RESUMEN
OBJECTIVES: To determine the diagnostic accuracy for high-resolution vessel wall image (HR-VWI) and brain biopsy according to angiographical classification in patients with primary central nervous system vasculitis (PCNSV). METHODS: We extracted the patients with PCNSV who underwent the complete brain MRI protocol and cerebral vascular image from Cleveland Clinic prospective CNS vasculopathy Bioregistry. The large-medium vessel variant (LMVV) was defined as patients with cerebral vasculature indicating vasculitis in proximal or middle arterial segments, whereas vessel involvements in smaller distal branches or normal angiography were considered as the small vessel variant (SVV). We compared clinical demographics, magnetic resonance imaging (MRI) findings, and diagnostic approaches between two variants. RESULTS: In this case-control study that included 34 PCNSV patients, the LMVV group comprised a total of 11 patients (32.4%), and 23 patients (67.6%) were classified as the SVV group. The LMVV had more strong/concentric vessel wall enhancement on HR-VWI (LMVV: 90% (9/10) vs. SVV: 7.1% (1/14), p<0.001). By contrast, meningeal/parenchymal contrast enhancement lesion was more frequently observed in the SVV group (p=0.006). The majority of SVV was diagnosed by brain biopsy (SVV: 78.3% vs. LMVV: 30.8%, p=0.022). The diagnostic accuracy of the brain biopsy was 100% (18/18) in SVV and 57.1% (4/7) in LMVV, respectively (p=0.015). CONCLUSIONS: Diagnostic approach for PCNSV differs concerning the affected vessel size. HR-VWI is a useful imaging modality for the diagnosis of LMVV. Brain biopsy remains the gold standard for proving PCNSV with SVV but is still positive in almost one-third of LMVV.
Asunto(s)
Imagen por Resonancia Magnética , Vasculitis del Sistema Nervioso Central , Humanos , Estudios de Casos y Controles , Angiografía Cerebral , Estudios Prospectivos , Estudios Retrospectivos , Encéfalo/diagnóstico por imagen , Vasculitis del Sistema Nervioso Central/diagnóstico por imagen , Angiografía por Resonancia Magnética/métodosRESUMEN
OBJECTIVES: Cerebral amyloid angiopathy (CAA) related inflammation (CAA-RI) is an autoimmune inflammatory condition occurring in patients with CAA. We aimed to determine the prevalence of radiological CAA-RI amongst patients with CAA and to describe their presenting clinical features. METHODS: We performed a retrospective review of electronic medical records across multiple centers within a single healthcare network. Patients who met radiological modified Boston 2.0 criteria for CAA and had white matter hyperintensity (WMH) were included. Scans were analyzed by a vascular neurologist and confirmed by a neuroradiologist blinded to clinical information for meeting criteria for possible or probable radiographic CAA-RI. RESULTS: Out of 1100 patients reviewed, 511 patients met radiological modified Boston criteria for CAA and 193 patients had WMH on MRI. A total of 55 (28.5 % of those with CAA and WMH, and 10.8 % of all CAA with or without WMH) patients had MRI brain imaging suggestive of possible or probable radiographic CAA-RI. The diagnosis of CAA-RI was reported in only 10 (18.2 %) patients initially while 20 (36.4 %) were diagnosed up to 74 months later (median 0, IQR 0-9 months). At the time of earliest probable CAA-RI findings on imaging, the most common concurrent findings were cognitive impairment (74.5 %), macro-hemorrhages (52.7 %), headache (30.9 %), seizures (14.5 %), and ischemic infarcts (14.5 %). Only 18 (32.7 %) patients were treated with immunosuppression. CONCLUSIONS: The prevalence of radiographic CAA-RI was high, and most cases were unrecognized and untreated. Further studies are needed to assess if earlier detection and treatment of radiologic CAA-RI may halt disease progression and prevent cognitive decline in these patients.
