Vasculitis   

Patient is asymptomatic. Labs showed +P-ANCA 1:160. Negative MPO and PR3.

Vasculitis activity has been assessed with serial CTA of the chest and head/neck. She has existing right axillary artery disease and new areas of invo...

How would you treat?  Would you view tocilizumab as an option in the future? 

The ADVOCATE trial only studied use for 52 weeks. 

Would you proceed with CABG procedure or treat the aortitis first?

Other manifestations improved (renal function has stabilized, fatigue, arthritis, and LCV have resolved). Ophthalmologist has controlled the uveitis u...

In a patient for whom rituximab is the best option for their disease management. 

Is there a role of rituximab therapy over repeat cyclophosphamide? (Cyclophosphamide induction 2 years prior)

When would you consider tapering? at one year? 18 months?

At what CD 19/20 level do you redose? Is this lab dependent?  Do you also use these levels to instruct patients when to get vaccinated?

MPO/PR3, P-ANCA negative. IgG4 normal.

Would the checkpoint inhibitor still work if blocking only that portion of the inflammatory cascade as opposed to more global blockade with steroids?

What are your primary and secondary agents?

Would you obtain imaging? If so, what type of imaging?

Is there a role for monitoring for relapse or increased disease activity as opposed to scheduled dosing?

Is there role for IVIG? Would you alter the dose or time course of steroid therapy?

In your experience, are there specific disease manifestations in which HLAB51 is particularly helpful?

Other than inflammatory markers and following symptoms/exam, do you need any other specific monitoring for progression to systemic disease? 

Would you suggest switching to a viral vector vaccine, such as J&J?

How does this vary from continued disease monitoring?

Additionally, what is the current role for temporal artery ultrasound in workup for GCA?

Do you obtain vascular imaging routinely in these cases, and if so, do you use cross-sectional or invasive angiography?

Thoughts on sarilumab vs methotrexate, or just treat with steroids alone

Small study in pediatric PACNS have evaluated this as a potential marker (Cellucci et al., PMID 22740622)

There are multiple difficulties that could be seen: steroids can precipitate a sickle cell crisis, vasculitis and sickle cell can produce similar clin...

Would you change rituximab maintenance dose or schedule?

Specifically, do you reach for Rituximab or cyclophosphamide?

ADVOCATE trial

E.g., MPO vs PR3, newly diagnosed vs relapsed, renal involvement. Acknowledge that the ADVOCATE study was not powered to detect these differences, but...

In the ADVOCATE trial, patients were not re-dosed with rituximab.

Given the slow recovery of nerve damage, what would you expect to see on a repeat EMG after treatment? Continued denervation or just sequelae of past ...

What if the patient has MGUS? Do patients with type 1 cryoglobulins need a bone marrow biopsy as part of the work up?

How would you label such a patient?  Would you treat differently if they have poor functional status?

This question is part of a collaboration with RheumMadness and is in reference to: Pathogenic ANCARAVE Trial

 If you use both, how do you decide which to use for a particular patient?