In this Mayo Clinic Minute, Jason Howland explores one of the rarer forms of vasculitis, Takayasu's arteritis.

Watch: The Mayo Clinic Minute

Journalists: Broadcast-quality video (1:10) is in the downloads at the end of this post. Please courtesy: "Mayo Clinic News Network." Read the script.

Takayasu's arteritis is a rare type of vasculitis, a group of conditions that cause blood vessel inflammation.

"It is in the category of large vessel vasculitis, meaning that it involves the aorta, that's the largest artery in the body, and its main branches," says Dr. Kenneth Warrington, a Mayo Clinic rheumatologist.

The inflammation causes the arteries to narrow or thicken. It can weaken blood vessel walls and potentially cause aneurysms, and eventually lead to stroke or heart failure.

"Takayasu's arteritis can be serious, particularly if not recognized or treated promptly," he says.

Dr. Warrington says early detection is key, but with a long list of symptoms similar to many other health issues, getting to that diagnosis isn't straightforward.

"Autoimmune diseases can be difficult to diagnose, but certainly need to be considered. So that, again, treatment can be started before there is progressive damage from the conditions," he says.

Takayasu's arteritis, like many other forms of vasculitis, is typically treated with drugs, like prednisone.

"In some patients were able to control it with medications, but there is still the possibility of progression of the disease and of flare-ups during the chronic phase of the disease," says Dr. Warrington.

Related post:

• Mayo Clinic Minute: How vasculitis affects the body

The post Mayo Clinic Minute: What is Takayasu’s arteritis? appeared first on Mayo Clinic News Network.

Watch: The Mayo Clinic Minute

Journalists: Broadcast-quality video (1:09) is in the downloads at the end of this post. Please courtesy: "Mayo Clinic News Network." Read the script.

Vasculitis is a group of conditions — all related to inflammation of blood vessels.

"White blood cells, which normally are circulating in the body and protect us from infection, become misdirected. And they actually invade and target the walls of blood vessels," says Dr. Kenneth Warrington, a Mayo Clinic rheumatologist.

The exact cause of vasculitis isn't fully understood, but in some cases, it might be genetic. It also could be triggered by an infection, cancer, immune disorder or a reaction to certain medications. The inflammation causes blood vessels to thicken and restrict blood flow, damaging organs and tissue.

"People may lose vision, there may be lack of blood supply to an extremity, or there could be organ failure, like kidney or respiratory failure," says Dr. Warrington.

Initially, the symptoms can be vague.

"Folks may just feel unwell. They may have a poor appetite. They may start losing weight," he says.

But as it progresses, more parts of the body will have symptoms.

"If there are multiple organs that are affected simultaneously, that's when we would suspect vasculitis," says Dr. Warrington.

Treatment is usually medications that target the immune system and reduce inflammation. Corticosteroids, such as prednisone, are the most common drugs prescribed.

Related post:

• Mayo Clinic Minute: What is Takayasu’s arteritis?

The post Mayo Clinic Minute: How vasculitis affects the body appeared first on Mayo Clinic News Network.

For Cori Kirkpatrick, going back to the place where her husband received his medical training brought with it a well of emotions — excitement, nostalgia, anxiety. Mayo Clinic had always represented possibility and hope for her, but those sentiments took on more meaning when Cori returned to Mayo searching for a solution to her own illness.

Corinne "Cori" Kirkpatrick remembers well the initial impressions Mayo Clinic left on her when she first arrived in Rochester, Minnesota, as the 25-year-old wife of a resident physician.

"The marble, glass and steel structure rising out of the cornfields was such an impressive facility," Cori says. "You just knew you were going to be well-cared for."

The three years in the mid-1970s that Cori and her husband, John, lived in Rochester were busy ones. Cori's life revolved around having and raising their children. John's days, and sometimes nights, were spent learning to care for patients in the collaborative, patient-centric care model established by the Mayo brothers a century earlier.

"This meant collegial, empathetic medical teamwork that put the patient's needs first and that never, ever gave up on an elusive diagnosis," Cori says.

