Plasma exchange does not prolong the survival of patients with severe ANCA-associated vasculitis connected with diffuse alveolar hemorrhage, a potentially life-threatening condition in which blood starts to build up in the lungs, a study has found.
The results challenge the notion that plasma exchange therapy might improve the prognosis of these patients, according to authors of the study “Clinical implication of plasma exchange on life-threatening antineutrophil cytoplasmic antibody-associated vasculitis,” which was published in the journal BMC Pulmonary Medicine.
Anti-neutrophil cytoplasmic autoantibody (ANCA) vasculitis, or AAV, is an autoimmune disease caused by the production of self-reacting antibodies that wrongly target and attack a person’s own cells and tissues, leading to blood vessel inflammation and damage in tissues and organs.
Guidelines from the European League Against Rheumatism (EULAR) and the European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) recommend a combination therapy of glucocorticoids and immunosuppressive agents to manage AAV in newly-diagnosed patients.
However, for people with severe AAV accompanied by kidney failure or diffuse alveolar hemorrhage (DAH), plasma exchange sometimes is recommended. The approach removes harmful antibodies circulating in the blood by replacing a patient’s plasma (portion of blood obtained after removing blood cells) for the plasma of an healthy donor.
Although some studies reported that plasma exchange was associated with positive outcomes in these patients, its potential benefits in those with life-threatening AAV and DAH are still controversial.
Here, researchers at Yonsei University College of Medicine in South Korea investigated the potential survival benefits of plasma exchange in a group of patients with severe AAV and DAH.
After reviewing the electronic medical records of 212 patients diagnosed with AAV at the Severance Hospital from October 2000 to March 2019, investigators selected nine with life-threatening AAV who received plasma exchange to be included in the analyses.
From the nine patients (five men and four women, with a median age of 71 years), six were treated with plasma exchange for DAH.
Four of the nine (44.4%) patients who received plasma exchange died at a median follow-up of 92 days (approximately three months), three from sepsis and one from a lack of response to plasma exchange.
When investigators compared the clinical characteristics of the six patients who received plasma exchange for DAH to four AAV patients who had symptoms of DAH but never received plasma exchange, they found no significant differences between the two groups.
“We also compared the cumulative patients’ survival rate between patients with DAH receiving PLEX [plasma exchange] and those not receiving PLEX … to assess the survival-benefit of PLEX for DAH. However, we found no significant difference between the two groups, which suggested that PLEX had no survival benefit for DAH in AAV patients,” the researchers wrote.
Based on these findings, investigators claimed the potential survival benefit of plasma exchange for patients with severe AAV associated with DAH remains controversial.
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