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Angiopatía Amiloide Cerebral , Hemorragia Cerebral , Humanos , Prevalencia , Angiopatía Amiloide Cerebral/complicaciones , Angiopatía Amiloide Cerebral/diagnóstico por imagen , Angiopatía Amiloide Cerebral/epidemiología , Imagen por Resonancia Magnética/métodos , Inflamación/diagnóstico por imagen , Inflamación/epidemiologíaRESUMEN
OBJECTIVES: High-resolution vessel wall imaging (HR-VWI) often demonstrates strong and concentric vessel wall enhancement (VWE) in patients with central nervous system vasculitis (CNS-V). However, little is known about follow-up VWE characteristics and monitoring the response to treatments. The aim of this study was to investigate serial VWE patterns and its clinical practice through the management of CNS-V. METHODS: We extracted 9 patients with diagnosed of CNS-V who underwent serial HR-VWI (baseline, 1st follow-up, and 2nd follow-up) from Cleveland Clinic CNS vasculopathy registry. VWE were analysed in 17 intracranial artery segments. VWE was graded on a 3-point scale (0; none, 1; mild/eccentric, and 2; strong/concentric). VWE grade for each arterial segment was summed to create a total VWE score. We investigated the relationship between serial VWE patterns and clinical course. RESULTS: In unique 153 intracranial arterial segments, 39 arteries (25.5%) had strong/concentric VWE on baseline HR-VWI. The positive rates of concentric VWE have decreased to 12.4% (19/153) at 1st follow-up and (10/153) 6.5% at 2nd follow-up, respectively (p<0.001). Mean total VWE scores have significantly decreased over time courses (p=0.034). Two patients had relapse at 1st follow-up image. In relapse cases, mean total VWE scores have worsened at 1st follow-up (baseline:2.0 to 1st follow-up: 6.0). After intensive immunosuppressive treatment, mean VWE scores have improved at 2nd follow-up (1st follow-up: 6.0 to 2nd follow-up: 2.0). CONCLUSIONS: Decreasing contrast VWE at follow-up images may indicate good response to treatment in CNS-V. By contrast, relapse patients might have temporal VWE worsening during the clinical course.
Asunto(s)
Angiografía por Resonancia Magnética , Vasculitis del Sistema Nervioso Central , Arterias , Humanos , Angiografía por Resonancia Magnética/métodos , Imagen por Resonancia Magnética , Recurrencia , Vasculitis del Sistema Nervioso Central/diagnóstico por imagen , Vasculitis del Sistema Nervioso Central/tratamiento farmacológicoRESUMEN
OBJECTIVES: To provide insight into the safety of recombinant zoster vaccine (RZV) in patients with immune-mediated inflammatory diseases (IMID). METHODS: Patients who received RZV in a single-centre rheumatology department were retrospectively included. An IMID flare was defined as (i) a documentation of flare in the office notes or patient portal communication or (ii) new prednisone prescription, in the 12 weeks after each dose. RESULTS: Six-hundred and twenty-two patients were included (67% female, median age 67 years), 8.5% of them experienced adverse events (AEs) and herpes zoster (HZ) incidence was 0.6% after median follow-up of 36 weeks. Of 359 IMID patients: 88 had RA (25%), 50 vasculitis (14%) and 29 PMR (8%). At vaccination, 35% were on glucocorticoids (GC). Fifty-nine patients (16%) experienced a flare, 18 flares occurred in temporal relation to a treatment change (31%). RA patients had the highest flare rate (n = 21, 24%), 25% of patients who flared required adjustment of immunosuppression. In a multivariate analysis, use of GC at time of vaccination was associated with flare after vaccination [odds ratio (OR) 2.31 (1.3-4.1), P =0.004]. A time-to-flare survival analysis (Cox-model) showed that GC was a significant predictor of IMID flare after first RZV dose [hazard ratio (HR) 2.4 (1.3-4.5), P =0.0039] and that a flare after the first dose was associated with flaring after the second RZV dose [HR 3.9 (1.7-9), P =0.0015]. CONCLUSION: RZV administration in patients with IMIDs was generally well-tolerated, though mild flares were not uncommon in the first 12 weeks after vaccination. These data may provide useful information for patient education when considering RZV administration.
