Abstract
BackgroundIntravenous pulse methylprednisolone (MP) is commonly included in the management of severe ANCA associated vasculitis (AAV) despite limited evidence of benefit. We aimed to evaluate outcomes in patients who had, or had not received MP, along with standard therapy for remission induction in severe AAV.MethodsWe retrospectively studied 114 consecutive patients from five centres in Europe and the United States with a new diagnosis of severe AAV (creatinine > 500 μmol/L or dialysis dependency) and that received standard therapy (plasma exchange, cyclophosphamide and high-dose oral corticosteroids) for remission induction with or without pulse MP between 2000 and 2013. We evaluated survival, renal recovery, relapses, and adverse events over the first 12 months.ResultsFifty-two patients received pulse MP in addition to standard therapy compared to 62 patients that did not. There was no difference in survival, renal recovery or relapses. Treatment with MP associated with higher risk of infection during the first 3 months (hazard ratio (HR) 2.7, 95%CI [1.4–5.3], p = 0.004) and higher incidence of diabetes (HR 6.33 [1.94–20.63], p = 0.002), after adjustment for confounding factors.ConclusionsThe results of this study suggest that addition of pulse intravenous MP to standard therapy for remission induction in severe AAV may not confer clinical benefit and may be associated with more episodes of infection and higher incidence of diabetes.
Highlights
Intravenous pulse methylprednisolone (MP) is commonly included in the management of severe antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) despite limited evidence of benefit
We found no difference in overall survival, renal recovery or relapses by 12 months between patients that received MP in addition to standard therapy and those that did not receive MP, despite the two groups being well balanced in terms of age, ANCA specificity and markers of severity such as Birmingham Vasculitis Activity Score (BVAS) score, presenting creatinine and percentage of glomeruli with crescents on renal biopsy
The MEPEX trial showed that plasma exchange was more efficacious at 12 months compared with pulse MP in patients with life-threatening renal disease [3]
Summary
Intravenous pulse methylprednisolone (MP) is commonly included in the management of severe ANCA associated vasculitis (AAV) despite limited evidence of benefit. The antineutrophil cytoplasmic antibody (ANCA) associated vasculitides (AAV) are a group of systemic, autoimmune, inflammatory conditions, that include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and its renal limited variant (renal limited vasculitis; RLV). They are Severity in AAV ranges from localised disease to severe involvement, the latter defined by the presence of life threatening manifestations that usually include rapidly progressive glomerulonephritis with a requirement for renal replacement therapy. The MEPEX trial compared plasma exchange (PEX) to intravenous pulse methylprednisolone (MP) over 3 days in addition to oral cyclophosphamide and high dose oral corticosteroids for induction of remission in severe AAV and showed improved renal. Many clinicians routinely use MP prior to commencing high dose oral corticosteroids in addition to PEX and cyclophosphamide
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