By Marti Webb Slay

When Angelo Gaffo, MD from the UAB Division of Clinical Immunology and Rheumatology works with residents, he always goes over the signs of vasculitis with them. 

“They will probably only see a few cases a year of this,” he said. “My message to them is, ‘you’ll see 100 times more cases of diabetes than this, but when a patient comes in with vasculitis, you don’t want to miss it.’

“Vasculitis diseases are characterized by inflammation of the blood vessels of different sizes and locations in the body, which leads to damage in a number of internal organs, so early identification and treatment is important.

“I’ve seen many patients who were not referred early enough. Usually there has already been, for example, kidney damage or peripheral nerve damage or lung damage. We can treat them, and we can get them better, but then we have to deal with the consequences of delayed diagnosis.”

He believes that because primary care doctors see vasculitis so rarely, it is not the first diagnosis to come to mind, and it can be missed while other possibilities are checked. Likewise, a shortage of rheumatologists and long waits to get appointments can result in delayed diagnosis and treatment. “Sometimes we find these patients in the hospital, because they are already so sick,” he said.

Gaffo said the key is to recognize the patterns and understand how the different forms of vasculitis can present. The most common form in the US Southeast is ANCA associated vasculitis. These diseases include granulomatosis with polyangiitis, microscopic polyangiitis, and the more rare eosinophilic granulomatosis with polyangiitis.

“The patterns can be a specific form of kidney disease, glomerulonephritis, diagnosed with a kidney biopsy,” Gaffo said. “This may occur with swelling, with hypertension and sometimes concurrently with a purpuric rash. Sometimes there is recurring sinusitis with crusting and bleeding, or recurring ear infections. Otitis media is common in children, but should not be common in adults. So we should ask if this something else. We should then explore if this person has a systemic illness.”

Nerve damage is another symptom that can be a red flag. “Nerve damage is very common in diabetes, so in that context, you may miss somebody who is not diabetic and has unexplained nerve damage,” he said.

Another form of vasculitis is giant cell arteritis, which is the most common form of vasculitis in general, but less common among African Americans and Latinos. It involves large blood vessels around the head and neck. “This can cause chronic head discomfort, headaches and painful and tired jaw, known as jaw claudication,” Gaffo said. “These patients can have incidents of blurry vision or double vision. These vision changes can herald the complication of vision loss that can happen in patients with giant cell arteritis. This is almost universally in patients over 50, and most commonly over the age of 60. A pattern of headache, fatigue and quite often shoulder and neck pain with jaw claudication and episodes of vision loss can indicate giant cell arteritis.

“When vasculitis is suspected, you should start with basic labs including inflammation markers. For ANCA associated vasculitis, you can run ANCA antibodies. That can increase your suspicions if it comes back positive, but use clinical judgment. A biopsy of an affected organ is always preferable. If you really suspect vasculitis, you should refer to a rheumatologist, even while labs are being collected. It is even more important with suspected giant cell arteritis. The rheumatologist should get involved very soon.

“When talking about vasculitis, we are considering more than six or seven different diseases. For some, the referral should be prompt and early.”

There is no cure for vasculitis. Physicians control the underlying autoimmune process with the available treatments. The first line of treatment is often a steroid to rapidly shut down inflammation and damage, concurrent with another medicine that is tailored to address the process that is driving that condition, which allows physicians to cut the steroids in the future.

Patients are generally followed by a rheumatologist for several years. In some cases, they may go into remission, but that is less common.

Gaffo is aware that referrals to rheumatologists can sometimes result in a significant wait time for a first appointment, but he stresses the need for prompt attention when vasculitis is suspected. “Don’t be shy about prompt referral,” he said. “When it is flagged as possible vasculitis, we try to rush these cases. It’s very important.”