Asunto(s)
Vacuna contra el Herpes Zóster/efectos adversos , Enfermedades Reumáticas , Brote de los Síntomas , Anciano , Femenino , Humanos , Masculino , Estudios RetrospectivosRESUMEN
PURPOSE OF REVIEW: The main purpose of this review is to present advances in diagnostics of central nervous system vasculitis (CNS-V). RECENT FINDINGS: Progress in molecular technologies and neuroimaging have added formidably to our knowledge of CNS-V. Next-generation sequencing has the promise to enhance our ability to diagnose, interrogate, and track infectious diseases, making this test attractive and capable of avoiding brain biopsy in cases where CNS infections are suspected. Further the continuum of neuroimaging progress has advanced our ability to diagnose CNS-V. Our capability to visualize the vessel wall have added a great value in differentiating inflammatory from noninflammatory vasculopathies. New genetic variations are being exposed with exome and genome sequences which will aid future diagnosis. SUMMARY: We have witnessed tremendous advances in CNS-V mainly by our ability to rule out mimics. Progress in molecular technologies, neuroimaging and genetic studies will continue to enhance the field further.
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Encéfalo/diagnóstico por imagen , Enfermedades Vasculares/diagnóstico , Vasculitis del Sistema Nervioso Central/diagnóstico , Biopsia , Diagnóstico Diferencial , Exoma , Humanos , Neuroimagen , Vasculitis del Sistema Nervioso Central/diagnóstico por imagen , Vasculitis del Sistema Nervioso Central/genéticaRESUMEN
OBJECTIVES: Neurosarcoidosis (NS) and primary angiitis of central nervous system (PACNS) are inflammatory diseases affecting central nervous system, with overlapping clinical and pathological characteristics. Distinguishing these diseases is important given distinct therapeutic implications. In this study, we aimed to compare demographic, CSF and MRI characteristics between these two conditions. METHODS: All the clinical, CSF and laboratory characteristics at the time of presentation were retrieved from electronic medical records. Brain and/or spinal cord MRI performed near the time of presentation were blindly evaluated by two neuroradiologists. Data regarding involvement of pachy- and leptomeninges, basal meninges, cranial nerves, cerebral grey and white matter, and spinal cord were recorded for each patient. RESULTS: 78 patients with PACNS and 25 patients with NS were included in the study. Mean age of patients was 43.7 (±16.7) and 43.6 (±12.5) in PACNS and NS, respectively. African-American race was found to be associated with the diagnosis of NS rather than PACNS. Patients with PACNS had higher frequency of cerebral involvement, while patients with NS demonstrated more frequent spinal cord, basal meningeal and cranial nerve involvements. CONCLUSIONS: These findings suggest that MRI can be an efficient tool in distinguishing PACNS from NS. A follow-up study with a larger sample size would be required to validate our results.
Asunto(s)
Enfermedades del Sistema Nervioso Central/diagnóstico por imagen , Sarcoidosis/diagnóstico por imagen , Vasculitis del Sistema Nervioso Central/diagnóstico por imagen , Adulto , Enfermedades del Sistema Nervioso Central/líquido cefalorraquídeo , Demografía , Diagnóstico Diferencial , Estudios de Seguimiento , Humanos , Imagen por Resonancia Magnética , Persona de Mediana Edad , Sarcoidosis/líquido cefalorraquídeo , Vasculitis del Sistema Nervioso Central/líquido cefalorraquídeoRESUMEN
PURPOSE OF REVIEW: The aim of this review is to provide the information necessary for recognizing scleritis in rheumatology patients, recognize associated systemic diseases in scleritis patients referred to rheumatology, and choose the best conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) or biologic therapy, based on the most recent data. RECENT FINDINGS: Scleritis is most often associated with systemic conditions, particularly autoimmune conditions. Rheumatologists should be most vigilant about rheumatoid arthritis and ANCA-associated vasculitis. A recent systemic association has been found with IgG4-related disease as well. Most of the therapeutic decisions are based on observational data. Most recently, there is increasing evidence of the role of rituximab for refractory disease. Scleritis is a severe inflammatory ocular condition that leads to complications including vision loss. It can precede a systemic autoimmune disease diagnosis, especially in the case of vasculitis. Prompt recognition and adequate therapy are needed in order to avoid serious ocular complications. There is an unmet need for (1) head-to-heat trials on csDMARDs and biologic drugs for the treatment of idiopathic scleritis and (2) randomized controlled trials on the treatment of refractory cases.
