At a recent Mission: Cure patient education webinar, Dr. Yasmin Hernandez-Barco of Massachusetts General Hospital and Harvard Medical School, joined by Katharine Provencher of the IgG4ward! Foundation, walked attendees through the essentials of autoimmune pancreatitis (AIP): what it is, how it’s diagnosed, and what treatment options are available today.
The conversation sparked so many great questions that we simply ran out of time to answer them all. We didn’t want anyone to leave without the information they came for, so we followed up with Dr. Hernandez-Barco to make sure every question got a response.
Below, you’ll find her answers to the questions we couldn’t get to during the webinar. Whether you’re a patient, a caregiver, or just trying to better understand a diagnosis, we hope this helps.
Yasmin Hernandez-Barco, MD
Medical Pancreatologist and Director of the Pancreatitis Treatment Center at Massachusetts General Hospital, Assistant Professor, Harvard Medical School
Diagnosis & Testing
Q: Does one have to have Pancreatic cancer to look into IGP IgG4 and/or ANA? If I have Severe Pancreatic Atrophy, & EPI since 2014 developed osteoporosis and diabetes?
No. If a patient has pancreatic cancer, this should be diagnosed and treated separately. Patients with pancreatic atrophy with EPI without a clear cause should be referred for genetic counseling, as there may be a genetic predisposition for “Idiopathic EPI.” Osteoporosis and diabetes may be related to the pancreas, if there is also chronic pancreatitis, or may be independent findings.
Q: I’ve been given a preliminary diagnosis of autoimmune pancreatitis, but the doctor who suspected it is still unsure, since the MRI with contrast appears normal. He referred me for neuroantigen testing — all neuroantigens came back elevated… Now he is sending me for a differential diagnosis of autoimmune encephalitis. What do you think about this situation?
I do not have expertise in autoimmune encephalitis.
Q: I suspect I may have type 2 AIP- It’s idiopathic CP currently. I don’t have any acute episodes anymore and most of my pancreas has atrophied except the head. No pancreas biopsy done other than an FNAC a few years ago (which was negative), and genetic results are negative and no other causes from the known causes list. I’m on treatment for EPI and type 3c diabetes. Is there a need to get a definitive diagnosis now, and if so, why? Will any biologics be used only in case of a potential flare, or can they be used to preserve any organ left (although I suspect not much organ function is left).
At this point, if it is Type 2 AIP, a diagnosis would not change management. If there is a chance this is Type 1 AIP, then knowing it is IgG4RD can make a difference because of other organs that might be involved. If truly idiopathic CP, you can consider genetic testing to understand why this might have happened, but it may not change your overall management since chronic pancreatitis has developed.
Treatment Options
Q: I was diagnosed with IgG4 AIP- first treated with stents, then steroids, then azathioprine for a few months, and then experienced sepsis. I was in remission for 3 years after that with dietary changes and rest before another flare last fall. Some questions…
a) Is there an increased risk for sepsis when treated with rituximab or inebilizumab? I had a bout of sepsis while taking azathioprine.
Yes, you can have an increased risk of infection with any immunomodulators/b-cell depletion. Knowing your infectious history and other comorbidities is very important to share with any provider that will prescribe any medications.
b) Are there any precautions to either of those meds for someone with Factor V Leiden and taking antiplatelet and blood-thinning prescriptions?
I would recommend asking this question of a hematologist.
c) I’d also like information about potential connections of involvement with the brain. Specifically, can IgG4 RD involve lesions to small blood vessels? After years of stability, I have evidence of an increase that occurred during the timeframe of my initial diagnosis and recent flare last September.
There are several known neurological manifestations of IgG4-RD. if the disease is well controlled, it should not flare. It is possible some of the symptoms are related to the steroids?
d) I have been in search of the right physician for a second opinion since my latest flare last Sept. I have been on prednisone since – weaned to a low dose until I have additional information. My specialists have described me as very complex, complicated, and in need of highly specialized care. I’ve had a very difficult time finding someone with experience. I’m wondering if Dr. Hernandez-Barco could possibly consult. I am based in Buffalo, NY.
I would recommend Dr. John Stone and his team at MGH as there appear to be multiple organs involved outside of just the GI organs.
