Vasculitis&nbsp;&nbsp;&nbsp;

What treatment regimen would you recommend for a patient with biopsy-proven giant cell arteritis and diffuse cutaneous systemic sclerosis?

When do you consider tapering tocilizumab in patients with GCA in remission?

4 Answers available

What is your preferred regimen for remission induction and maintenance in EGPA with cardiac involvement?

What biologic or conventional/synthetic DMARD would you use as a steroid sparing agent in a patient with GCA and a history of diverticulitis?

When do you obtain nerve biopsy to evaluate for vasculitic neuropathy in patients with distal symmetric polyneuropathies?

What would be your approach to managing severe ANCA-associated vasculitis in a patient who is also septic from a bacterial infection?

How long do you continue rituximab in patients with ANCA associated vasculitis who have achieved remission?

What information do you use to make this determination? Do you tend to transition to an oral agent like azathioprine after discontinuation to prevent ...

How would you approach the treatment for patients with renal-limited ANCA vasculitis who have persistent proteinuria, hematuria, and ANCA titers and have completed a steroid taper and received three doses of rituximab?

How do you determine which patients with ANCA associated vasculitis may be good candidates for reduced dose glucocorticoid tapering?

This question is part of our collaboration with ACR Convergence 2024 to Continue the Conversation from the meeeting. This question was inspi...

How soon after starting treatment for Takayasu arteritis do you decide on the need for any vascular interventions to manage chronic damage?

How would you manage a patient with Takayasu arteritis controlled on TNFi who develops erythema nodosum that is only partially responsive to NSAIDs?

Would you view this as a sign that disease is not adequately controlled despite labs and imaging suggesting no active vasculitis?

How soon after starting treatment would you repeat imaging in patients with Takayasu to monitor response and ensure you have the correct diagnosis?

Do you monitor patients with CTAs or MRAs?

How would you manage active psoriasis and psoriatic arthritis in patient on Rituximab and prednisone for MPO positive vasculitis?

The patient has ongoing inflammatory arthritis despite methotrexate, apremilast, and jak inhibitor trials.

What would be the preferred treatment option for PMR if patient develops gastric perforation soon after initiation of steroids?

How would you approach management of nodular scleritis in the setting of suspected GCA?

Can nodular scleritis be a presenting manifestation of GCA?

How would you manage a patient with severe Hurley Stage 3 active, draining, HS who is also currently requiring Rituxan for management of vasculitis?

Would you be comfortable combining a TNFi with Rituxan in this patient?

How would you approach the workup and management of a young patient with recurrent biannual non-scarring oral ulcers and new onset neurologic symptoms with associated CNS white matter lesions concerning for Behcet’s?

How would you manage a patient with ANCA associated vasculitis who has recurrent flare including pulmonary hemorrhage, persistent MPO antibody positivity while on maintenance rituximab and avacopan regimen even after induction rituximab therapy?

How would you approach management of a patient with a medium-to-large vessel vasculitis who developed perforation of the stomach and colon on steroids and cyclophosphamide?

Vasculitis involving aorta, subclavian, common carotid, SMA, IMA, renal artery, and celiac artery.

How would you approach the workup and management of isolated inflammatory subglottic stenosis in a young previously healthy patient that is steroid responsive with a completely negative serologic autoimmune workup?

Would you push for biopsy before deciding on treatment?

How do you approach the workup of clinically diagnosed cutaneous vasculitis in healthy young individuals without systemic symptoms?

How do you approach the work up of pulmonary artery aneurysm in the absence of other clinical features of Behcet’s?

What features on CTA/MRA are most helpful for differentiating large vessel vasculitis from atherosclerosis?

What is your approach to management of pulmonary fibrosis in patients with a history of microscopic polyangiitis who do not have other active organ involvement?

Patient is with past history of glomerulonephritis in remission after rituximab for the past 2 years. +MPO/P-ANCA

How would you approach a pulmonary-renal PR3+ ANCA vasculitis patient who has persistent re-narrowing of mainstem bronchus after several dilatation and stenting procedures, with other anca features well-controlled on rituximab & avacopan?

5 Answers available

Do you avoid tocilizumab in patients with prior bariatric surgery given the risk of GI perforation?

Is there a subset of ANCA vasculitis patients for which you would try plasma exchange?

