Clinical implication of plasma exchange on life-threatening antineutrophil cytoplasmic antibody-associated vasculitis
Abstract Background: We assessed the rate of and the predictor for all-cause mortality in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients receiving plasma exchange (PLEX) and evaluated the survival-benefit of PLEX for diffuse alveolar haemorrhage (DAH) between AAV patients receiving PLEX and those not receiving. Methods: We retrospectively reviewed the medical records of 212 AAV patients. Demographic, clinical and laboratory data at the time of PLEX was collected from both 9 patients receiving PLEX and 10 AAV patients with DAH. The follow-up duration was defined as the period from the time of PLEX or DAH occurrence to death for the deceased patients and as that to the last visit for the survived patients. Results: The median age of 9 AAV patients receiving PLEX was 71.0 years and 5 patients were men. Four of 9 patients receiving PLEX died at a median follow-up duration of 92.0 days. Three died of sepsis and one died of no response to PLEX. When patients with DAH receiving PLEX and those not receiving were compared, there were no significant differences in variables between the two groups. The cumulative patients’ survival rate between patients with DAH receiving PLEX and those not receiving were also compared using the Kaplan-Meier survival analysis but no survival-benefit of PLEX for DAH was observed. Conclusion: The rate of all-cause mortality in 9 AAV patients receiving PLEX was assessed as 44.4% and it was controversial that PLEX is beneficial for the improvement of prognosis of AAV-related DAH.