Subscribe Now

By entering these details you are signing up to receive our newsletter.

RARE REV-inar episode 002

ANCA-associated vasculitis webinar

RARE REV-inar Episode 002 ANCA-associated vasculitis. Hear from Dr Duvuru Geetha and Dr Alvin Wells on an overview of ANCA-associated vasculitis and the impact on patients and families living with this rare disease. Sponsored by Amgen.

RARE REV-inar Episode 002 ANCA-associated vasculitis. Hear from Glen Massie, patient and advocate, about living with ANCA-associated vasculitis and the impact on him and his family living with this rare disease. Sponsored by Amgen.

RARE REV-inar Episode 002 ANCA-associated vasculitis and it’s impact on patients and families. Hear from Joyce Kullman executive director at Vasculitis Foundation the impact on patients and families living with ANCA-associated vasculitis and the support programmes they have in place. Sponsored by Amgen.


Welcome to the second in our series of RARE REV-inars.

Today at the topic is ANCA-associated vasculitis and its impact on patients and families and

we’re bringing this to you in partnership with ChemoCentryx. RARE REV-inar Archives – Rare Revolution (

I’m Geoff Case digital editor at RARE Revolution magazine, my colleague Becky Pender is also

with me busy behind the scenes taking care of the all-important technical aspects.

It’s fantastic to have so many people register for today’s webinar there isn’t a live Q+A

but please do feel free to put questions into the chat, these will be answered individually

if possible or through the article that will be published following the webinar.

Today’s session will also be available to re-visit on demand. We’ll share links for that in due course. We’re fortunate to have with us today an excellent panel so we can look at the topic from different perspectives Dr Duvuru Geetha, Professor of clinical medicine at John Hopkins University School of sorry Johns Hopkins University School of Medicine, Glenn Massie patient advocate, Joyce Kullman executive director at Vasculitis Foundation. Before we hear from our speakers let’s start with a few words from Brian Tumminello to get us a little bit of background about ChemoCentryx.

BRIAN TUMMINELLO ANCA-associated vasculitis webinar

Thank you Geoff, first I would like to say thank the RARE Revolution team for inviting 

me here today and for organizing an excellent webinar. As Geoff mentioned my name is Brian

Tumminello, I’m our senior medical director here at ChemoCentryx which is now part of

Amgen within our medical affairs family. Just a quick background on ChemoCentryx and

then a few words on Amgen and then I’ll turn it back to Geoff.

ChemoCentryx  it’s a bio pharmaceutical company that was founded about 25 years ago 

based in San Carlos, California by a brilliant researcher named Thomas Schall. We’re focused on discovering and developing orally administered therapeutics to treat autoimmune 

diseases, inflammatory disorders and cancer. And we’re primarily focused on orphan and

rare diseases.

We were founded or sorry we were actually acquired and we’re part of the Amgen family

as of last week so this is kind of news hot off the presses, and Amgen, it has is really 

a premier biopharma company that was founded in 1980 and focuses on areas of

high unmet medical need and leverages its expertise with solutions that improve healthcare

outcomes and really dramatically improves healthcare lives. Amgen, it has a presence

in about 100 different countries and regions around the world and it reaches millions of

patients and really is developing a pipeline of medicines with breakaway potential so I’m

happy to be here today and and on behalf of the ChemoCentryx family we’re proud to be

part of the Amgen family so thank you.


Thanks so much Brian for setting the scene with that background the next part of the

webinar is about getting an overview of the condition to understand what ANCA-associated

vasculitis is so let’s turn to Dr Geetha with our first question. Dr Geetha can you tell

us a bit about ANCA-associated vasculitis and how it presents and manifests in patients?


Hi good morning uh good afternoon to everyoneand thank you all for joining. So just to give

a brief outline of what vasculitis is first before we jump onto ANCA-associated vasculitis.

So vasculitis is a general term that refers to inflammation of blood vessels leading to

narrowing, scarring or weakening of the blood vessels and it can pretty much affect any

blood vessel in the body so the symptom would vary from person to person and really depends

on what organ is affected.

