AAV Does Not Raise Healthcare Use, Mortality in Goodpasture’s Syndrome, Study Says

ANCA-associated vasculitis does not increase healthcare resource utilization or in-hospital mortality rates in patients with Goodpasture’s syndrome, a study reports.

The study, “Impact of ANCA-Associated Vasculitis on Outcomes of Hospitalizations for Goodpasture’s Syndrome in the United States: Nationwide Inpatient Sample 2003–2014,” was published in the journal Medicina.

Goodpasture’s syndrome (GS) is a rare but potentially life-threatening autoimmune disease that can co-exist with several disorders, including anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV).

“Studies have demonstrated that the coexistence of ANCA and GS is recognized to occur at a much higher frequency than expected by chance alone, given the rarity of the individual diseases,” the researchers wrote.

Although patients with both disorders have shown severe kidney disease, the impact of AAV on mortality rates and healthcare resource utilization among those with GS is still unclear.

To tackle this question, researchers in the U.S. and Thailand used the National Inpatient Sample (NIS) — a publicly available, inpatient, all-payer U.S. healthcare database — to analyze mortality rates and resource utilization among patients with both GS and AAV.

Using the NIS, the investigators identified 964 hospitalized patients who had been diagnosed with GS from 2003 to 2014. From these, 84 (8.7%) had also been diagnosed with AAV.

Compared to GS patients without AAV, those who had both disorders tended to be older (mean age of 57 versus 54), and were more likely to be men (54% versus 47%), and have hemoptysis (42% versus 27%). Hemoptysis is a medical condition in which patients cough up blood or blood-stained mucus.

The researchers also found that while in hospital, GS patients who also had AAV were more likely to require mechanical ventilation (breathing support) and non-invasive ventilation support compared to those with only GS. However, these differences were not statistically significant.

In contrast, patients with both disorders were less likely to require renal replacement therapy and plasma exchange (plasmapheresis) compared to those with GS alone, but these differences were insufficient to reach statistical significance. Of note, plasma exchange refers to the process by which the liquid part of a patient’s blood (plasma) is replaced after being separated from blood cells to remove harmful self-antibodies.

“Our study confirmed that hospitalized patients with coexistence of ANCA vasculitis and GS had similar rates of renal failure and renal replacement therapy when compared to those with GS alone,” they wrote.

Furthermore, the researchers found no significant differences in other outcomes, including organ failure, sepsis, and in-hospital mortality, between GS patients, with or without AAV.

“Our study demonstrated no significant differences between resource utilization and in-hospital mortality among hospitalized patients with coexistence of ANCA vasculitis and GS, compared to those with GS alone,” the investigators wrote.

However, they added that “future large multicenter studies with long-term follow-up are needed to evaluate the impact coexistence of ANCA on the long-term renal prognosis of patients with GS.”