While recognizing systemic vasculitis is a challenging task, it is more difficult to diagnose and identify vasculitis affecting the central nervous system (CNS).
“CNS vasculitis is a rare condition that comes up quite a lot in the work up of patients who have unexplained neurologic deficits,” said Rula A. Hajj-Ali, MD, Professor of Medicine at Cleveland Clinic and Director of the Central Nervous System Vasculitis Program and Inflammatory Eye Disease Clinic.
“Vasculitis can affect the blood vessels of the brain, causing a range of symptoms that are not very specific,” she noted. “It can be challenging to diagnose based on symptoms only given the myriad presentation that can vary from encephalopathy to strokes, chronic headaches, cognitive or personality changes, vision problems, seizures, and other problems.”
Dr. Hajj-Ali will present the session CARE: Central Nervous System Vasculitis on Sunday, Nov. 12, from 2–3 p.m. PT in Exhibit Hall A-B in the San Diego Convention Center. She will discuss some of the more challenging diagnostic and clinical questions in CNS vasculitis with a focus on potential CNS vasculitis questions on rheumatology board exams.
The session will be livestreamed and available on-demand within 24 hours of the session for registered ACR Convergence 2023 participants.
The wide variety of potential symptoms makes it difficult to identify CNS vasculitis, although multiple diagnostic tests are available. The difficulty, Dr. Hajj-Ali said, is interpreting multiple diagnostic tests, since there is no one specific test for the diagnosis of CNS vasculitis. She will offer four case-based assessments to explore the pitfalls of diagnosis, multiple diagnostic modalities, and common mimics.
The presence of neurologic symptoms in the context of immune-mediated inflammatory diseases may suggest CNS vasculitis, she continued. CNS vasculitis can be secondary to systemic vasculitis, such as granulomatosis with polyangiitis, systemic vasculitides, such as Behçet’s disease, polyarteritis nodosa, systemic immune-mediated inflammatory diseases, such as rheumatoid arthritis, various connective tissue diseases, and sarcoidosis. CNS vasculitis can also be secondary to bacterial or viral infection, which is crucial to recognize.
“The diagnosis of CNS vasculitis is not always certain, so it is very important to make sure the diagnosis is as accurate as possible,” Dr. Hajj-Ali said. “CNS vasculitis can also be present just in the brain or the spinal cord with no other organ involvement, referred to as primary CNS vasculitis.”
Whatever the etiology, inflamed vessel walls can impede blood flow and oxygen supplies to the brain, resulting in a loss of brain function and potentially one or more strokes. Unexplained neurologic abnormalities and/or strokes that are not explained by traditional risk factors can be suggestive of CNS vasculitis.
So can abnormal results from cerebral angiogram or other cerebral vascular imaging, such as magnetic resonance angiogram (MRA) or computer tomography angiogram (CTA), although imaging alone may not be sufficient for a solid diagnosis.
“It is important to remember to at least consider the multiple conditions that mimic CNS vasculitis and to understand the different diagnostic modalities we have available,” Dr. Hajj-Ali said. “There may be multiple blood tests needed and a lumbar puncture for cerebrospinal fluid analysis may be appropriate. So might an MRA, CTA, or cerebral angiogram as well as brain biopsy.
“It is just as important to understand the limitations of each of these diagnostic modalities and some of the mimics that may contribute to the same blood vessel abnormalities in the brain as CNS vasculitis,” she continued. “This session will enhance your knowledge of how to approach diagnostic workup for CNS vasculitis and your understanding of the many mimics of this condition.”