ANCA-associated vasculitis is a vasculitis that affects predominantly small and medium-sized 

blood vessels and majority of the patients with ANCA-associated vasculitis have a circulating

protein that’s called ANCA that attacks the body’s own cells and tissues and that’s why

it’s an autoimmune disease so ANCA vasculitis commonly affects kidneys, lungs, skin, peripherals and joints and it is a serious but actually treatable disease so early diagnosis and treatment are really critical to prevent life-threatening complications and treatment often involves

using steroids with other medications that suppress your immune system and help to reduce

the inflammation. So one other point I really like to make is it’s very common for patients

with vasculitis to experience cycles of relapse and remission so it’s really really important

to have regular doctor visits and regular monitoring of your blood and urine tests.

That’s kind of like a brief overview and in addition ANCA vasculitis is a term that encompasses

three main diseases one is called the GPA or the granulomatosis with polyangiitis. The 

other one is MPA or microscopic polyangiitis and the other one is EGPA or eosinophilic

granulomatosis with polyangiitis.

Regardless of the name all of them are kind of treated similarly at least as of now. ANCA-associated vasculitis webinar


That’s super thank you so much. Dr Geetha – You’re welcome. Geoff – Now you mentioned how it’s multi-systemic condition. Does that have implications does it make it the condition

more difficult to diagnose and treat?


Yeah what we need to remember about ANCA vasculitis is number one is a very rare disease so you know it can be very hard for people to know because unless you have expertise in this

you’re not going to think about the condition. So in some of the studies that looked at the

median time to diagnosis it was about seven months and you can imagine what would happen

if there was a delay of seven months if someone has vasculitis of the kidney. Because it’s very

rapidly progressing right so the rarity of the disease makes it a really diagnostic dilemma and leads to delays in diagnosis. Secondly, ANCA vasculitis can actually have symptoms that mimic other common diseases right so many patients can just be feeling lousy they can have fevers, night sweats, weight loss. So often times other rheumatologic diseases or infections or cancers or allergies can present the same way so it’s really hard for a primary care physician who has not seen a case of vasculitis to actually think about this condition. And thirdly as you mentioned

it affects multiple organs so some patients are seen by multiple specialists for months

before a diagnosis is considered. They maybe see an ENT doctor and go through five

rounds of antibiotics before a diagnosis of vasculitis is considered. So the multi-system

nature, the rarity of the disease makes it a real diagnostic dilemma and can lead to

delays in diagnosis. And that’s why collaboration among specialists is really important here.


Okay that’s great so I’d also like to look at you know the the challenges and gaps that exist in the care pathways. So perhaps we can turn our attention to that so again with you Dr Geetha for now, what do physicians look for in a person when they diagnose ANCA-associated vasculitis?


Most majority of the patients can just come and say they just don’t feel well. A lot of times they say “I’m not feeling well” and then you specifically ask they say “I haven’t been eating, I don’t have energy, I’ve lost weight, fevers or very nauseous” with symptoms sometimes and are other times depending on the organ that is involved you know if there’s some sinus involvement they’ll say I’m bleeding from the nose, crusting from the nose or if they have lung involvement, shortness of breath or cough, or even coughing of blood with kidney involvement it’s kind of they don’t have any symptoms until it’s kind of like a really late stage so really for the renal disease, blood tests and urine tests are your first pointers, seeing blood or protein in your urine, seeing your creatine on the blood tests go up those are the early signs and if they have eye problems

they can have painful eyes, red eyes, loss of vision, they can have a skin rash if skin

is affected.

The nerves are affected, they can have tingling, numbness and loss of movement of you know

your hands or feet so it depends on the organ that is involved but sometimes that can be

non-specific as well. I mean I think the key is if someone comes with a systemic disease you know the more than one organ and one we have to think about vasculitis in addition to everything else like cancer, malignancy, rheumatic disease.


Okay a little bit about the challenges and gaps here in care pathways. In terms of, in terms of the collaboration maybe between colleagues across specialties when it comes to diagnosis.


So as you mentioned you know the multi-faceted nature of ANCA vasculitis is a challenge for

healthcare okay and given the multi-system involvement they’re often cared for by different 

specialists and the most commonly is the rheumatologists or nephrologists or pulmonologists or the ENT doctors are the ones that are you know caring for these patients and so diagnosing

and managing ANCA vasculitis actually requires a team of experts who are comfortable with

diagnosing the condition, managing and also have a knowledge of managing the complications

that are related to both the disease as well as the treatment.

