Plasma Exchange In ANCA-associated Vasculitis Research Articles

Plasma exchange in ANCA-associated vasculitis: the pro position.

Plasma exchange (PLEX) is capable of removing significant amounts of circulating antibodies. In anti-neutrophil cytoplasmic antibody-associated vasculitis, PLEX was reserved for patients with severe presentation forms such as rapidly progressive glomerulonephritis and pulmonary haemorrhage. The Plasma Exchange and Glucocorticoids in Severe ANCA-Associated Vasculitis (PEXIVAS) trial included all comers with a glomerular filtration rate <50 mL/min/1.73 m2 and thus aimed to answer the question of whether PLEX is an option for patients with no relevant kidney function impairment or not. PEXIVAS revealed that after a follow-up of almost 3 years, routine administration of PLEX does not provide an additional benefit to reduce the rate of a composite comprising end-stage kidney disease or death. In the absence of histological parameters, it is tempting to speculate whether PLEX is effective or not in those with a potential for renal recovery. A subset of patients presented with alveolar haemorrhage, and there was a trend towards a better outcome of such cases receiving PLEX. This would be in line with observational studies reporting a recovery of alveolar haemorrhage following extracorporeal treatment. In this PRO part of the debate, we highlight the shortcomings of the PEXIVAS trial and stimulate further research paths, which in our eyes are necessary before abandoning PLEX from the therapeutic armamentarium.

Plasma exchange for the management of ANCA-associated vasculitis: the con position.

Advances in the diagnosis and treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis have led to continued improvement in survival and prognosis over the course of the last 4 decades. Nevertheless, the most acute and severe disease manifestations, including severe kidney disease and alveolar hemorrhage, continue to be associated with increased early mortality from disease activity or treatment complications as well as risk for the development of end-stage kidney disease (ESKD), which in turn directly affects the overall prognosis of ANCA-associated vasculitis. Plasma exchange (PLEX) has long been proposed and used for these most severe disease manifestations under the assumption that its effects are swift and supported by our understanding of the pathogenic role of ANCA. Yet convincing evidence of a beneficial effect of PLEX in ANCA-associated vasculitis has been lacking, as early studies and small trials have generated conflicting results. The controversy regarding PLEX has been accentuated recently as the largest randomized controlled trial ever conducted in ANCA-associated vasculitis, the Plasma Exchange and Glucocorticoids in Severe ANCA-associated Vasculitis trial, which was specifically designed to evaluate the efficacy of PLEX in patients with severe renal disease or alveolar hemorrhage, failed to show a difference in the combined primary outcome measure of death or ESKD in patients who received PLEX versus those who did not. In light of these disappointing results, we herein review the currently available data on PLEX for ANCA-associated vasculitis and explain why we believe that these data no longer support the use of PLEX in ANCA-associated vasculitis.

PEXIVAS-The kiss of death for plasma exchange in ANCA-associated vasculitis?

PEXIVAS-The kiss of death for plasma exchange in ANCA-associated vasculitis?

PEXIVAS: The End of Plasmapheresis for ANCA-Associated Vasculitis?

Kidney involvement in ANCA-associated vasculitis is common (64%–85%) and associated with high morbidity and mortality. Despite recent advances in therapy, a significant number of patients still progress to kidney failure or death. As such, therapies that improve kidney outcomes have been pursued

Antineutrophil Cytoplasmic Antibody-Associated Vasculitides and Respiratory Disease

Vasculitides associated with serum positivity for antineutrophil cytoplasmic antibodies (ANCAs) are a well-established subgroup affecting small- to medium-sized vessels that are commonly recognized as ANCA-associated vasculitis, which includes necrotizing granulomatous vasculitis (NGV) [formerly Wegener granulomatosis], microscopic polyangiitis (MPA), and Churg-Strauss syndrome. NGV usually starts as a granulomatous disease of the respiratory tract and progresses to systemic disease with proteinase 3 (PR3)-ANCA-associated vasculitis, suggesting an aberrant cell-mediated immune response to exogenous or endogenous antigens in the respiratory tract and resulting in granuloma formation. In NGV, granulomata may represent lymphoid structures ultimately responsible for PR3-ANCA production. In both NGV and MPA, necrotizing glomerulonephritis and necrotizing pulmonary capillaritis may well result from an injury orchestrated by ANCA. Untreated NGV and MPA normally are rapidly progressive and fatal. Pulmonary capillaritis with alveolar hemorrhage is a severe complication in patients with MPA and NGV. Because plasma exchange removes circulating ANCAs and other proteins from the blood, its use has been advocated in critical situations of severe renal and pulmonary involvement. However, no studies of plasma exchange in ANCA-associated vasculitis focused on pulmonary involvement have been reported. Dissecting the mechanisms of inflammation may identify molecular targets for future therapies in ANCA-associated vasculitis. Thus, biological agents are emerging as potential therapies in refractory cases. Notably, rituximab and infliximab have been trialed with apparent initial clinical success.