Forty-five years have passed since Cori and John lived in Rochester. Following John's internal medicine residency at Mayo Clinic, the couple moved back to their hometown of Seattle. But the familiar aura of compassion and commitment was immediately apparent to Cori when she returned in January 2019 seeking her own elusive diagnosis.

"Your doctors were just going to keep after this until they figured out what was wrong with you and figured out what they could do about it," Cori says. "That sense was still there."

For Cori, those values of dedication and understanding shone through in the comprehensive testing and collaboration that ultimately yielded a name — giant cell arteritis — for the painful and debilitating inflammatory condition of the blood vessels that had been wreaking havoc on her life.

"To have a place like this to turn to when you're really sick and no one knows what's wrong, it's really something," Cori says. "I feel so grateful that I've been able to access care like this."

Alarming, rapid deterioration

The decades since she left Rochester were largely healthy ones for Cori. In spring 2018, however, Cori experienced an odd type of pain. Even slight exertion, such as walking, caused her calves to ache and her feet to go numb. But it usually subsided if she continued walking. The problem, Cori later learned, was called claudication — pain caused by too little blood flow to muscles during exercise.

Cori mentioned the symptom to her physician during a routine checkup in Seattle. At the appointment, her doctor recommended an ankle-brachial index. During the test, which is performed while the patient lies down, Cori's blood pressure was found to be extremely high. That led to an appointment with a kidney specialist, who recommended magnetic resonance angiography, or MRA — a test that uses a magnetic field, radio waves and a computer to evaluate blood vessels.

The images revealed that a number of Cori's arteries — the blood vessels that take oxygen from the heart to the rest of the body — were narrowed, impeding her circulation. Arteries to her kidney and bowel, as well as femoral arteries, were all affected.

"I was down to 113 (pounds), and I could not gain it back. It was awful. I felt just horrible."

Cori Kirkpatrick

The resulting diagnosis, atherosclerosis, couldn't have come at a worse time. Cori and John were about to embark on a European vacation with 16 members of their family to celebrate their 70th birthdays. "Everyone was hesitant. Should they send me (on the trip)? Should we do a surgery?" Cori says. "They ended up putting me on a statin drug, so I wouldn't develop further atherosclerosis, and on a blood thinner, and we went on the trip."

Cori made it through the trip with only slight changes to their itinerary. But upon her return, she became fatigued and lost her appetite. "Over the last year, I'd lost 10 pounds," Cori says. "I'm not quite 5'8" and weigh between 120 and 125. I was down to 113, and I could not gain it back. It was awful. I felt just horrible."

Cori stopped wanting to see friends, and she abandoned most of her usual activities. "All I wanted to do was read," she says. "I didn't feel like myself, and it wasn't normal for me."

During this time, John had been in communication with friends and medical colleagues who asked if they'd considered seeking a second opinion. The conversations ignited a spark that led John to reach out to an old Mayo Clinic colleague, who referred Cori to Mayo Clinic's Vascular Center. There she was seen by vascular specialist Ana Casanegra, M.D. and rheumatologist Kenneth Warrington, M.D.

Collaborative, comprehensive approach

When Cori and John arrived at Mayo Clinic in Rochester in January 2019 for a week of evaluation and consultation, a lifetime of experiences had changed them, but much about Mayo Clinic and Minnesota remained constant.

"We'd forgotten about Minnesota. That everybody there was so nice and helpful and sensitive and concerned, so that was really pleasant," Cori says. "The other thing that was fun to see again was the convergence of the people at Mayo. You stand in that lobby area by the grand piano, and you see every walk of life — and I think that makes it a really special place. You really feel like they're caring for the world there."

The spirit of collaboration among physicians also was unchanged by the passage of time. During the first week Cori spent at Mayo, a team of physicians, including vascular medicine specialists, rheumatologists, nephrologists and interventional radiologists, evaluated her.

That team approach was critical to identifying Cori's hard-to-diagnose illness. "She came to the Vascular Center, which is basically a clinic that takes care of blood vessel problems," Dr. Warrington says. "Within the Vascular Center is embedded a multispecialty Vasculitis Clinic."

"Collaboration and communication are our everyday practice."

Ana Casanegra, M.D.