Are there patients with granulomatosis with polyangiitis on maintenance rituximab therapy for whom you do not co-administer glucocorticoid therapy?

Would you stop azathioprine in a patient with ANCA vasculitis who has been in long term remission on azathioprine, but has a new diagnosis of lung cancer requiring initiation of immunotherapy?

When is brain biopsy useful/warranted as part of the diagnostic work-up in patients with suspected primary CNS angiitis?

How long do you continue PJP prophylaxis in a patient with GPA who is able to wean steroids and remains only on rituximab for maintenance therapy?

Do you extend the duration of maintenance therapy past 24 months for patients with ANCA glomerulonephritis who have multiple organ involvement?

What is your approach to maintenance treatment in a patient with Sjogren's who received rituximab for mononeuritis multiplex?

And how would you monitor response?

How would you approach the work up of a patient with nasal septal perforation, a negative infectious workup, and negative ANCA titers?

Do you typically screen every patient with headaches after the age of 60 with ESR?

6 Answers available

How do you approach an older male with a chronic and persistent granulomatous cutaneous medium vessel vasculitis that has been unresponsive/worsened on high dose steroids, cyclophosphamide, and rituximab immunosuppressive agents with a thorough and unremarkable serologic, infectious, and imaging work-up?

How would you approach a patient with new temporal headache, temporal artery tenderness and TA biopsy with mild thickening, but normal inflammatory markers?

What else do you consider in the differential diagnosis for pulmonary-renal syndromes if there is low clinical and serologic evidence of AAV, Goodpasture's or other rheumatologic disease (SLE, RA, APS, Scleroderma)?

What is your approach to further workup and treatment of abdominal aortitis found on imaging in asymptomatic individuals with elevated inflammatory markers?

Is there a role in temporal artery biopsy?

How do you approach tapering immunosuppression in a patient with a history of Susac Syndrome who has stabilized on MMF and IVIG?

How would you treat a young patient who presents with coronary vasculitis and aortitis with persistently positive MPO but no other features of AAV?

How would you interpret a temporal artery biopsy demonstrating focal chronic inflammation in the adventitia associated with small adventitial vessels and nerves without inflammation of the intima and media and without giant cells?

Would you continue Rituximab infusions in a patient with GPA and renal involvement who has been in remission on Avacopan and Rituximab, but had PRES post Rituximab infusion?

How would you approach additional workup and management of a patient with active Crohn’s disease, who has multiple lung and brain nodules, with lung pathology demonstrating necrotizing granulomatous inflammation and brain biopsy with granulomatous inflammation and medium-large vessel vasculitis?

The patient is on mesalamine for Crohn’s and CellCept and HCQ for skin manifestations (currently on hold during workup). The infectious workup i...

Is there a role for monitoring serum ANCAs to assess ANCA associated vasculitis disease activity?

This question is part of a collaboration with RheumMadness and is in reference to Pathogenic ANCA (RAVE Trial).

Would you check ANCA titers in a patient with a history of PR-3-ANCA glomerulonephritis in remission and a stable creatinine but with recurrent microscopic hematuria?

6 Answers available

How would you approach peri-operative immunosuppressive management of a patient with Behcet's, currently controlled on azathioprine, who needs genital surgery?

The patient has not responded/has contraindications to apremilast, colchicine, and adalimumab. When they were off azathioprine for 5 days in the past,...

Do you recommend monitoring IgG level in patients with AAV receiving rituximab?

And when do you consider (if ever) IGRT?

Do you routinely obtain baseline vascular imaging (CTA, MRA, PET) in patients with suspected GCA, but negative temporal artery biopsy?

Per 2021 GCA Vasculitis Guidelines: low evidence, but conditional recommendation for CTA neck, chest, abd/pelvis. Is anybody following this or do you ...

How would you approach management of incidentally identified unilateral retinal vasculitis with subsequent labs revealing +P-ANCA?

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

What additional therapies would you consider in a pregnant woman with Bechet's on an antiTNF and azathioprine who has uncontrolled non-pulmonary large vessel vasculitis?

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 approach a patient with GCA who develops necrotizing fasciitis and then flares because they are off of tocilizumab?

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

How do you approach deciding how long to use Avacopan for ANCA-associated vasculitis?

The ADVOCATE trial only studied use for 52 weeks.

