Neuroophthalmologic Aspects of the Vasculitides
There have been significant advances in the understanding of the vasculitides in the past several years, leading to more precise classification and nosology. Ophthalmologic manifestations may be the presenting feature of and a clue to the diagnosis of vasculitis, or develop in the course of the illness owing to a common disease mechanism. Precise diagnosis and prompt treatment prevents short- and long-term ophthalmologic sequela.
Epidemiology of the Vasculitides
The epidemiology of vasculitis has witnessed extraordinary advances in the past decade influenced by the worldwide increased recognition and accurate classification and diagnosis of the vasculitides, and insights brought by genome-wide association studies and online genetic biological repositories that permit researchers to freely access a wide array of genetic and clinical resources that contribute to the understanding of the heritable factors of the systemic vasculitides. This article reviews the current knowledge of the epidemiology of vasculitides in different global regions.
Treatment of Vasculitis of the Nervous System
The diagnosis of primary central and peripheral nerve vasculitides should be established with certainty if suspected before commencing potent immunosuppressive therapy. The aim of induction therapy is to rapidly control the underlying inflammatory response and stabilize the blood-brain and blood-nerve barriers, followed by maintenance immunosuppression tailored to the likeliest humoral and cell-mediated autoimmune inflammatory vasculitic processes.
Central Nervous System Vasculitis due to Infection
Several pathogens have the propensity to involve blood vessels during central nervous system infection, which can lead to cerebrovascular complications. Infection is a recognized cause of secondary central nervous system vasculitis. It is important not to miss the diagnosis of infection-related central nervous system vasculitis because specific antimicrobial therapy may be necessary; this article reviews the major implicated organisms.
Eleven Themes in the History of Systemic and Nervous System Vasculitides
Vasculitis is defined as inflammation of blood vessel walls for at least some time during the course of the disease, and affects arteries and veins of varying caliber. Two Chapel Hill Consensus Conferences, in 1994 and 2012, provide consensus on nosology and definitions for the commonest forms of vasculitis. The category of single-organ vasculitis, suggesting the limited expression of a systemic vasculitis, includes primary central nervous system vasculitis and nonsystemic peripheral nervous system vasculitis. The historical aspects of systemic and limited forms of vasculitis are reviewed in 11 relevant themes.
Overview of the Vasculitides
The systemic vasculitides are heterogeneous clinicopathologic disorders that share the common feature of vascular inflammation. The resulting disorder can vary depending on involvement of specific organs, caliber of blood vessels, the underlying inflammatory process, and individual host factors. The cumulative result is diminished blood flow, vascular alterations, and eventual occlusion with variable ischemia, necrosis, and tissue damage. An international revised nomenclature system provides the necessary nosology and findings relevant to classify each of the vasculitides. This article is an introduction and overview of the clinical presentation, differential diagnosis, laboratory evaluation, and treatment of systemic and nervous system vasculitides.
Granulomatous Angiitis: Twenty Years Later
Granulomatous inflammation, the prototypical histopathology of adult and childhood vasculitis, is characterized by inflammation of blood vessels accompanied by giant cells and epithelioid cells in the walls of cerebral vessels ranging from small leptomeningeal veins to large named cerebral arteries. Headache, hemiparesis, mental changes, abnormal cerebrospinal fluid protein content, and pleocytosis are suggestive features that warrant brain and leptomeningeal biopsy to make the diagnosis certain and begin cytotoxic therapy to improve outcome.
Stroke due to Vasculitis in Children and Adults
The vasculitides are diseases characterized by inflammation of blood vessels and inflammatory leukocytes in vessel walls. There is an increased propensity for ischemic stroke, resulting from compromise of vessel lumina with distal tissue ischemia; and hemorrhagic or nonhemorrhagic stroke, and aneurysmal formation and bleeding, due to loss of vessel integrity.
Giant Cell Arteritis
"Giant cell arteritis (GCA) is a chronic, idiopathic, granulomatous vasculitis of medium and large arteries comprising overlapping phenotypes of cranial arteritis and extracranial GCA. Vascular complications are generally due to delay in diagnosis and initiation of effective treatment. Advancements in MRI and MR angiography, computed tomography angiography, 18fluoro-deoxyglucose/PET, and color duplex ultrasonography have led to improved diagnosis. Corticosteroids are the mainstay of therapy in GCA; however, their use is associated with predictable and occasionally serious side effects. Biological agents are effective and safe corticosteroid-sparing agents in treating GCA. This article reviews the epidemiologic, clinicopathologic features, diagnosis, and treatment of GCA."
Autoimmune encephalitis is a severe inflammatory disorder of the brain with diverse causes and a complex differential diagnosis. Recent advances in the past decade have led to the identification of new syndromes and biological markers of limbic encephalitis, the commonest presentation of autoimmune encephalitis. The successful use of serum and intrathecal antibodies to diagnose affected patients has resulted in few biopsy and postmortem examinations. In those available, there can be variable infiltrating inflammatory TÂ cells with cytotoxic granules in close apposition to neurons, consistent with an inflammatory autoimmune basis, but true vasculitis is rarely seen. The exception is Hashimoto encephalopathy.