of glucocorticoids.
“For a rare disease group, I think this is very good progress, but there are still many open questions, so we have a long research agenda,” Hellmich said.
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Hellmich B. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Presented at: EULAR 2022 Congress; June 1-4, 2022 (virtual meeting).
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