How long do you continue immunosuppression in patients with Behcet's who have a history of mucocutaneous and ocular disease, but are now in remission?

How would you approach a patient incidentally found to have PET avid large vessel vasculitis on CT angiogram during pre CABG workup?

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

What is your second line therapy for patients with EGPA with mainly pulmonary and sinonasal features who did not respond to mepolizumab 300 mg/month and still require high doses of steroid?

How would you approach persistent episcleritis/uveitis due to PR3+ GPA, which needs low dose prednisone to control, despite induction with Rituximab?

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

Would you consider re-challenging a patient with rituximab after they experienced delayed neutropenia?

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

What is your approach for induction therapy in dual positive anti-GBM/PR3 disease with ESRD presenting with recurrent DAH previously treated with cyclophosphamide?

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

Do you continue to utilize ESR and CRP in patients on tocilizumab?

How would you taper tocilizumab in a patient treated with the GIACTA 26 week steroid and tocilizumab protocol?

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

How do you approach using CD 19/20 levels to redose Rituximab for ANCA associated vasculitis?

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?

What is your approach to maintenance rituximab in patients with IgG4-RD?

How would you approach a patient with incidentally noted infrarenal periaortitis with positive C-ANCA, normal inflammatory markers and no systemic symptoms?

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

Do you routinely obtain a sural nerve biopsy in patients with EMG evidence of mononeuritis multiplex and clinically suspected polyarteritis nodosa?

What is your approach to timing of medications like Rituximab and Cyclophosphamide around plasmapheresis in patients on daily PLEX?

4 Answers available

Do you routinely screen for pulmonary artery aneurysm in patients with Behcet's?

Is IL-6 inhibition an option in patients who are going to be rechallenged with checkpoint inhibitors after previously developing ICI-mediated temporal arteritis?

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

Do you recheck screening labs (ESR, BUN/Cr, urinalysis, ANA, etc) for patients with recurrent small vessel vasculitis of unknown origin with each flare?

How do you approach treating idiopathic aortitis?

What are your primary and secondary agents?

How would you treat localized cutaneous PAN recalcitrant to high-dose steroids?

What is your approach to further workup, monitoring, and treatment of Sjogren's syndrome patients with positive cryoglobulins but no clear signs of vasculitis?

How would you approach a rising PR3 in a patient with limited GPA who is currently asymptomatic on methotrexate?

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

What is your approach to treatment for patients with microscopic polyangiitis who have sustained remission at 6 months after induction rituximab?

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

How would you treat systemic polyarteritis nodosa with orchitis, mononeuritis and glomerulonephritis in a person who is being treated for HiV with low level viremia?

How do you approach differentiating segmental arterial mediolysis from abdominal vasculitis?

What is your approach to the workup for an underlying systemic condition in a patient presenting with retinal vasculitis?

How would you approach the treatment of checkpoint-inhibitor-mediated temporal arteritis?

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

How important do you feel HLAB51 testing is in diagnosing Behcet's disease?

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

Would you consider using avacopan in PR3+ mononeuritis multiplex without other systemic involvement?

How do you approach relapsing ANCA-associated vasculitis in a patient who has already undergone kidney transplant?

Can giant cell arteritis present with a partial cranial neuropathy?

How do you approach treating cutaneous PAN?

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

Do you generally recommend low dose aspirin for patients with giant cell arteritis?

Would you advise a patient with quiescent vasculitis who flared with the first Covid mRNA vaccine to obtain an additional dose?

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

What is your approach to initial assessment of disease extent in patients with Behcet's disease?

How does this vary from continued disease monitoring?

How would you approach new-onset large vessel vasculitis in a young patient with Crohn's disease?

Do you always perform temporal artery biopsy in patients with positive inflammatory markers and high clinical suspicion of GCA?

Is either ESR or CRP more sensitive or specific for the diagnosis of GCA?

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

What is the utility in repeating a temporal ultrasound (US) in a previously US diagnosed positive GCA patient who has received treatment and is presenting with recurrent GCA symptoms?

Is there any experience of using sarilumab instead of tocilizumab for steroid sparing effect in GCA during national tocilizumab shortage?

What is the role for angiography in the workup of digital ischemia?

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

What is your steroid-sparing agent of choice to treat GCA given the current tocilizumab shortage?

