Summary
“Whether or not” and “how” to taper off the last bit of medication for patients with clinically stable ANCA-associated vasculitis are hotly debated topics in the vasculitis field. Here we present a large case series of patients with ANCA-associated vasculitis who remained in sustained remission without requirement for any medications following the withdraw of all immunosuppressive therapy and steroids.
Why was the study done?
ANCA-associated vasculitis is an autoimmune disease in which patients experience inflammation of the blood vessels. Historically, this was a fatal disease but recent advances in treatment have substantially improved outcomes for patients. Currently, people with vasculitis are treated with strong medications that inhibit the immune system (immunosuppressive drugs) and help them stay in a disease-free state. However, “whether or not” and “how” to withdraw the last bit of medication, usually 5 mg of low dose steroids, for patients with stable disease is currently unknown. No guidelines currently exist to guide clinical practice in this regard and it has been thought to be unachievable. We wanted to better understand which patients could successfully remain in remission without any need for immunosuppressive drugs or steroids.
How was this study done?
Patients seen for their routine clinical care at the Johns Hopkins Vasculitis Center from 2000-2016 were included in this study. From these, 18 patients were identified who were off immunosuppressive drugs for at least 36 months without a disease relapse. They were then studied over an average of 5 years to understand if sustained disease-free withdraw of all medications, including steroids, was possible in these patients.
What were the major findings?
While most patients received maintenance therapy with low-dose steroids for some time after withdraw of immunosuppressive drugs, 3 did not and 15 were weaned completely off of steroids during the follow up period. Only 3 people experienced a relapse of their disease during the follow up period, one who was on low-dose steroids and 2 who were not on any steroids. This suggests that at least some patients with stable ANCA-associated vasculitis may be able to come off all immunosuppression and steroids.
What is the impact of this work?
This work strengthens the emerging view that select patients with ANCA-associated vasculitis may be able to come off immunosuppression completely and remain in remission. Our findings emphasize the importance of future studies to thoroughly characterize which patients would be able to successfully come off medication.
This research was supported by:
- The Jerome L. Greene Foundation
Link to the original research publication:
Gapud EJ, Manno R, Seo P, Hanouneh M, Geetha D. Long-term Clinical Course of Antineutrophil Cytoplasmic Antibody-associated Vasculitis Patients off Maintenance Therapy. Muacevic A, Adler JR, eds. Cureus. 2018;10(3):e2372. doi:10.7759/cureus.2372.