Vasculitis was not immediately suspected when Cori arrived at the Vascular Center, Dr. Casanegra says. But a slate of tests, including blood tests, CT scans and MRIs, and an interdisciplinary review of the results, honed in on the illness. When a form of vasculitis was identified as the source of Cori's symptoms, Dr. Warrington, an expert in vasculitis and inflammatory conditions, was brought onto the team.

"We are very fortunate to have a Vasculitis Clinic in the Vascular Center," Dr. Casanegra says. "It makes a difference to work on an interdisciplinary team that can cover all the needs of patients with any type of vascular diseases. Collaboration and communication are our everyday practice."

In addition to the collaboration, blood work and advanced imaging technologies aided in Cori's diagnosis, "She had complex imaging tailored to evaluating patients with vasculitis," Dr. Warrington explains. "So the availability of that advanced imaging at Mayo Clinic is what allowed for a better diagnosis."

After the week of evaluation, Cori and John flew home to Seattle but still lacked a name for Cori's condition. A week later, she returned for additional testing. The second set of tests included another MRA. This one focused on the thoracic aorta, which is the primary blood vessel carrying blood from the heart to the rest of the body. The test revealed the wall of the thoracic artery was thickened and inflamed, but it did not contain any fatty deposits.

"That was the definitive test," Cori says. "They knew by seeing that. They knew something else was going on."

Unusual symptoms

The condition affecting Cori was giant cell arteritis. A form of vasculitis, giant cell arteritis is an autoimmune disorder that causes changes to the blood vessels that eventually can lead to organ damage.

"The moment when Dr. Casanegra said, 'We've figured it out,' I was so excited and grateful."

Cori Kirkpatrick

"Most patients present with cranial symptoms, such as headache and jaw pain with chewing," Dr. Warrington says. "Some people may lose vision or go blind, and that is why it's so serious. Ms. Kirkpatrick didn't have those symptoms. Her vascular problems were in the aorta and lower extremities. It was an atypical presentation which likely led to the delay in diagnosis."

Although Cori had never heard of giant cell arteritis, learning the name of the condition was a transformative moment. "The moment when Dr. Casanegra said, 'We've figured it out,' I was so excited and grateful," she says.

Cori learned that the condition frequently occurs in people who've also been diagnosed with polymyalgia rheumatica, or PMR. "I had no idea that one-third of the people who have PMR go on to develop giant cell arteritis. I had PMR in my 50s, and 15 years later developed giant cell arteritis."

Life-changing treatment

Treatment for giant cell arteritis has historically been glucocorticoid (steroid) therapy. To jump-start Cori's recovery, she immediately began taking daily doses of prednisone.

"Since the 1940s, prednisone was recognized as being very effective at alleviating patients' symptoms and at preventing blindness from the disease," Dr. Warrington says. "The main concern with prednisone is that it has a long list of possible side effects. So for the last 70 years, researchers have been looking for a suitable alternative."

In 2017, the first steroid-sparing agent for the disease that was approved by the Food and Drug Administration became available. "The medicine has a similar effect on the immune system as what we're trying to achieve with prednisone without as many side effects," Dr. Warrington says.

Cori started on that drug in May 2019. Delivered via an injection under the skin or as an infusion, the medicine doesn't offer a cure, but it reduces inflammation to the point where, eventually, a patient may be able to stop taking it.

Since beginning treatment, Cori's pain has decreased substantially. "It's not like it was before all of this started, but I feel so much better," she says.

Getting her health back has increased Cori's appreciation of life's little details. "I've had a really wonderful life, and I could not ask for more," she says. "I've got three great kids and eight wonderful grandchildren, and John and I have had a wonderful marriage. So as I was circling the drain I thought, 'If this is how my life is going to end, it's OK because I've been so lucky.' But then when you find out this (condition) is treatable, and you've got a lot of good days ahead, you really start appreciating just reading the Sunday paper together and lingering over coffee."

HELPFUL LINKS

• Learn more about giant cell arteritis.
• Read about vasculitis.
• Check out Mayo Clinic's Vascular Center.
• Explore Mayo Clinic.
• Request an appointment.

The post Medical Solutions Found Where a Medical Career Began appeared first on Mayo Clinic News Network.