Asunto(s)
Enfermedades Autoinmunes , Escleritis , Antirreumáticos/uso terapéutico , Enfermedades Autoinmunes/diagnóstico , Enfermedades Autoinmunes/tratamiento farmacológico , Enfermedades Autoinmunes/etiología , Humanos , Rituximab/uso terapéutico , Escleritis/diagnóstico , Escleritis/tratamiento farmacológico , Escleritis/etiologíaRESUMEN
OBJECTIVES: Primary angiitis of the central nervous system (PACNS) is a vasculitis confined to the brain and spinal cord, which often presents with severe cognitive and functional deficits. Despite progress in diagnosis, little is still known about long-term outcomes. Our aim was to evaluate long-term functional capabilities, quality of life, and depression, and to determine the effect of treatment duration on patient outcomes. METHODS: We identified patients by ICD-9 codes for cerebral angiitis, and included them if they met two of the three following criteria: inflammatory cerebrospinal fluid (CSF), cerebral angiogram typical of vasculitis, or findings of vasculitis on pathologic examination of brain tissue. Disability was assessed by the Barthel Index, quality of life was assessed by EuroQol, and depression was assessed with Patient Health Questionnaire. RESULTS: Seventy-eight patients met the inclusion criteria, of which 27 responded to the questionnaire (34.6%). Mean follow-up of those who responded was 5.5 years (± 4.7). Nineteen of 27 patients (70.4%) had mild disability; meanwhile, 5 (18.5%) had severe disability. Fourteen of 27 patients (51.9%) had no mobility problem, 18 (66.7%) had no problems with self-care, 15 (55.6%) had no problems with usual activities, 14 (51.9%) had no pain, and 8 (29.6%) had no anxiety. Approximately 70% of patients had minimal or no depression. CONCLUSIONS: This is the longest reported follow-up of patients with PACNS described in the literature to date. Most patients had mild long-term disability and minimal to no depression, which may be reflective of treatment advances.
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Trastornos del Conocimiento/diagnóstico , Trastorno Depresivo/diagnóstico , Vasculitis del Sistema Nervioso Central , Angiografía Cerebral , Trastornos del Conocimiento/etiología , Trastorno Depresivo/etiología , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Examen Neurológico , Calidad de Vida , Vasculitis del Sistema Nervioso Central/líquido cefalorraquídeo , Vasculitis del Sistema Nervioso Central/complicaciones , Vasculitis del Sistema Nervioso Central/diagnósticoRESUMEN
PURPOSE OF REVIEW: The goal of this review is to provide an up-to-date approach to diagnosis and management of patients with central nervous system (CNS) vasculitis. RECENT FINDINGS: Challenges in diagnosis of CNS vasculitis still exist due to the broad differential diagnosis and generally nonspecific initial clinical manifestations. Differentiation between primary angiitis of the CNS (PACNS) and secondary causes is important in guiding management. Recent longitudinal cohort studies have improved our understanding of PACNS. Advances in neuroimaging and molecular testing have enhanced diagnostic decision-making. Therapy remains largely empiric, guided by observational data. Despite the limited use of targeted therapies, glucocorticoids and cyclophosphamide remain the mainstays of therapy in PACNS. Securing a diagnosis through a careful, team-based approach with emphasis on ruling out possible mimics is paramount in the management of patients with CNS vasculitis.
