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Participants:
Ed: PRSN Moderator
Dr. Kathy McKinnon: Guest lecturer; rheumatologist from UPMC
Dr. Eric Hoy: PAN advisor
Mary: RN & PAN volunteer
Mark: PAN patient
Cindy: MPA patient
Linda: RN & PAN volunteer
Don: PAN patient
Jess: PAN patient
Ottavio: PAN patient
Key topics discussed: Vasculitis & research, new medications, Q&A w/Dr. McKinnon.
Ed Moderator: Hello Dr. Kathy. Welcome and back by popular demand. Thanks for joining us tonight, Dr. Kathy. We'll start in a few minutes. Dr. Eric should be joining us shortly.
Ed Moderator: We ll start off with introductions, just jump in. I m Ed your Moderator in Pittsburgh
Mark: Mark from Kentucky
Kathy McKinnon: Kathy McKinnon, Pittsburgh, rheumatologist.
Mary: Mary from Cranberry.
Cindy: Hi there! Cindy from Chicago.
Jessica: Jessica from Pittsburgh.
Ed Moderator: Cindy you have MPA, correct?
Cindy: Yes.
Ed Moderator: Jessica, you have PAN?
Jessica: Yes.
Ed Moderator: Well, I'm going to do a questions and answers with Dr. Kathy for a few minutes. Just her and I.
Ed Moderator: Dr. Eric from Texas, an immunologist has joined us.
Ed Moderator: Dr. Kathy and I will chat for a few minutes and then open to questions. Remember, if you have a question raise your hand by typing a !. I'll call on you and most importantly, remember to put a # when you finish your thought. Just like this# Okay, welcome back Dr. Kathy. It has been awhile. How are you doing?#
Kathy McKinnon: Things are good here, good to be back.#
Ed Moderator: I understand you went to a conference not too long ago, a few weeks ago. How was that?#
Kathy McKinnon: Very interesting. Progress slow but continuing in vasculitis research.#
Ed Moderator: What are some areas of "excitement" in vasculitis research, Dr. Kathy?#
Kathy McKinnon: Areas of excitement include new medications, and more research about why people develop vasculitis.#
Ed Moderator: Can you share anything about new meds on the horizon?# I guess the aim is to get less destructive meds#
Kathy McKinnon: Some of the treatments being studied are biologic agents. The hope is that we'll be able to better target the culprit cells, and not cause so many side effects with newer treatments.#
Ed Moderator: How about any environmental links? I get this question all the time; someone in our group, a doctor with vasculitis has begun researching into arsenic in the water supply exposure.#
Kathy McKinnon: There have been some small studies looking at toxin exposures, and some looking at infections, but nothing conclusive yet.
Ed Moderator: I guess silica is one of the environment s suspects and still lots more research, I guess#
Kathy McKinnon: I get questions about food preservatives, environmental pollutants, hopefully we'll know more about this aspect soon.#
Ed Moderator: Last question for now, from me. You know, I was thinking that you or I suppose any doctor for that matter is much like a detective. You get a list of symptoms and then you have to try and find a diagnosis. When you have a patient come to you with two symptoms that appear to be vasculitis. But four or five that aren't, how do you proceed? How do you know what path to go down?#
Kathy McKinnon: There are a number of conditions that can be associated with vasculitis. Vasculitis can be primary, like PAN or be secondary to other autoimmune diseases, like Lupus, Sjogren's, Rheumatoid arthritis. Vasculitis also can come from infections or cancer. Vasculitis is just a starting point. Looking for a reason for vasculitis is often the next step and often it's what my patients tell me about their health, other symptoms, other conditions, medications, can help us get started down the right path.#
Ed Moderator: This may sound like a dumb question but I've been known to ask. But is there a test, and if so what is it that you finally achieve a definite diagnosis of PAN. Is it a biopsy or when the cumulative evidence and test results is when you can say to the patient, "you have vasculitis." #
Kathy McKinnon: That's a very good question Ed. Vasculitis can be a very difficult diagnosis to make, as those of you who have it know. And often it's what my patients tell me about their health, other symptoms, other conditions, medications, can help us get started down the right path. Especially for PAN, it tends to be a summary of the evidence that leads to the diagnosis. A biopsy or angiogram is very helpful but eliminating an infectious cause is important.
Ed Moderator: But those alone won't be the basis to make a definite diagnosis?#
Kathy McKinnon: No, it is a combination of the biopsy/angiogram, clinical history, and blood tests all put together that help make the diagnosis.
Mark: !