These days, Molly Hanson feels awesome. Free from pain, she's now eager to get back into her life. Molly's energy today stands in stark contrast to how she felt a year ago — before she visited Mayo Clinic and learned an undiagnosed medical condition was to blame for many of her debilitating symptoms.

Molly Hanson's life was spinning out of control. In near-constant pelvic and abdominal pain, she was chronically constipated, got lightheaded all the time, had irregular heartbeats and kept breaking out in a rash. For close to a decade, Molly ping-ponged between health care providers, searching for answers to her persistent medical issues. Despite many diagnoses — including slow bowel transit and postural tachycardia syndrome — and a multitude of treatments, nothing provided lasting relief.

"It's almost like I kept being dismissed, like what I had going on wasn't an issue," says Molly, who lives with her husband in Glenwood Springs, Colorado. "Deep down, I thought I was losing my mind."

Although Molly lost faith that she'd ever feel well, she continued searching for a path to improved health. One of those paths led her to a conversation with a co-worker, who asked Molly if she'd considered seeking care at Mayo Clinic.

"I never thought that going to Mayo Clinic they would find anything wrong because I was used to never getting answers, but I was so hopeful," Molly says.

That hope compelled Molly to request an appointment, and in November 2018, she flew to Mayo Clinic in Arizona. There she met the first member of a multidisciplinary care team who finally connected the dots between her many symptoms and was able to provide her with effective treatment.

"I have been through the wringer," Molly says. "From doctors looking at me and saying, 'I don't know what's wrong,' or 'We don't think there's anything wrong,' or 'We can't fix you,' to feeling like it's in my head, then going to Mayo and telling them my story and learning that everything I have is so rare, but hearing them say, 'We know what is wrong.' Mayo Clinic has saved my life and changed my life in so many ways."

A complicated history

When Molly arrived at Mayo Clinic, she'd been waging a 10-year war against her digestive system and slow bowel transit. Her condition resulted in constipation so severe she could go for weeks without having a bowel movement. Although Molly won a battle in June 2015 with the surgical removal of most of her colon and connection of her small intestine to the sigmoid colon, the fight continued when constipation returned.

Dizziness, lightheadedness and irregular heartbeats landed Molly the diagnosis of postural tachycardia syndrome, or POTS — a condition that affects the way the heart beats when shifting from sitting to standing. Molly received prescription medication to treat it. She also received prescriptions for anxiety and ulcers, as well as Hashimoto's disease — a condition in which the immune system affects thyroid function. And Hashimoto's wasn't the only autoimmune condition Molly developed. Her body broke out in a rash, and she was diagnosed with vasculitis, an inflammation of the blood vessels.

"I'm on thyroid pills and not getting better. I'm on anxiety medication. My bowels are not getting better. My rash keeps coming and going. What am I supposed to do?"

Molly Hanson

On top of all that, the pain associated with constipation was compounded by several bouts of severe pelvic pain and pressure that were so excruciating Molly required hospitalization. "I tried Dilaudid, morphine, all this stuff went into my IV, but nothing dulled my pain," Molly says. Eventually, the pain dissipated. It routinely returned, however, and Molly was hospitalized four more times.

Molly sought advice from her gynecologist, who suggested the pain could be due to the colon surgery causing her to develop a tight pelvic floor. "At this point, I'm frustrated. I was mad," Molly says. "I'm on thyroid pills and not getting better. I'm on anxiety medication. My bowels are not getting better. My rash keeps coming and going. What am I supposed to do?"

The answer to that question came not from a doctor, but from the work colleague who suggested Mayo Clinic. It was some of the best advice she'd ever received, Molly says. "I am doing so much better because Mayo did it so much differently."

A comprehensive approach

That difference was evident during Molly's first Mayo Clinic appointment in November 2018 with Paul Snyder Jr., M.D., in Consultative Medicine. "I've never had a doctor honestly sit down with me and really listen and dive in and make me feel like I'm not losing my mind," Molly says. "Paul Snyder looked into my eyes and said: 'This is not in your head. There is something wrong, and we're going to find it.' It was just such a relief."

Dr. Snyder referred Molly to Mayo specialists in the departments of Gastroenterology and Hepatology, Neurology and Rheumatology. They ordered a slate of tests focused on her most troublesome symptoms.

