Patient complaints of overall pain and general malaise can send providers in a myriad of directions, seeking the right diagnosis. In many cases, vasculitis isn’t at the top of their list because there are very few symptoms that point to it directly.
But, a missed diagnosis can have negative consequences for patients, making it critical for providers to have a better understanding of vasculitis and its related syndromes. In a presentation given at the Rheumatology Nurses Society annual meeting earlier this month, Jeffrey Kaine, M.D., an independent rheumatology consultant based in Cullowhee, N.C., discussed the challenges in diagnosing and treating vasculitis.
In this Q&A, Dr. Kaine covers some of the high points from his talk.
Why is it so important for a rheumatologist or rheumatology nurse to understand the underlying nature of vasculitis?
“Understanding the pathophysiology of vasculitis is critical because it facilitates a better understanding of the clinical manifestations of disease and a better understanding of the classification of various vasculitis symptoms.
The most important thing the provider in the room with the patient needs to know is what kinds of symptoms or what kinds of physical findings or constellation of findings might be significant and what should make them think of a diagnosis of vasculitis. Based on what they hear from the patient, the provider can think about classification and any additional work up they might need to pursue with the patient. Overall, they must initially recognize that their patient is sick with a multi-system disease and decide how to proceed accordingly.”
What makes diagnosis so difficult?
“Vasculitis presentation is extremely diverse. Most patients will have multiple complaints that, on the surface, don’t seem to relate. They almost always are systemically ill with symptoms, such as fatigue, fever, aches and pains, and a global sense of just being sick the majority of the time. When those aches and pains are coupled with arthralgia or frank arthritis, the likelihood of there being inflammatory rheumatic disease dramatically increases. And, when you think inflammatory rheumatic disease, you’re automatically including vasculitic syndromes in your differential diagnosis.
The decisions regarding treatment are based on whether you can pigeon hole the patient into a distinct vasculitic syndrome. Certain syndromes are treated very specifically, and others are less clear cut. Remember that you’re talking about at least 20 different diseases here, and treatment decisions are based on the urgency of the clinical situations, as well as the specific disease. For instance, if you’re talking about a patient with temporal arteritis, the individual is at a very high risk of losing their vision if they have premonitory symptoms. They would be treated earlier and one might not even wait for a definite diagnosis because the consequences of being wrong and missing the diagnosis are so substantial. But, if someone has a skin rash, and it’s chronic, relatively indolent, and it’s been there for six months, there’s not the same type of urgency in that situation. So, every encounter is based on the clinical situation, as well as the suspected diagnosis.
Unfortunately, making the diagnosis isn’t always possible. There’s always the possibility that you’ll have to treat the patient empirically. Many vasculitic syndromes have overlapping features and can’t be precisely categorized into one specific diagnosis. Additionally, vasculitis can be associated with other rheumatic diseases. For instance, a patient could have vasculitis associated with lupus, and, as a provider, you need to take both aspects of disease into account when making any treatment decisions.”
With disease presentation and symptoms being so varied, how should providers approach treatment?
“Providers need to remember that vasculitic syndromes can present with a wide variety of symptoms. No. 1, I think it’s important to recognize the systemic nature of the disease. Always be on the look out for renal disease, arthralgia, or frank arthritis.
Secondly, you should always think about the mimics of vasculitis. These vasculitis mimics are diagnosed differently, and they’re treated differently. If a patient is misdiagnosed, it can theoretically have disastrous consequences. HIV disease, hepatitis, endocarditis, syphilis — these are all infections that can be vasculitis mimics. There can also be an overlap with underlying disease, such as rheumatoid arthritis or lupus, that will also need to be treated differently. So, vasculitis mimics will need to be excluded before pursuing treatment.
Third, providers should also be aware that there are two specific diseases where there have been significant advancements in treatment. The first is giant cell arteritis or temporal arteritis. Data published a couple of years ago has really changed how we think about treating this disease. An international group or investigators studied the use of tocilizumab, also knows as ACTEMRA®, in the treatment of this disease. It was clear that patients who receive tocilizumab were able to be weaned off of their corticosteroid therapy more aggressively, and that, ultimately, reduces some of the co-morbidities of therapy. The second disease is ANCA-associated vasculitic syndrome. It’s actually a group of diseases, but the use of rituximab has really revolutionized the treatment paradigm, and patients are now achieving both more durable remission, as well as sustained remission in the long term. It’s important to recognize that vasculitic syndromes are diseases that are all capable of remission, but also exacerbation. So, providers need to be diligent about looking at disease activity over the long haul regardless of what the vasculitic disease is.”
What is your take-away message?
“Providers need to recognize, or at least be suspicious of, a diagnosis of vasculitis based on the constellation of symptoms the patient presents. Each patient should be worked up individually based on his or her symptoms and basic lab studies. Be aware of vasculitis since there are so many different syndromes, as a whole, they’re not all that rare. Have a wide, diverse investigation of different kinds of symptoms. Don’t just concentrate on one thing, look for evidence of systemic disease. Don’t forget about vasculitic mimics because they’re critically important to exclude. And, remember that evaluations for evidence of vasculitis will oftentimes require a diverse variety of tests and multiple biopsies to confirm a diagnosis.”