Asunto(s)
Vasculitis del Sistema Nervioso Central/diagnóstico , Ciclofosfamida/uso terapéutico , Diagnóstico Diferencial , Glucocorticoides/uso terapéutico , Humanos , Inmunosupresores/uso terapéutico , Vasculitis del Sistema Nervioso Central/tratamiento farmacológicoRESUMEN
OBJECTIVE: Literature on the effect of cell-derived extracellular vesicles (EV), ≤1 µm vesicles shed from various cell types during activation or apoptosis, on microvascular endothelial cell (MVEC) signaling is conflicting. Thrombospondin-1 and related proteins induce anti-angiogenic signals in MVEC via CD36. CD36 binds EV via phosphatidylserine exposed on their surface but the effects of this interaction on MVEC functions are not known. We hypothesized that EV would inhibit angiogenic MVEC functions via CD36. APPROACH AND RESULTS: EV generated in vitro from various cell types or isolated from plasma inhibited MVEC tube formation in in vitro matrigel assays and endothelial cell migration in Boyden chamber assays. Exosomes derived from the same cells did not have inhibitory activity. Inhibition of migration required endothelial cell expression of CD36. In mouse in vivo matrigel plug assays, EV inhibited cell migration into matrigel plugs in wild type but not in cd36 null animals. Annexin V, an anionic phospholipid binding protein, when incubated with EV partially reversed inhibition of migration, suggesting a phosphatidylserine-dependent effect. EV exposure induced reactive oxygen species generation in MVEC in a NADPH oxidase and Src family kinase-dependent manner, and their inhibition by apocynin and PP2, respectively, partially reversed the EV-mediated inhibition of migration. Annexin V partially reversed EV-induced reactive oxygen species generation in murine CD36 cDNA-transfected HVUEC but not in CD36-negative human umbilical vein endothelial cell. CONCLUSIONS: These studies establish a general inhibitory effect of EV on endothelial cell proangiogenic responses and identify a CD36-mediated mechanistic pathway through which EV inhibit MVEC migration and tube formation.
Asunto(s)
Antígenos CD36/metabolismo , Movimiento Celular , Células Endoteliales/metabolismo , Vesículas Extracelulares/metabolismo , Microvasos/metabolismo , Neovascularización Fisiológica , Transducción de Señal , Animales , Anexina A5/metabolismo , Antígenos CD36/deficiencia , Antígenos CD36/genética , Línea Celular Tumoral , Movimiento Celular/efectos de los fármacos , Células Endoteliales/efectos de los fármacos , Inhibidores Enzimáticos/farmacología , Células Endoteliales de la Vena Umbilical Humana/metabolismo , Humanos , Masculino , Ratones Endogámicos C57BL , Ratones Noqueados , Microvasos/efectos de los fármacos , NADPH Oxidasas/antagonistas & inhibidores , NADPH Oxidasas/metabolismo , Neovascularización Fisiológica/efectos de los fármacos , Fosfatidilserinas/metabolismo , Especies Reactivas de Oxígeno/metabolismo , Transducción de Señal/efectos de los fármacos , Transfección , Familia-src Quinasas/antagonistas & inhibidores , Familia-src Quinasas/metabolismoRESUMEN
BACKGROUND: Long-term outcomes of reversible cerebral vasoconstriction syndrome (RCVS) have not been systematically investigated. METHODS: The following validated questionnaires were mailed to patients recruited from the RCVS registries of two academic hospitals: headache screening form, Headache Impact Test, Migraine Disability Assessment Test, Barthel Index (BI), EuroQoL (EQ-5D-5L) and Patient Health Questionnaire (PHQ-9). RESULTS: Of the 191 patients in the registries, 109 could be contacted and 45 responded. Median follow-up time after symptom onset was 78 months. After RCVS resolution, 24 (53%) patients continued to have headache, but the majority (88%) reported improvement in its severity. Thirteen of the 24 patients with persistent headache had a history of migraine prior to RCVS diagnosis. The majority (97.5%) of respondents were functionally independent based on BI scores. EQ-5D-5L showed better scores in the domains of mobility, self-care and usual activities, as compared to pain and anxiety/depression. Patients with persistent headache had significantly higher levels of EQ-5D-5L pain scores. PHQ-9 scores revealed only one patient (3%) with severe depression. CONCLUSION: More than half of RCVS patients will continue to have chronic headaches of mild to moderate intensity that are distinct from the "thunderclap" headaches at RCVS onset. The vast majority regain complete functional ability. However, pain and anxiety/depression are frequent, often aggravated by concomitant chronic headaches, and may be associated with lower quality of life.