Q: Are there any challenges going from Ritux to Inebilizumab?
(Another patient responded to this question with- I transitioned from Rituximab to Inebilizumab at my last infusion. I had 1 infusion of Rituximab before switching to Inebilizumab (and having 2 infusions of this). The only challenge I had was my post infusion fatigue was extreme and lasted longer than usual. Other than that, I haven’t encountered any other challenges.)
We do not have sufficient data about patients switching from Rituximab to inebilizumab at this time, but in theory as long as you wait 6 full months before switching, it should be ok.
AIP and Related Conditions
Q: I have been diagnosed with multiple Autoimmune diseases over the course of decades. Once my acute pancreatitis (9 years ago) was ruled to not be genetic by basic testing, I was told I have AIP several years ago. There was no treatment, other than the already advised lifestyle changes. I have total dependence on EPI and multiple pancreatic cysts that were followed by bi-annual EUS for multiple years. One year ago, I relocated and am now being followed with MRCP’s every 6 months. Would it be worthwhile at this point in time to seek out the other testing with my new GI? And if so, which ones?
It may be worthwhile to ask your new GI to review your prior history in detail and see if a cause of your pancreatitis can be found. If it was in fact AIP, depending on whether it is type 1 or type 2 there is treatment for it. If it is not AIP, then other causes should be evaluated. Pancreatic cysts are very common and understanding the features/size is important to know what time of cysts you have and what kind of follow-up. For example – an IPMN needs closer follow-up depending on the size and other features.
Q: Does having autoimmune pancreatitis or Severe Pancreatic Atrophy associated with IgG place stress on heart issues?
True IgG4-RD can involve the cardiac vessels and cause heart issues. Pancreatic atrophy by itself does not cause heart issues unless there is significant malnutrition.
Q: I had genetic testing because of the cysts on my liver, kidney, ovary, and pancreas to rule out Von Hippel-Lindau Syndrome. I did not have Von Hippel, but a CDC73 gene variant did show up. Does this relate to autoimmune diseases of the pancreas?
To our knowledge, there is no association with AIP at this time.
Q: I have type 2 pancreatitis and Crohn’s disease. I am currently on Rinvoq 15 mg daily and had done a course with Medrol 5 months ago. My questions:
a) I have severe acne that breaks out after severe anxiety/ fear or anger. I can’t stand it and I am trying everything to manage it and when it’s almost gone it comes back stronger…… Will it ever be gone 🙁
Hormones are closely associated with acne flares and breakouts. Medications such as steroids can also worsen acne as it impacts cortisol balance. Working with a dermatologist can be helpful to prevent flares and to have treatments in the moment that can help calm a flare of acne.
b) And also will I ever be able to drink alcohol again in my life with these conditions?
In general, because alcohol can impact the pancreatic function, we recommend avoiding alcohol in order to prevent additional damage to the pancreas.
Q: Should everyone impacted by IgG4RD be cautious of pancreas symptoms (what are the chances of me developing pancreatitis if I have IgG4RD in other organs)?
If your disease is well controlled, the risk of flaring is low in other organs. If you are being monitored off of treatment, the risk of flare in a new organ is as high as 30% over 3 years in patients that do have a flare. Much of this is based on how long you have already been treated for IgG4RD.
Navigating Care
Q: Will appreciate guidance for patients whose multiple providers pass diagnostic and treatment responsibilities off to each other – aka, hesitant to treat?
Sorry to hear this is happening to you. I would look for a GI specialist who feels comfortable managing pancreatic disease. If they are not, perhaps ask if there is a specialist/colleague that can help provide this longitudinal care for you. You can go to Mission Cure or NPF website and look for centers of excellence for pancreatic disease and see if there is one close to you.
Learn More About Autoimmune Pancreatitis
If you’d like to hear more from Dr. Hernandez-Barco, we encourage you to watch the full webinar, including the live Q&A session where she answered additional questions in real time.
Other Autoimmune Pancreatitis Resources
Keep Learning
Mission: Cure offers a growing library of free webinars, short form videos, blog posts, and tools to help people living with chronic or recurrent pancreatitis manage symptoms, reduce pain, and improve quality of life.
Check out our other resources featuring experts in pain management, nutrition, mental health, and more!
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