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

What is your approach to checking serum vWF antigen levels in adult patients with CNS vasculitis for monitoring disease activity?

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

Would you pursue temporal artery biopsy in patients with PMR who develop cranial GCA symptoms and have been on PMR dose steroids for one year?

How long do you continue steroid-sparing agents such as tocilizumab for GCA once the disease is in remission off steroids?

How do you approach treatment of vasculitis in a sickle cell disease patient?

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

How do you approach patients with ANCA vasculitis who had partial response, but continue to have disease activity on q 6 month maintenance rituximab and remain on steroids?

Would you change rituximab maintenance dose or schedule?

How do you choose between oral and IV cyclophosphamide for the treatment of ANCA-associated vasculitis?

What is your approach for treating mononeuritis multiplex in patients with ANCA vasculitis?

Specifically, do you reach for Rituximab or cyclophosphamide?

Would you start tocilizumab in a patient with temporal arteritis and cirrhosis?

Should the use of avacopan be limited to those patients at increased risk of steroid toxicity given the anticipated high cost of this medication?

ADVOCATE trial

How do you treat an AAV patient with recurrent bronchial stenosis that has not responded to rituximab induction therapy and dilatations?

When would you use tocilizumab to treat Takayasu's?

Are there features of a nerve biopsy that help distinguish late nerve damage from vasculitis versus from a demyelinating disease?

How should the results of the ADVOCATE trial be applied in AAV patients who receive rituximab induction and maintenance therapy?

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

Are there particular subsets of AAV patients in which avacopan is more effective?

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

Do you treat HIV/AIDS-associated CNS vasculitis with antiretrovirals alone or in combination with steroids?

How do you monitor response to treatment in patients with mononeuritis multiplex secondary to vasculitis?

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 ...

How would you approach refractory gangrenous Raynaud's secondary to type one cryoglobulins?

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 approach a patient with MPO-positive isolated peripheral nerve vasculitis?

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

Are there particular concerns related to the use of B-cell depletion therapies such as rituximab for rheumatic disease during the COVID-19 pandemic?

Should all patients with suspected giant cell arteritis get a PET scan to look for large vessel disease?

Are there AAV patients for whom you would consider combination therapy with cyclophosphamide and rituximab?

What is considered an adequate treatment period to assess response of AAV induction therapy with rituximab or cyclophosphamide?

How do you approach a patient found to have a coronary lesion suggestive of vasculitis on coronary CT?

Which EGPA patients are most likely to benefit from treatment with anti-IL-5 agents such as mepolizumab?

Should venous thrombosis in a patient with Behcet syndrome be treated with both immunosuppression and anticoagulation?

How do you screen for colon cancer in patients with Behcet syndrome with colonic involvement?

How do you handle hypogammaglobulinemia detected in patients prior to maintenance rituximab infusion?

At what point in GCA management do you typically introduce tocilizumab?

Which ANCA vasculitis patients are better candidates for cyclophosphamide rather than rituximab?

How do you manage incidentally identified chemotherapy-associated aortitis/large vessel vasculitis?

How do you counsel Takayasu patients on their prognosis?

Do you monitor RS3PE patients for GCA with the same vigilance that you do in PMR patients?

How does your treatment algorithm differ for drug-induced ANCA vasculitis compared to non drug-induced ANCA vasculitis in cases with severe/organ-threatening manifestations?

What agents can you use for treatment induction besides rituximab in cryoglobulinemic vasculitis (without hepatitis)?

Are there certain subsets of ANCA vasculitis patients for whom you would consider life long maintenance therapy?

When is a biopsy necessary to diagnose relapsing polychondritis?

What is your approach to bladder cancer surveillance in patients who have received cyclophosphamide?

What do you use to treat a GCA patient refractory to methotrexate who responded but had adverse effects with both tocilizumab and sarilumab?

What clinical factors do you use to determine timing of on-demand dosing of rituximab in ANCA vasculitis maintenance therapy?

Do you recommend on-demand versus fixed-schedule dosing for rituximab maintenance in ANCA vasculitis?

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

Is there a role for tocilizumab in treatment of polymyalgia rheumatica in patients who do not have features of giant cell arteritis or rheumatoid arthritis?

When do you stop immunosuppressants in patients with GPA?