Asunto(s)
Calidad de Vida , Recuperación de la Función , Vasoespasmo Intracraneal/complicaciones , Adulto , Femenino , Estudios de Seguimiento , Cefalea/epidemiología , Cefalea/etiología , Humanos , Masculino , Persona de Mediana Edad , Encuestas y CuestionariosRESUMEN
Autosomal dominant retinal vasculopathy with cerebral leukodystrophy is a microvascular endotheliopathy with middle-age onset. In nine families, we identified heterozygous C-terminal frameshift mutations in TREX1, which encodes a 3'-5' exonuclease. These truncated proteins retain exonuclease activity but lose normal perinuclear localization. These data have implications for the maintenance of vascular integrity in the degenerative cerebral microangiopathies leading to stroke and dementias.
Asunto(s)
Encefalopatías/genética , Exodesoxirribonucleasas/genética , Mutación , Fosfoproteínas/genética , Enfermedades de la Retina/genética , Secuencia de Aminoácidos , Encefalopatías/enzimología , Línea Celular , Núcleo Celular/metabolismo , Citoplasma/metabolismo , Exodesoxirribonucleasas/química , Exodesoxirribonucleasas/metabolismo , Genes Dominantes , Predisposición Genética a la Enfermedad , Humanos , Proteínas Luminiscentes/genética , Proteínas Luminiscentes/metabolismo , Microscopía Confocal , Datos de Secuencia Molecular , Fosfoproteínas/química , Fosfoproteínas/metabolismo , Proteínas Recombinantes de Fusión/genética , Proteínas Recombinantes de Fusión/metabolismo , Enfermedades de la Retina/enzimología , Homología de Secuencia de Aminoácido , TransfecciónRESUMEN
BACKGROUND: Systemic vascular alterations have not been described in reversible cerebral vasoconstriction syndrome (RCVS). We present a case series of RCVS patients having cardiac dysfunction during ictus, with a subset showing complete resolution of cardiomyopathy. METHODS: Retrospective case-series: Cardiac left ventricular ejection fraction (LVEF) and wall motion abnormalities (WMA) visualized on transthoracic echocardiography (TTE), performed during RCVS ictus and follow-up was analyzed. RESULTS: Of 68 patients, 18 (26%) had a TTE performed around ictus. Three of 18 (17%) patients demonstrated WMA on initial TTE. All three patients were female without previous coronary artery disease or heart failure, and were asymptomatic from the cardiac dysfunction. WMA resolved completely on follow-up in Patients 1 and 2. Global LV dysfunction persisted for at least 90 days in Patient 3. CONCLUSION: Although the exact pathophysiology of the cardiomyopathy is uncertain, it may be related to localized coronary vasoconstriction causing myocardial ischemia/infarction. Vasoconstriction may not be limited to the cerebral vasculature and may involve extracerebral organs. Cardiac ventricular abnormalities may be a part of the RCVS spectrum.
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Vasoconstricción , Vasoespasmo Intracraneal/diagnóstico , Vasoespasmo Intracraneal/fisiopatología , Adulto , Cardiomiopatías/diagnóstico , Cardiomiopatías/etiología , Cardiomiopatías/fisiopatología , Femenino , Humanos , Persona de Mediana Edad , Estudios Retrospectivos , Síndrome , Vasoconstricción/fisiología , Vasoespasmo Intracraneal/complicacionesRESUMEN
Central nervous system vasculitis is one of the foremost diagnostic challenges in rheumatology. It results in inflammation and destruction of the vasculature within the CNS. When vasculitis is confined to brain, meninges or spinal cord, it is referred to as primary angiitis of the CNS. Secondary CNS vasculitis occurs in the setting of a systemic vasculitis, auto-inflammatory or infectious disease. Prompt and accurate diagnosis of CNS vasculitis is essential to prevent irreversible brain damage, and to secure precise treatment decisions. Progressive debilitating and unexplained neurological deficits, associated with abnormal cerebrospinal fluid is the typical picture of the disease. Biopsy of the brain remains the gold standard diagnostic test. The differential diagnosis of CNS vasculitis is highly diverse with a broad array of mimics at the clinical, radiographic and angiographic levels.
