How to treat ANCA‑associated vasculitis: Practical messages from 2016 EULAR/ERA‑EDTA recommendations

Jan Sznajd, Chetan Mukhtyar

Research output: Contribution to journalReview articlepeer-review

7 Citations (Scopus)
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The European League against Rheumatism (EULAR) with the European Renal Association - European Dialysis and Transplant Association recently published an update of 2009 EULAR recommendations with a focus on the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). In this article, we discuss the following key messages for clinical practice derived from these recommendations: 1) biopsy should be performed if possible to confirm new diagnosis or relapse; 2) glucocorticoid therapy is an extremely important adjunct to the management of AAV, but it is also responsible for the majority of adverse effects; the dose should be tapered to 7.5 to 10 mg/d at 3 to 5 months; 3) cyclophosphamide or rituximab are the mainstay of remission induction; 4) patients with major relapse should be treated like those with new disease, but rituximab is the preferred option in those patients who relapse after prior cyclophosphamide; 5) minor relapse should not be treated with glucocorticoid alone, and a change in immunosuppressive regimen should be considered; 6) rituximab can be used not only for remission induction but also for maintenance; 7) maintenance therapy should continue for at least 2 years, after which gradual taper could be considered; 8) while ANCA are extremely useful for diagnosis and rising ANCA levels seem to be associated with relapse, serial monitoring should not guide treatment decisions; 9) monitoring of AAV patients should be holistic with a structured assessment tool and monitoring for effects related to the vasculitis as well as treatment; 10) management should be either at or in conjunction with an expert center; and 11) patients should be involved in decision making and have access to educational resources.

Original languageEnglish
Pages (from-to)781-788
Number of pages8
JournalPolskie Archiwum Medycyny Wewnetrznej
Issue number10
Publication statusPublished - 28 Oct 2016


  • Aged
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis
  • Europe
  • Humans
  • Middle Aged
  • Practice Guidelines as Topic
  • Societies, Medical

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