Ed Moderator: Okay Mark, I'm ready to open it up for questions. Remember to type a! and I'll do my best to call on you in turn. Mark, go ahead#
Mark: Kathy, have you ever diagnosed a patient with PAN, without a positive biopsy or a positive angiogram#?
Kathy McKinnon: One of the hallmarks of PAN is vascular involvement, so it would be hard to make that diagnosis without establishing in some way that the blood vessels are abnormal. Sometimes biopsy of other tissues, like a nerve or muscle biopsy can point us in that direction though. There have been some small studies looking at toxin exposures, and some looking at infections, but nothing conclusive yet. But eliminating an infectious cause is important.#
Mary: !
Ed Moderator: Go ahead, Mary#
Mary: So, Dr. Kathy, if you have a patient's clinical history, symptoms and recent labs, is it safe to say you need a biopsy to confirm a diagnosis of vasculitis? Or could it be a secondary diagnosis to say an auto immune disorder?#
Kathy McKinnon: Even if a patient fits many clinical criteria for vasculitis, since the treatments can have serious side effects, and vasculitis can have many mimics (other diseases that look like vasculitis), we usually do get a biopsy to confirm the diagnosis. Sometimes we have diagnosed underlying infections or found evidence of another autoimmune disorder that way.#
Mary: So is a skin biopsy going to help with the diagnosis?#
Kathy McKinnon: A skin biopsy can be very helpful. We have found other autoimmune conditions, like lupus, sarcoidosis, and other diseases that way.#
Don: I saw mention of silica, I used it for 6 years, also worked at Beaver Valley nuclear plant for 5 years. My father was also exposed to high levels of cadmium do any of these lead to vasculitis.#
Kathy McKinnon: Re: Don's question-silica has been implicated in a number of autoimmune conditions.#
Ed Moderator: Hello Ottavio, welcome.
Ottavio: Ciao
Ed Moderator: What is your first name Ottavio and. where are you from?#
Ottavio: Comprendi. Italy.
Cindy: The doctor mentioned the new biological therapies--what are the side effects that they are currently aware of with those?
Kathy McKinnon: By looking at other signs and symptoms of their vasculitis (joint pain, cough, nasal crusting) and also looking at blood tests, urine tests, x-rays/CT scans. As far as new treatments they also cause immunosuppression-leaving you at risk of infections, but so far seem to not have the serious toxic effects of drugs like Cytoxan#
Cindy: Are they linked to cancers?#
Kathy McKinnon: Some of the newer agents are actually treatments for cancers-like Rituxan-is used for the treatment of lymphomas.#
Cindy: I understand that the costs of these drugs are quite high. Will that impact there use once the trials are done?#
Kathy McKinnon: It seems like the more data that we get supporting their use; the more likely insurance companies are to pay for them. I have been able to get almost all treatments by either petitioning insurance company or calling the drug company.#
Linda: I got Rituxan although Medicaid didn't pay for it. The doctor s office manager found an assistance program for Rituxan. I was poor enough and sick enough to be eligible for it.#
Ed Moderator: So there are assistance programs out there, you just need to dig for them I guess#
Linda: But it took her 2 months to find the program and if I was eligible. I wish I knew what program it was to tell others.#
Kathy McKinnon: Often times it is a battle. More and more companies are offering compassionate use assistance, thankfully.#
Ed Moderator: What is compassionate use assist? The insurance companies grant it without knowing a lot about the drug? They don't fight it?#
Mary: The drug companies provide the assistance, not the insurance companies#
Jessica: Those companies only do that for a short time span, though.#
Kathy McKinnon: I have a patient who has been on over a year now, and they've continued to supply the medication for her. Depends on the company. Insurance companies seem to do everything not to pay for these medications.#
Linda: But if you have insurance, including Medicaid, it won't help. Even if the insurance companies won't pay.#
Kathy McKinnon: Drug companies will help, even for patients with insurance. We've had patients with good jobs, decent insurance and the company has supplied drug knowing that most people can't afford out of pocket.#
Linda: Sounds like you help your patients with this, so many doctors don t help.#
Kathy McKinnon: And yes, Linda, I do get involved with the medication approval. Sometimes it can make a difference.#
Linda: Any tips on whether it's a flare or the symptoms are worse due to a sinus infection?#
Kathy McKinnon: I usually approach this with my patients by looking at other signs and symptoms of their vasculitis (joint pain, cough, nasal crusting).#
Ed Moderator: Mary, you had another question?#
Mary: Dr. Kathy, is it true that some patients with auto immune diseases are more at risk for lymphoma?#
Kathy McKinnon: This is true. We know lupus, Sjogren's, rheumatoid patients with increased risk of lymphoma and other cancers.#
Mary: Is there a percentage? Or do you just wait for symptoms to appear?#
Kathy McKinnon: As far as cancers, we have all patients get routine cancer screening; mammograms, PAP smear, colonoscopy (at age) and see and examine them regularly. And if any worrisome symptoms are more aggressive about sending them for biopsy, cat scan, etc.#
Linda: In Sjogren's disease, they say lymphoma risk is 44x normal?#.