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Encéfalo , Meninges , Médula Espinal , Vasculitis del Sistema Nervioso Central/clasificación , Vasculitis del Sistema Nervioso Central/diagnóstico , Encéfalo/inmunología , Encéfalo/metabolismo , Encéfalo/patología , Diagnóstico Diferencial , Cefalea/diagnóstico , Cefalea/inmunología , Cefalea/patología , Humanos , Incidencia , Inflamación/inmunología , Inflamación/metabolismo , Inflamación/patología , Meninges/inmunología , Meninges/metabolismo , Meninges/patología , Estudios Retrospectivos , Convulsiones/diagnóstico , Convulsiones/inmunología , Convulsiones/patología , Factores Sexuales , Médula Espinal/inmunología , Médula Espinal/metabolismo , Médula Espinal/patología , Hemorragia Subaracnoidea/diagnóstico , Hemorragia Subaracnoidea/inmunología , Hemorragia Subaracnoidea/patología , Vasculitis del Sistema Nervioso Central/inmunología , Vasoconstricción/inmunologíaRESUMEN
Central nervous system vasculitis (CNSV) is a complex disease that poses considerable diagnostic and therapeutic challenges. It is divided into primary angiitis of the CNS (PACNS), or secondary angiitis of the CNS when associated with systemic conditions. Clinical presentation can be extremely variable and there is no classic presentation. In addition, there is no single diagnostic test and the sensitivity/specificity of all currently available tests is suboptimal. As such, an exhaustive approach with thorough historical data, physical examination, and corroborating investigations should be performed to exclude or confirm a diagnosis of CNSV. Infectious, neoplastic, and autoimmune conditions should be carefully evaluated. Knowledge of CNSV mimics is crucial given the therapeutic and prognostic implications. Reversible cerebral vasoconstriction syndrome is now recognized as the most common mimicker of PACNS. Much progress has been made in the understanding of PACNS histopathology, and specific clinical subsets and their response to treatment. Contrary to historical reports, recent PACNS cohorts achieved favorable clinical outcomes when early diagnosis and prompt treatment was initiated. Successful treatment with newer drugs has also been reported in PACNS cases. Newer imaging modalities hold promise for distinguishing inflammatory from noninflammatory vascular lesions. In this review, the authors provide a broad overview with updates on the types of adult CNSV along with its clinical assessment, diagnosis, and treatment.
Asunto(s)
Vasculitis del Sistema Nervioso Central/diagnóstico , Vasoespasmo Intracraneal/diagnóstico , Diagnóstico Diferencial , Humanos , Examen Físico , PronósticoRESUMEN
Autoimmune diseases are a group of heterogeneous inflammatory disorders characterized by systemic or localized inflammation, leading to ischemia and tissue destruction. These include disorders like systemic lupus erythematosus and related diseases, systemic vasculitides, and central nervous system (CNS) vasculitis (primary or secondary). Headache is a very common manifestation of CNS involvement of these diseases. Although headache characteristics can be unspecific and often non-diagnostic, it is important to recognize because headache can be the first manifestation of CNS involvement. Prompt recognition and treatment is necessary not only to treat the headache, but also to help prevent serious neurological sequelae that frequently accompany autoimmune diseases. In this review, we discuss headache associated with autoimmune diseases along with important mimics.
Asunto(s)
Enfermedades Autoinmunes/complicaciones , Cefalea/etiología , Enfermedades Autoinmunes/diagnóstico , Diagnóstico Diferencial , Cefalea/diagnóstico , HumanosRESUMEN
Reversible cerebral vasoconstriction syndrome (RCVS) is a clinico-radiological syndrome characterized by recurrent thunderclap headache, with or without neurologic symptoms, and reversible vasoconstriction of cerebral arteries. RCVS affects patients in various racial and ethnic groups and in all age groups, although most commonly in the fourth decade of life. Many conditions and exposures have been linked to RCVS, including vasoactive drugs and the peripartum period. Disturbance of the cerebral vascular tone is thought to contribute to the disease's pathophysiology. RCVS generally follows a monophasic course. Associated strokes and cerebral hemorrhages are not uncommon. In this review we will attempt to provide a comprehensive overview of RCVS, with emphasis on the controversies in the field and the newest findings in the reported literature.
