2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis (Part 5)

We now continue with our discussion of the guidelines of the American College of Rheumatology (ACR) in relation to the antineutrophil cytoplasmic antibody (ANCA)–associated vasculitides (AAV).
Before we move forward, let us review the terms that will be often appearing in this series. ANCA-AAV is comprised of the diseases known as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA).
The expert panel formed by the American College of Rheumatology (ACR) says that it is recommended that patients with active, severe GPA/MPA with alveolar hemorrhage, should be given plasma exchange to remission induction therapies in certain conditions.
Based on 2 trials evaluated, the use of plasma exchange in patients presenting with alveolar hemorrhage, and no differences in mortality or remission rates were noted.
Thus, plasma exchange, as it appears may not provide any huge benefit for patients with alveolar hemorrhage and is linked with a heightened risk of serious infection.
Plasma exchange could be recommended to be used for certain patients with active glomerulonephritis or those who are critically ill and whose disease is not responding to common remission induction therapies.
Plasma exchange is something that can be used for patients with GPA or MPA and also have anti–glomerular basement membrane disease.
On the other hand, for patients with active, severe GPA/MPA, either intravenous pulse glucocorticoids or high-dose oral glucocorticoids are beneficial as part of first level therapy.
The fact remains that there are no trials where the efficacy of intravenous pulse glucocorticoids is compared to high-dose oral glucocorticoids. Higher doses of glucocorticoids are given to patients with organ-or life-threatening disease but could be at risk of getting serious infections.Likewise, for patients with active, severe GPA/MPA, it is recommended, with some conditions, a reduced-dose glucocorticoid therapy over a standard-dose glucocorticoid for remission induction.

It was shown in a recent study that giving reduced-dose of glucocorticoid leads to a similar benefit compared to a standard-dose therapy, and was linked with a lowered risk of infection.
It is widely known that there are toxicities linked with long-term glucocorticoid usage. Minimal therapeutic use of glucocorticoid may help improve the health of ANCA-AAV patients.
Glucocorticoid use is something that should be done in a case-to-case basis.

We will be moving our discussion on remission induction for active, nonsevere disease, in next week’s column.