Kathy McKinnon: Well, most patients with these diseases don't get lymphoma, but that doesn't help if you are one of the few.#
Linda: But is the risk still small? That is, at 44x normal, is that tiny risk still small?#
Ed Moderator: Dr. Kathy, is it a chicken or egg? I mean does cancer more often lead to vasculitis or vice versa?#
Kathy McKinnon: We know that the immune system is abnormal in patients with autoimmune diseases and the immune system is what helps protect you from cancers. In addition, drugs that we use to treat these diseases like Cytoxan can also increase the risk of cancer.#
Linda: But not in the same way that would increase cancer risk? Immune system abnormalities, I mean.#
Kathy McKinnon: But in some patients, they had cancer first, and then vasculitis afterward. So it's likely that the abnormalities of the immune system are an important part of the cancer risk. The treatments, in some cases, increase this risk.#
Linda: Although, I forgot and I have it. Common variable immune deficiency and autoimmune disorders and they often go together.#
Kathy McKinnon: Yes this is true.#
Ed Moderator: Does anyone else have questions for Dr. Kathy?#
Jessica: I do#
Ed Moderator: Go ahead Jessica#
Jessica: Does having PAN increase my chances of getting Endometriosis?#
Kathy McKinnon: There isn't much information about this in PAN.#
Jessica: I have it, I just don't know why. I just know I have to take pills.#
Linda: That's the chicken and egg; Ed. Which comes first, autoimmune problem or immune deficiency?#
Kathy McKinnon: But I have seen quite a bit of endometriosis in my patients with autoimmune disease.#
Ed Moderator: Any thoughts on why that might be, Dr. Kathy?#
Kathy McKinnon: I think the theme of the night is the abnormal immune system. But also, a hormonal relationship.#
Jessica: I don't want my bulimic uterus to be an immune problem, too.#
Linda: Don't they think endometriosis may be autoimmune problem?#
Kathy McKinnon: I don't think anyone is sure about that. But there are clearly connections between hormones and autoimmune diseases. Diseases like lupus, Takayasu's arteritis very female dominant diseases. But I don't think anyone knows for sure about endometriosis yet, Jessica.#
Linda: So sometimes people think estrogen is a culprit. But I have estrogen deficiency, since age 32 and 3 autoimmune disorders.#
Cindy: My question is whether the numbers of patients with vasculitis will be enough to put any treatment on label?#
Kathy McKinnon: I doubt that most companies will fight for FDA approval for many of these medications just because of the rarity of these diseases, but as more information is published about their effectiveness in vasculitis, this seems to be an important means of getting them covered.#
Cindy: So insurance companies will always have an out.#
Ed Moderator: Actually, I was going to ask that of you Jessica, was it your mid teens right when you were diagnosed?#
Jessica: Yes. Most people get this mid-life; I got it when I was 11.#
Ed Moderator: Before we leave, Dr. Eric do you have anything to add to discussion? Can you tell anything from the lab world of research?#
Dr. Eric: Not a lot, Dr. Kathy is right about everything. We are looking at infections as a trigger and the biopsy is still the gold standard, but we're looking for less invasive tests#
Ed Moderator: When you say infection, do you mean from a cut or wound or from illness or both? #
Dr. Eric: Really from any source, we're looking at the common bacteria, staph and strep, and lots of viruses.#
Linda: Which infections so far? I know you've identified a few. I think it's certain pathogens. I had too many risk factors. Radiation. Polio.#
Dr. Eric: With the ANCA associated vasculitides, it appears that the activation of the neutrophils might be a trigger for the auto-antibodies, so we're looking for similar associations in other forms of auto immune diseases.#
Ed Moderator: Dr. Kathy, THANK YOU for coming here. You pulled in one of our biggest crowds ever. We appreciate your time and hope you'll come back again.#
Cindy: Yes, thank you!
Mary: Thanks Dr. Kathy!
Kathy McKinnon: Ed, thank YOU for all of your tireless work for us and everyone out there with vasculitis. I am grateful, and I know my patients are too!#
Ed Moderator: It's my calling and I'm glad to be doing it.
