Should we be Treating ANCA Vasculitis Earlier?

Admittedly, I'm not a "vasculitis expert," however, I have seen a few vasculitis patients over the past 10 years. I'd also like to quietly admit that I've been using off-label rituximab to keep patients in remission. As I ponder the complex web of issues with AAV patients, I can't but help think of where therapy in this field should go in the future.

I don't understand the very early phases of AAV and perhaps I miss patients with early disease. We know there is often a considerable time lag between the onset of initial symptoms and the realization that this is AAV. There are reports in the literature of AAV presenting with issues such as Achilles tendonitis or monoarthritis where I would not typically think of AAV. I personally have one patient who presented with an intermittent seronegative oligoarthritis who eventually developed a vasculitic rash. In truth, her arthritis was probably related to the AAV all along. My point is that I do not understand the early manifestations of AAV. Dr. Peter Merkel said in his talk, "There is always more to acute vasculitis than you think. If you look for more problems … you will usually find them."

I don't know how to treat my patients with vasculitis. Pardon? Honestly, I'm not certain. I've heard two talks at this conference that emphasized the impact of cumulative damage over time in AAV patients. This clearly begs the conclusion that we are not properly treating these patients. Why aren't we treating them properly? Probably three reasons that quickly come to mind: (1) We don't fully appreciate the extent of the disease. (2) The disease has considerable clinical heterogeneity, and we cannot truly know the natural progression in each individual patient. (3) Therapy is not free of toxicity. It is for all of these reasons that we probably under-treat AAV. Perhaps there are other reasons you can think of?

The same patient with oligoarthritis flared with a vasculitic rash, sinus disease, mild periorbital inflammation, fatigue, and arthritis. She did not have any renal or lung involvement. I found myself in a conundrum ... I didn't know how this disease would progress. Would it involve her lungs? Would it involve her kidneys? How much sinus destruction could there be? Should I use steroids with methotrexate or azathioprine, or should we use rituximab up front? There is nothing to guide us on the treatment of patients with early vasculitis before damage has accrued.

After careful consideration and discussion with the patient we used rituximab and methotrexate early on. She continues on a small dose of methotrexate and q6month rituximab and she is in complete remission. Her PR3 ANCA titres remain elevated.

More reports from ACR 2015