ANCA-associated vasculitis webinar

So I think the collaboration really becomes important and it’s a really a central pillar in managing these patients. And also when you have good collaboration you know I may have an ENT colleague who’s seeing someone with sinusitis and he maybe think it’s a bacterial sinusitis and going through two or three rounds of antibiotics. I’m a kidney doctor and if I get urine tests, a blood test and notice that is blood or protein in the urine then my automatic next thing is to do an ANCA test on your kidney biopsy so I can help expedite that diagnosis of ANCA vasculitis that has not been considered. So I think collaboration is key both in diagnosis and managing these patients. It’s a challenge but also a key. Just because you know expertise among specialists is not available in all the places so you know often times patients have to travel a lot to see the specialists or the experts. Yeah and so I think education here becomes

very important both to primary care doctors as well as patients.


Okay that’s that’s great thank you yes and that of course all of that travel is quite a burden on patients um uh so um You’ve talked about the importance of collaboration um in or what what other challenges are there for HCPs healthcare professionals in care management for people with ANCA-associated vasculitis?


I think I mean one other thing is the level of education right among not only healthcare 

providers but also patients, most patients have limited information and that it’s really a knowledge gap regarding the type of information that the patients need. There’s only a few studies there was one year like 2014 which looked at the patients educational needs and the patients very clearly mentioned that they would prefer to get the information from healthcare providers. Both to internalize the information as well as to contextualize what the disease meant to their life. Right so they really want this to be part of that care, the fundamental part of their care. The patients really want more information and then I believe last year a group from 

Denmark or Netherlands one of them in 2021 they published a study they just interviewed like 10 patients with vasculitis looking for their educational needs and basically you know it came with three main areas where we need to focus on information.

Number one you need to provide information whether it is written or oral. Number two focus on the content of information and number three you know learning their prerequisites you know what kind of psychosocial support do they need?

Things like that so those are important things for patients that we need to focus on, that is a real knowledge gap. I think improving patient education about vasculitis and management and how to cope with it, improving education to the primary care physicians as well as the specialists so you know they know when to refer to the expert.

I think that’s important I think those are important things to improve or move the field forward and improve the patient outcomes.


Okay. What other resources, I can I can see how improving a patients knowledge and confidence in their care is key. What other resources are available for patients to learn about their conditions? Support groups, non-profits, online forums?


Okay so with regard to that I think the Vasculitis Foundation, I’m sure Joyce is going to cover it

during her session. I would, I encourage all my patients to join the Vascilitis Foundation as well as to look on their website. It’s an excellent resource for everything, you know not only to know about the disease but even about trials and new, you know, new therapeutics that have been like approved, things like that and similarly just like Vasculitis Foundation the VF in Canada is Vasculitis in UK I don’t know the exact name and they all have links to the support groups you know there’s a GPA support group. I think Lauren Currie Foundation is another one I think in the UK that they have so lots of foundations you know who will link you to support groups and I think patients get a lot of information.

I think during the COVID those kinds of social networks really help the patients. 


ANCA-associated vasculitis webinar

Dr Wells there were some questions that I was um hoping to put to you so let me you know stay staying with this introductory section or sections about ANCA-associated vasculitis from the HCP perspective let’s talk a little bit about remission. Firstly what’s your definition of remission



So yeah remission is patients have a little clinical symptoms like Dr Geetha is talking about they have skin, lung or kidney disease. You want to get those things as as close to normal as possible and then also aim for remission in the setting of our lowest possible dose of corticosteroids and indeed we know for every one milligram increase in corticosteroids 

you know you ended up with other side effects. We’re looking at now the glucocorticore toxicity 

score and it looks at 12 different organs, so somebody has cataracts, someone that gets diabetes, somebody has osteoporosis etc and all those things I think in as we go to 2023 are


So we’re looking for a mechanism to control the disease without having side effects that’s 

due to the medication and that’s the challenging part that we have as rheumatologists and we

indeed just like Geetha said we work very closely with our primary care physicians. I see my

patients um I’m busy now but virtually every three months and then at least every six to nine months physically in the clinic. But in between those times they’re getting their labs done, they’re getting their urine done, they’ll get any imaging. All those things we need to make sure the disease under control.

