EULAR Updates Vasculitis Treatment Recommendations


The European League Against Rheumatism has updated its 2009 recommendations for the management of antineutrophil cytoplasmic antibody-associated vasculitis.

The European League Against Rheumatism (EULAR) has updated its 2009 recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).

The updated recommendations include the addition of rituximab (Rituxan/Genentech) which, in combination with glucocorticoids, was approved for the treatment of polyangiitis (GPA) and microscopic polyangiitis (MPA) by the U.S. Food and Drug Administration (FDA) in 2011. It is currently the only FDA-approved treatment for these diseases. 

While the 2009 recommendations were on the management of small and medium vessel vasculitis, the updated recommendations focus on AAV exclusively, partly because of the vast amount of literature published in the last five years and the licensing of rituximab for AAV.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAVs) includes granulomatosis with polyangiitis (GPA, Wegener’s granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome). GPA, MPA and EGPA have respective annual incidence rates of 2.1–14.4, 2.4–10.1 and 0.5–3.7 per million in Europe, and the prevalence of AAV is estimated at to be 46–184 per million. The 5-year survival rates for GPA, MPA and EGPA are estimated to be 74–91%, 45–76% and 60–97%, respectively.  

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The EULAR task force that lead the development of the new recommendations developed 15 treatment recommendations for standard therapies, which included the use of biological agents, the prognostic value of histopathology and management of long-term complications.

The recommendations were based on the level of evidence which was rated between one and four and it was based on the grade of recommendation which fell between A and D.

The recommendations:

-  Patients with AAV should be managed in close collaboration with disease-specific experts or at facilities prepared to treat this rare disorder.

-  Biopsies are recommended for a new diagnosis and for further evaluation for patients suspected of having relapsing vasculitis.

-  For remission-induction of new-onset organ-threatening or life-threatening AAV, treatment with a combination of glucocorticoids and either cyclophosphamide or rituximab is recommended.

-  For remission-induction of non-organ-threatening AAV, treatment with a combination of glucocorticoids and either methotrexate or mycophenolate mofetil is recommended.

-  For a major relapse of organ-threatening or life-threatening disease in AAV, treatment with a combination of glucocorticoids and either cyclophosphamide or rituximab is recommended.

-  Plasma exchange should be considered for patients with AAV and a serum creatine level of ≥500 mmol/L (5.7 mg/dL) due to rapidly progressive glomerulonephritis in the setting of new or relapsing disease. Plasma exchange can also be considered for the treatment of severe diffuse alveolar hemorrhage.

-  For remission-maintenance of AAV, treatment with a combination of low-dose glucocorticoids and either azathioprine, rituximab, methotrexate or mycophenolate mufti is recommended.

-  Continue remission-maintenance therapy for AAV for at least 24 months following induction of sustained remission.

-  For patients with AAV refractory to remission-induction therapy, switching from cyclophosphamide to rituximab or from rituximab to cyclophosphamide is recommended.

-  A structured clinical assessment, rather than ANCA testing, should inform decisions on changes in treatment for AAV.

-  The ongoing investigation of persistent unexplained hematuria in patients with prior exposure to cyclophosphamide is recommended.

-  Hypoimmunoglobulinaemia has been seen after treatment with rituximab. Testing of serum immunoglobulin levels prior to each course of rituximab and in patients with recurrent infection is recommended.

-  The periodic assessment of cardiovascular risk for patients with AAV is recommended.

-  Patients with AAV should be given a clear verbal explanation of the nature of their disease, the treatment options, the side effects of treatment, and the short-term and long-term prognoses.

-  Following the remission-induction phase of treatment, patients with AAV should be assessed for the extent and ongoing impact of comorbidities associated with their diagnosis. Patients should then be advised where they might find the necessary therapies or support for these conditions.



M Yates, RA Watts, et. al.

"EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis."

Ann Rheum Dis doi:10.1136/annrheumdis-2016-209133 

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