CLEVELAND — Leonard H. Calabrese, DO, of the Cleveland Clinic and Chief Medical Editor of Healio Rheumatology, provided a diagnostic update for patients with central nervous system vasculitis at the Primary Vasculitides Pre-symposium of the Biologic Therapies VII Summit.
Leonard H. Calabrese
Calabrese defined seven steps in diagnosis. He noted the first method — before vascular wall imaging — is to identify comorbidities with a high likelihood of being associated with CNS vasculitis. These include chronic meningitis, recurrent focal deficits and unexplained focal and diffuse neurological dysfunction. For isolated CNS vasculitis, features include headaches, encephalopathy, change in behavior, focal motor or sensory abnormalities, ataxia, cranial neuropathies, visual changes, myelopathy and radiculopathy. The second method is to perform a lumbar puncture, which he said should only be omitted if there is risk from CNS mass effect. Calabrese said the third method is non-vascular imaging, which he noted is highly sensitive (97%) but has low specificity, so it cannot be used to confirm diagnosis completely. Non-vascular imaging includes CT scan, MRI, single-photon emission CT and positron emission tomography. Non-specific findings in non-vascular imaging include multiple ischemic lesions, hemorrhages, leukoencephalopathy and gad enhancing lesions. The fourth method he noted is direct and indirect vascular imaging. The fifth method is brain biopsy, which he said can be used to confirm vascular imaging due to its high specificity. The sixth method is to rule out infections, which includes varicella, hepatitis C, HIV, cytomegalovirus, lymphocytic choriomeningitis, mycobacteria, fungi, rickets, spirochete and parasites; and to rule out cancer, which includes intravascular lymphoma, CNS lymphoma and leptomeningeal metastasis of a solid tumor. The seventh method is lack of progress on cyclophosphamide and glucocorticoid treatment.
In an overarching system, Calabrese designed three scenarios physicians should follow. The first is a positive biopsy followed by a confirmation and treatment; the second is a positive “angiogram” followed by a confirmation and treatment; and the third is suspicious clinical setting or neuroimaging, and input is needed from other specialties about the diagnostic approach and likelihood for disease.
“For best practice, it is a team sport.” Calabrese said. “This is not a single-person sport, and it requires people to know a little bit about all of these things.” – by Will Offit
Calabrese L. Clinical decision making: Is it CNS vasculitis or something else? Presented at: Primary Vasculitides Pre-symposium of the Biologic Therapies VII Summit; April 4-8, 2017; Cleveland.
Disclosure: Calabrese reports no relevant financial disclosures.