So for me remission is going to be few clinical symptoms, very few flares in the setting of low corticosteroids.


Oh great so that’s very clear thank you um are, do you refer to treatment guidelines when defining remission? 


You know we did not in the clinic we do not because the guidelines a little cumbersome to use on a on a daily basis now when I do the clinical trials of course we do so that we make sure hey what I’m doing in Milwaukee, Wisconsin is the same in Stockholm, Sweden. So to make sure all the patients are uniform across the board and indeed that’s what we do when we do clinical trials. But in a busy practice you know for me I only spend 15 minutes with the follow-up

patient but before I get on with them in 15 minutes I have the blood work I have the imaging

I have their urine I have all those features that we need and then those 15 minutes to

say “Hey how are you doing? Have you met the goals that you want? Are there any new problems or other symptoms that are going on? And we kind of go from there.


ANCA-associated vasculitis webinar

Okay and the BVAS the Birmingham vasculitis activity score is that uh something you use

To confirm remission?


Yes we use that as more of a kind of gestalt and do we do all the specific measures of it but I think that’s a very very nice measure I call it a kind of a ruler or a yardstick and that’s why many people try to go on aim for. We’re not able to get there in all of our patients but that’s really truly I think an aim and that’s a reasonable one I think that people should have in mind.


Okay thank you okay so I think you touched on this already but just to perhaps recap a little bit.

What labs, clinical presentations do you look at?


I think you touched on already so I’m definitely working looking at the CBC the blood count because indeed some of our medicines are the immunosuppressive guess what they’re going 

to lower the blood count we aim for it to get their white cell count down because that drives the disease but I don’t want to go too low because I’m worried about infections we have to think about COVID, we have to think about bacterial infections all those things down the road.

I’m also looking at the liver function. We know that some of these diseases can attack the liver so we’ll make sure they don’t have an involvement or overlap with autoimmune hepatitis and we always tell people these autoimmune diseases run in families. So one disease, once you have one disease you can get others. We kind of look at that. We also follow the creatinine, many of the drugs that we use they are metabolized by the kidneys.

We want to make sure that creatinine stays normal. We also know the creatinine itself can be compromised just from the inflammation from the disease itself. So that’s some of the things that we want to look at. So I do a blood count, a creatinine, liver function test, and then I’m looking

at my inflammatory parameters, my sedimentation rate, my c-reactive protein, to see hey where

that patient is in that inflammatory pathway.


ANCA-associated vasculitis webinar

Okay thank you what is the difference between remission and complete remission finally please?


Yeah so it it’s almost analogy we use with rheumatoid arthritis, we have some patient with rheumatoid arthritis as we talk about complete remission that means they have no clinical signs, they have no laboratory signs, and they have no imaging signs of remission so I’ve almost kind of borrowed those for the same thing when I’m talking about somebody who has an ANCA-associated vasculitis. They have no clinical signs of disease, their imaging is almost normal okay not worse and so often I’m doing a CT scan to the lungs. I’m looking at imaging of the kidneys. I have you know looking at the skin and then also we talk about hey what are the laboratory studies look like so clinical, imaging and laboratory, all those together give you complete remission.


Okay um just uh briefly on disease severity. How do you define disease severity? Can a localized disease be severe?


Yes and I tell you that’s what the patient makes in their mind could be one. So somebody hey with uh let’s say that EGPA they can inolve the sinuses uh they have severe sinus problems.I mean were they requiring inhalers and all these different things that are used to get them under control. They require multiple medications to control those symptoms and on top of that they might get a multiple infections so we have to control those. So even though it might be localized that could be severe in that individual patient so I know when guidelines come out they say hey this drug is approved for moderate to severely active vasculitis. Moderate for one patient could be severe for the next and that’s where the job as a physician comes in to help kind of balance that out and see which of these patients afford and demands aggressive therapy.

ANCA-associated vasculitis webinar

Resources – Rare Revolution (

RARE REV-inar Archives – Rare Revolution (

Skip to content