Diagnosing and Treating Vasculitis: an Update

Vasculitis is among a group of rare autoimmune conditions and it’s also one of the most difficult to diagnose. Rheumatologists are simultaneously hampered by the non-specific nature of the disease and their own lack of experience in treating it.

Vasculitis is among a group of rare autoimmune conditions and it’s also one of the most difficult to diagnose. Rheumatologists are simultaneously hampered by the non-specific nature of the disease and their own lack of experience in treating it.

“Most rheumatologists don’t have familiarity with vasculitis. They don’t have a lot of experience with it simply because they don’t have a great number of patients with vasculitis,” said Sharon Chung, M.D., director of the University of California at San Francisco Vasculitis Clinic. “There’s even less familiarity among primary care physicians.”

Although vasculitis affects approximately 200,000 individuals annually, new research is burgeoning. And, being able to recognize the signs and symptoms to make a diagnosis is paramount to patient health.

Latest Research

According to Eric Matteson, M.D., chair of rheumatology at Mayo Clinic in Minnesota, the majority of investigations into vasculitis in recent years have centered around drug development. The biggest push has been to replace glucocorticoids as the main treatment because the side effects, including increased risk of infection, diabetes, and bone deterioration, are so severe.

"We want to use these new drugs across the board because our major problem is steroids and the side effects,” he said. “Anything we can do to reduce the amount of steroids will be a major advantage for the patient.”

Ultimately, he said, the goal is to find a balance between disease control and side-effect minimization.

One of the biggest advancements, he said, is the use to tocilizumab to treat giant cell arteritis, a form of vasculitis that affects individuals over age 50. Already approved for combatting rheumatoid arthritis, tocilizumab is up for approval from the Food & Drug Administration. By blocking the effects of pro-inflammatory protein interleukin-6, research shows tocilizumab reduces the need for steroid use among vasculitis patients by 50 percent, Dr. Matteson said.

Rituximab has been used to treat a number of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) forms for several years. However, recent research revealed a subset of granulocytes, present in patients with AAV, can be used to predict whether they will achieve remission on rituximab. Greater success rates lead to reduced use of chemotherapy and steroids, he said.

Additional research with abatacept - medication that arrests inflammation by blocking T-cells from talking to each other - showed it is more effective in treating giant cell arteritis than other vasculitis forms, such as Takayasu’s arteritis. Based on research published in Arthritis & Rheumatology abatacept lengthens remission from giant cell arteritis by almost three times over placebo (9.9 months vs. 3.9 months), but remission from Takayasu’s is slightly less than on a placebo (5.5 months vs. 5.7 months.) This knowledge can help rheumatologists target who can best benefit from abatacept.

An Unconfirmed Diagnosis

Regardless of which medication is needed, Dr. Chung said, primary care providers and rheumatologists must work together to diagnose vasculitis as quickly as possible. If left untreated, the condition can have irreversible effects or lead to death.

For individuals who have small-to-medium vessel vasculitis, a form that attacks the lungs, kidneys, and skin, not being diagnosed quickly enough or receiving treatment in a timely manner can be fatal. Roughly 80 percent of these patients will die within a year if they don’t receive therapy, she said.

Patients with giant cell arteritis, inflammation of the blood vessel lining, can experience diminished eyesight or permanent blindness, as well as stroke, if providers don’t recognize the problem early enough.

Signs and Symptoms

Much like other autoimmune diseases, the vasculitis signs and symptoms are relatively non-specific. While they can indicate a type of vasculitis is present, they can also point to a wide number of other problems, Dr. Matteson said.

Primary care physicians should pay close attention to a patient’s complaints, especially if they persist, and refer a patient to a rheumatologist for specialty treatment. General feelings of weakness or numbness in hands or feet and fatigue are common, as well as shortness of breath, weight loss, and a lingering cough. Patients may cough up blood. Patients can also develop skin rashes, fever, vision changes, swelling in the legs, and muscle pain.

“All of these symptoms can happen with other diseases, so we have to try hard to put the pieces together,” Dr. Matteson said. “It’s important to take a very good patient history to try to rule out infection, heart failure, or other diseases with these symptoms.”

To help diagnose vasculitis as quickly as possible, both rheumatologists and primary care physicians have a few tools at their disposal.

Blood test:  A blood test will identify low hemoglobin and hematocrit levels, high C-reactive protein levels in the liver, and a high erythrocyte sedimentation rate. It can also reveal the presence of ANCA antibodies that attack white blood cells.

Urinalysis:  High levels of protein or blood cells in urine can indicate the kidneys are affected by vasculitis.

Imaging:  CT scans, MRIs, and X-rays, including angiography, can non-invasively pinpoint nodules, thickening of blood vessel linings, and diminished blood flow. These are all signs of potential vasculitis.

Biopsy:  Biopsy is the only way to definitely determine whether a patient has vasculitis. A pathologist examines a small tissue sample – from the skin, blood vessels, or affected organs - to see if inflammation is present.

Best Practices

Because each vasculitis case is different, treatments must be, too, Dr. Chung said. “Best practices are very dependent on what types of vasculitis the patient has. There are different prongs to the approaches, but once we have the disease under control, and even in relapse, we can identify a plan before significant damage occurs,” she said

Overall, she said, using immunosuppressive medications alongside consistent, proper monitoring is the best approach. Doing so will lead a rheumatologist to recommend a targeted medication.

“Our goal is to help patients return to normal life and function,” Dr. Chung said.

References:

Stone, J.H., et al., “Efficacy and Safety of Tocilizumab in Patients with Giant Cell Arteritis: Primary & Secondary Outcomes from a Phase 3, Randomized, Double-Blind, Placebo-Controlled Trial,” Arthritis Rheum. 2016; DOI: 10.1002/art.39977.

Butte, AJ., et al., "Reanalysis of the Rituximab in ANCA-Associated Vasculitis trial identifies granulocyte subsets as a novel early marker of successful treatment,” Arthritis Rheum., 2015; DOI: 10.1186/s13075-015-0778-z.