During her Neurology appointment, Molly underwent a tilt table test that confirmed her POTS diagnosis. She also was tested for mast cell disease, or systemic mastocytosis, an autoimmune condition that can cause symptoms such as itching, flushing, rapid heartbeat and lightheadedness. The results of that test came back positive. But that wasn't the only surprising finding Molly's Mayo team made during her initial two-week workup.

"I felt so relieved that finally someone found something."

Molly Hanson

A CT scan to look for an intestinal obstruction revealed something else: a pelvic venous outflow obstruction. The anatomical condition, known as May Thurner syndrome, occurs when the left common iliac vein gets compressed between the spine and the right common iliac artery. That inhibits the flow of blood as it travels through the pelvis to the heart. Among the many symptoms caused by May Thurner are pelvic pain, abdominal swelling and blood clots, as well as lethargy, depression, headaches and nausea.

"I felt so relieved that finally someone found something," Molly says. In March, Molly made an appointment with Grace Knuttinen, M.D., Ph.D., in the Interventional Radiology Clinic to undergo diagnostic testing and determine the severity of the blockage. It turned out that Molly's case was quite severe.

Molly also spent time in March with Mayo gastroenterologists to address her persistent constipation. As part of those appointments, Molly underwent tests to examine her remaining colon. After the testing, she met with surgeon Tonia Young-Fadok, M.D., in Colon and Rectal Surgery, who explained that during Molly's 2015 surgery, a portion of her colon was left behind, and it had become dysfunctional.

"She explained to me that she was going to take out the remaining sigmoid colon, which is the last bit of colon I had left, and basically attach the small intestine to the rectum," Molly says. "With it, we wouldn't have to worry about the slow transit."

Learning she required a second colon surgery was unnerving at first, Molly says. "I was like: 'Are you kidding me? We have to go through surgery again?' But I trusted her. I felt comfortable with her. She was amazing."

A lasting solution

Molly, who works as a middle school financial secretary, scheduled the colon surgery during her summer break. But Molly was back at Mayo long before then. Just one week after she returned home in March, she received a follow-up phone call from Dr. Knuttinen about the May Thurner syndrome. Dr. Knuttinen described the severity of Molly's obstruction at 97%, and she explained that the blocked venous blood flow may be contributing to Molly's chronic pelvic pain.

Molly returned to Mayo in April for a stenting procedure to relieve the venous obstruction. It involved inserting a catheter through her groin into her left common femoral vein. Using intravascular ultrasound, Molly's team then assessed the length and severity of the obstruction and determined the size of stent needed to open it and allow for normal blood flow from the pelvis to the heart.

Normally, the stent placement for May Thurner is an outpatient procedure, but Dr. Knuttinen wanted Molly to stay longer than that. "I admitted her because I was unsure how her improved blood return would impact her nervous system as it related to her POTS," Dr. Knuttinen says.

"I just started to feel calmer. I had more energy and just felt like I was starting to feel alive."

Molly Hanson

During Molly's hospitalization, Dr. Knuttinen visited her twice a day. "She is the most caring, sweet, probably my favorite doctor that I've ever had," Molly says. "I had quite a lot of back pain for about a month after the procedure. But instantly, I started feeling relief (from my other symptoms). My vision improved. The heaviness I felt in my abdominal area was getting better. I just started to feel calmer. I had more energy and just felt like I was starting to feel alive."

Two months after she received the stent, Molly returned to Mayo for her colon surgery, which was performed laparoscopically. "The colon surgery went really good. Everything healed up really well," Molly says. "So far I've noticed a difference where I have a bowel movement, and it doesn't hurt."

Life-changing care

Although Molly's Mayo Clinic journey is not over — she continues to work with her Mayo endocrinologist and neurologist to fine-tune the treatments for Hashimoto's and POTS and will be regularly monitored for neurological changes related to the disease — the change in her life since her surgeries has been profound.

"Who would've thought, 'Let's get you some proper blood flow and see what that makes you feel like?'" Molly says. "I'm finally getting my POTS under control. We're readjusting my thyroid medication, so that's becoming more under control. My energy is up, and every day I'm getting better. I can't tell you how long it's been since I've felt like this. I am so grateful I was given the opportunity to be seen by such amazing doctors and nurses at Mayo."

