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August 09, 2019
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High suspicion ‘critical’ to avoid misdiagnosis of vasculitis mimics, treatment errors

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Jeffrey L. Kaine

ORLANDO — Rheumatology providers should “maintain a high index of suspicion” when diagnosing patients with suspected vasculitis as several under-recognized syndromes mimic this condition, and could worsen after treatment with glucocorticoids, according to a presenter at the Rheumatology Nurses Society Annual Conference.

“This may well be the most important information I share with you today: Watch out for mimics of vasculitis,” Jeffrey L. Kaine, MD, president of Independent Healthcare Associates Inc., told attendees. “It is critical because if you misdiagnose vasculitis and you treat the patient with high-dose steroids initially, you may be doing the patient a disservice.”

In particular, Kaine noted, endocarditis is a classic example of a vasculitis mimic, presenting in a similar fashion with multisystem involvement and posing a significant diagnostic challenge.

“I recall seeing a patient that was referred to a rheumatologist for suspected new-onset rheumatoid arthritis,” Kaine said. “The treating rheumatologist did not order blood cultures and did not consider the fact that the patient had a fever. Guess what? The patient actually had endocarditis and subsequently had a stroke and was permanently disabled. The patient obviously didn’t have rheumatoid arthritis but instead had a false-positive rheumatoid factor.”

The most common cause of vasculitis among adults would be drug-induced vasculitis, which can be difficult to differentiate from idiopathic vasculitis syndromes. “Don’t forget to consider drug use in adults as both cocaine and amphetamine users can display vasculitis-like syndromes,” Kaine said.

Yet another convincing vasculitis mimic is cholesterol emboli, which may have laboratory findings typical of vasculitis including elevated acute phase reactants, thrombocytopenia, eosinophilia and transient hypocomplementemia.

“In cholesterol emboli, it is often times very critical to have an ophthalmologist look at the retina,” Kaine said. “If the patient has cholesterol emboli presenting as diffuse distal infarcts of skin or extremities, and the ophthalmologist looks at the retina and sees evidence of cholesterol emboli, you may make a diagnosis that you otherwise had no way of making. So never forget to consider retinal disease.”

Lastly, Kaine noted that there are also very rare conditions that can mimic vasculitis syndromes, most notably the central nervous system pseudovasculitis known as reversible cerebral vasoconstriction syndrome. “These are often called ‘thunderclap headaches,’ in which patients have reversible cerebral ischemia mimicking isolated angiitis of the central nervous system.”– by Robert Stott

Reference:
Kaine JL. Navigating the pathophysiology and treatment of vasculitis. Presented at: Rheumatology Nurses Society Annual Conference; Aug. 7-10, 2019; Orlando.

Disclosure:Kaine reports speaking and consultant roles with Bristol-Myers Squibb, Eli Lilly, Gilead Sciences, Merck, Pfizer and Regeneron.