"These patients are very unique and need to be evaluated, diagnosed and treated very carefully."

Grace Knuttinen, M.D., Ph.D.

While Molly's journey has been long and complicated, she is not alone, Dr. Knuttinen says. "Pelvic venous insufficiency is underdiagnosed, misdiagnosed and undertreated. The impact that pelvic venous outflow lesions can have on various organ systems in the body is complex and challenging to understand."

As a result of treating patients like Molly and realizing the complexity of the diagnosis and management of these patients, Dr. Knuttinen formed a multidisciplinary chronic pelvic pain conference held monthly with her colleagues from Gastroenterology, Gynecology, Radiology and Urology. It also includes pelvic floor physical therapy specialists.

"These patients are very unique and need to be evaluated, diagnosed and treated very carefully. It's very complex how all of these things are related. But it's fascinating how the pathophysiology can all be linked together in some degree and offer insight to the whole puzzle," Dr. Knuttinen says. "I give Molly kudos for never stopping until she found answers."

HELPFUL LINKS:

• Read about chronic pelvic pain.
• Learn more about postural tachycardia syndrome.
• Check out the departments of Radiology and Gastroenterology and Hepatology.
• Connect with others talking about women's health on Mayo Clinic Connect.
• Explore Mayo Clinic.
• Request an appointment.

The post Sharing Mayo Clinic: A puzzling problem with a precision care solution appeared first on Mayo Clinic News Network.

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Researchers found that patients with a higher amount or higher proportion of an inflammatory protein called type 1 interferon beta compared with another inflammatory protein, type 1 interferon alpha, do not respond as well to tumor necrosis factor inhibitors as others. They looked at white blood cells called monocytes, a major cell type involved in rheumatoid arthritis, and found that those cells behaved differently in one group than in the other.

The discovery paves the way for a more personalized approach to treatment in rheumatoid arthritis based on the biology of a particular patient’s disease.

“Investigating these pathways may identify other targets for therapy or other markers that predict treatment response,” says first author Theresa Wampler Muskardin, M.D., a rheumatologist at Mayo Clinic in Rochester, Minn. “It will help rheumatologists find the right drug for each patient and spare patients medications that won’t work for them.”

MEDIA CONTACT: Sharon Theimer in Mayo Clinic Public Affairs, 507-284-5005 or newsbureau@mayo.edu

The Rheumatology Research Foundation funded the study. Mayo rheumatologist Timothy Niewold, M.D., was the study’s senior author.

In other studies presented at the meeting, Mayo Clinic researchers found:

• Sarcoidosis, the growth of tiny clusters of inflammatory cells called granulomas, carries a higher risk of heart disease and venous thromboembolism. Researchers also found there is seasonal variation in the incidence of sarcoidosis: Rates are consistently lower in autumn. Physicians believe that sarcoidosis may be triggered by the body’s immune response to environmental factors, such as something inhaled from the air.

• The risk of coronary artery disease among patients with polymyalgia rheumatica is 70 percent higher than it is among others.

• Heart and circulatory abnormalities are common in Takayasu’s arteritis, a form of vasculitis in which large blood vessels become inflamed.

Mayo rheumatologists and cardiologists formed the Mayo Clinic Cardio-Rheumatology Clinic to research and pioneer better prevention, detection and treatment of heart disease and other cardiovascular problems in patients with rheumatic diseases.

###

About Mayo Clinic
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Granulomatosis with polyangiitis—yes, it’s a mouthful. It’s also a rare disease, afflicting as few as two in 100,000 people. It’s so uncommon a general practitioner might not recognize it. Says Fernando C. Fervenza, M.D., Ph.D., a Mayo Clinic nephrologist. “A lot of physicians down in the community are probably going to see one case in their lifetime.”

But granulomatosis can be deadly. The body’s immune system attacks itself, causing inflammation of small blood vessels, a condition known as vasculitis. And vasculitis can damage sinuses, lungs, kidneys, eyes, ears, skin, nerves, and joints. Until the 1960s “basically it was fatal,” says Dr. Fervenza, who is often called in on such cases when vasculitis inflames the millions of vessels that populate the kidneys.

Then came a drug called cyclophosphamide, which suppressed the white blood cells that multiplied to mount the body’s immune response. Cyclophosphamide tempered the body’s attempts to attack itself. “That was a revolution to treatment,” says Dr. Fervenza. An 80 percent mortality rate at one year turned to an 80 percent survival rate.

Yet there was a downside. Cyclophosphamide is toxic. Up to 20 percent of patients either don’t respond to treatment or can’t survive it. “Could we get better results,” Dr. Fervenza asked, “with another drug?” Other doctors wondered the same.

The pervasive nature of vasculitis cases brought Dr. Fervenza together with Ulrich Specks, M.D., the chair of Mayo’s Division of Pulmonary and Critical Care Medicine. “This is a multisystem disorder,” says Dr. Specks. “I would take care of their lung disease, and he would take care of their renal disease, and we would decide on the immunosuppressive therapy together.”

The patient without options

That is exactly what happened with a granulomatosis patient who had been treated with cyclophosphamide but relapsed four years later. When Dr. Specks first saw the patient in 1998 he complained of headaches, scalp tenderness, and large-joint pain. He was treated with cyclophosphamide. While his vasculitis abated, the patient became anemic, probably from the drug itself. Six weeks later he returned to Mayo Clinic with more joint aches, malaise, chills, night sweats, low-grade fever, an inflamed eye, microscopic traces of blood in his urine, a nodule in his lung, and failing kidneys.

He was dying but could take no more cyclophosphamide, the only drug known to put him into remission. Says Dr. Specks. “So then the question is what other agents are there?”

Dr. Specks made his rounds with residents and fellows in tow. After visiting the vasculitis patient, Dr. Specks told the team that cyclophosphamide suppresses the body’s B cells, which seem to play a key role in the possibly fatal autoimmune reaction. That’s when he heard from “one very bright resident at the time” — Marie C. Hogan, M.D., Ph.D., a nephrologist from Ireland.

Dr. Hogan (now on staff at Mayo Clinic) mentioned that a new drug, rituximab, was used to target B cells in patients with lymphoma. As a nephrologist, she was familiar with the use of similar drugs to suppress immune reactions in kidney transplant patients. In fact, at Mayo Clinic she had infused rituximab as an experimental drug in a patient with breast cancer. Back in Dublin, she had seen the drug administered to patients with kidney involvement and had noted its mild side effects compared with cyclophosphamide. She suspected that this drug would selectively target the B cells involved in this form of vasculitis. Might rituximab help the granulomatosis patient? she asked Dr. Specks.

The question sent Dr. Specks to research the drug. Rituximab, he discovered, had recently been licensed by the U.S. Food and Drug Administration to treat B-cell lymphoma. Rituximab attacked the B cells and effectively eliminated them. Unlike the older drug, cyclophosphamide, rituximab seemed to have little effect on the rest of the immune system. Nor did it seem to cause many serious side effects.

“It was an off-label use of a drug that was on the market,” says Dr. Specks. “I put one and one together and we said maybe this is a good alternative for this patient. Why don’t we try this on a compassionate use basis?” Compassionate use is a bit of a medical euphemism that means, this treatment is unproven but you have nothing to lose. Says Dr. Specks, “I discussed this with this patient in detail and he went for it.”

The patient tolerated the experimental treatment without significant side effects. After a second treatment, he went into remission.

Team research has huge impacts

The case of the gravely ill vasculitis patient offered an illuminating case study in collaboration. Dr. Hogan’s off-hand suggestion led Dr. Specks, Dr. Fervenza, and several other researchers (including Dr. Hogan) on a long series of studies and productive papers—and a new and safer treatment for several kinds of vasculitis.

Collaboration is a mysterious alchemy. Hard work and luck. Professional relationships and friendships. Deliberate study and random moments. Dialog between clinical and research. “How many hours do you have?” Dr. Specks asks, as he begins to explain what makes for effective collaboration in research. “That is probably the most difficult question that anybody could ask.”

Dr. Specks and Dr. Fervenza are both physicians and researchers. Dr. Specks is the principal investigator for the Vasculitis Clinical Research Consortium, sponsored by the National Institutes of Health. Dr. Fervenza is the director of the Nephrology Collaborative Group. Over the years, they developed a special relationship with one another, leading to research projects together.

Dr. Fervenza still speaks in a broad Brazilian accent. He is self effacing and chatty. He left Brazil for medical school in England, including time at Oxford. Back in Brazil, he found little use for his specialized training. He came to the United States on a fellowship at Stanford, but had to do a residency again to become eligible to take his boards. That is how he came to Mayo Clinic and met Dr. Specks, who had received his medical degree from the University of Bonn. Dr. Fervenza saw Dr. Specks as a mentor.

After his residency, Dr. Fervenza was hired to develop Mayo’s glomerulonephritis treatment. He and Dr. Specks continued to see some of the same patients, especially when the disease was some form of vasculitis.

Clinical practice often triggers research, says Dr. Specks. “The research questions have always come from patient observations and from problems that arose from not being able to help the patient the way we would have liked to,” he says. “How can we do better?”

Answering that question drove both men to collaborate with each other and a wide range of other investigators to learn more about using rituximab as a vasculitis treatment. Use in small groups proved encouraging. Next was a study of nearly 200 vasculitis patients in nine different medical centers, published in 2010 in the New England Journal of Medicine. This huge study confirmed that rituximab was every bit as effective as conventional treatment. A flurry of later papers further parsed the data obtained in the big multicenter study. Says Dr. Specks, “If you design one good experiment, it’s the gift keeps on giving.”

The studies demonstrated that rituximab effectively controlled vasculitis, oftentimes better than the old drug cyclophosphamide, and was well tolerated by patients. In 2011, the FDA approved rituximab for two forms of vasculitis.

Research continues into the use of rituximab to treat different forms of vasculitis, and into new drugs or new uses of existing drugs that are even more focused in their effects than rituximab is—“drugs that only do what is necessary to affect the mechanism that leads to the relapse,” says Dr. Specks. “That’s ultimately the goal.”

Drugs aren’t the only focus of vasculitis research. Perhaps the most compelling question is this, says Dr. Specks—“How can we identify the patient that will relapse down the road once the diagnosis has been established and once the patient has been put into remission?” Genetic tests or studies on immune cells may be able to offer a clearer prognosis than is currently possible.

Toward better solutions

The stereotype of the lone genius has been exposed as a myth—or at the least the rare exception that proves the rule. Dr. Specks’ and Dr. Fervenza’s research is an example of the truism that two heads are better than one. That’s perhaps the principal reason for scientific collaboration. Says Dr. Specks, “You go to meetings and ideas get tossed around and you discuss, what’s the next question, and you realize that you can better answer it together than in isolation. This is how one thing leads to another.”

Mayo Clinic in particular fosters this kind of collaboration, says Dr. Specks. Its reputation for excellence draws some of the brightest minds in medicine, as well as patients seeking answers when past treatment has failed. Moreover, “clinical care, research and education have always been intertwined and valued. It was understood that we need to excel in all three in order to move the field forward and provide better solutions for patients.”

Moreover, Mayo breaks down barriers between disciplines to improve patient care. That same work environment encourages effective collaboration in research. Of that there’s no better example than Dr. Fervenza and Dr. Specks.

Says Dr. Specks, “We see the same patient together and we discuss this when questions come up. We ask how can we answer these questions together? How can we do studies together? That’s how these things come about. It’s relatively easy at Mayo, probably easier than in many other institutions where the departmental boundaries are much stronger.”

It’s not surprising, perhaps, that the personable Dr. Fervenza would put things somewhat differently—that successful collaborations depend on friendships and social skills. The key? “Not to have a big head,” he says. “Because if you believe that you know everything, then people don’t like to collaborate with you. You need to give and take, and give credit where credit is due.”

- Greg Breining

November 2015

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This shortage of blood can result in organ and tissue damage, even death. Vasculitis can affect anyone, though some types of vasculitis are more common among certain groups. Some forms of vasculitis improve on their own, but others require treatment — often including taking medications for an extended period of time.

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