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Enfermedades Arteriales Cerebrales/fisiopatología , Cefaleas Primarias/fisiopatología , Vasoconstricción , Vasoespasmo Intracraneal/fisiopatología , Enfermedades Arteriales Cerebrales/diagnóstico , Enfermedades Arteriales Cerebrales/terapia , Diagnóstico Diferencial , Progresión de la Enfermedad , Cefaleas Primarias/etiología , Cefaleas Primarias/terapia , Humanos , Síndrome , Vasoespasmo Intracraneal/diagnóstico , Vasoespasmo Intracraneal/terapiaRESUMEN
Primary angiitis of the central nervous system (PACNS) is a rare idiopathic inflammatory syndrome targeting the vessels of the brain and spinal cord. Clinical presentation is variable, insidious, and non-specific; headache and encephalopathy are the most common symptoms. Multiple strokes affecting numerous vascular territories may be seen, and both focal and diffuse neurologic dysfunction may be present. Cerebrospinal fluid (CSF) analysis is crucial; a normal CSF along with normal brain parenchymal imaging carries a high negative predictive value in excluding PACNS. The role of imaging continues to evolve, and most patients have abnormal vascular imaging; however, the specificity of imaging for PACNS has historically been poor. Cerebral and meningeal biopsy is a valuable tool in confirming the diagnosis and excluding mimics. PACNS generally responds to immunosuppressive therapy. Failure to respond should prompt evaluation for an alternative diagnosis. Given the rarity of this disorder, exclusion of mimics such as the reversible cerebral vasoconstriction syndromes (RCVS) and infectious processes is essential.
Asunto(s)
Vasculitis del Sistema Nervioso Central/diagnóstico , Constricción Patológica/diagnóstico , Diagnóstico Diferencial , Glucocorticoides/uso terapéutico , Humanos , Inmunosupresores/uso terapéutico , Imagen por Resonancia Magnética , Pronóstico , Vasculitis del Sistema Nervioso Central/tratamiento farmacológico , Vasoespasmo Intracraneal/diagnósticoRESUMEN
PURPOSE: The pathogenesis of age-related macular degeneration (AMD) involves aberrant complement activation and is a leading cause of vision loss worldwide. Complement aberrations are also implicated in many systemic immune-mediated inflammatory diseases (IMIDs), but the relationship between AMD and these conditions remains undescribed. The aim of this study is to first assess the association between AMD and IMIDs, and then assess the risk of AMD in patients with specific IMIDs associated with AMD. DESIGN: Cross-sectional study and cohort study. SUBJECTS AND CONTROLS: Patients with AMD were compared with control patients with cataracts and no AMD to ensure evaluation by an ophthalmologist. Patients with IMIDs were compared with patients without IMIDs but with cataracts. METHODS: This study used deidentified data from a national database (2006-2023), using International Classification of Diseases 10 codes to select for IMIDs. Propensity score matching was based on patients on age, sex, race, ethnicity, and smoking. Odds ratios were generated for IMIDs and compared between AMD and control patients. For IMIDs associated with AMD, the risk of AMD in patients with the IMID versus patients without IMIDs was determined utilizing a cohort study design. MAIN OUTCOME MEASURES: Odds ratio of IMID, risk ratios (RRs), and 95% confidence intervals (CIs) of AMD diagnosis, given an IMID. RESULTS: After propensity score matching, AMD and control cohorts (n = 217 197 each) had a mean ± standard deviation age of 74.7 ± 10.4 years, were 56% female, and 9% of patients smoked. Age-related macular degeneration showed associations with systemic lupus erythematosus (SLE), Crohn's disease, ulcerative colitis, rheumatoid arthritis (RA), psoriasis, sarcoidosis, scleroderma, giant cell arteritis, and vasculitis. Cohorts for each positively associated IMID were created and matched to control cohorts with no IMID history. Patients with RA (RR, 1.40; 95% CI, 1.30-1.49), SLE (RR, 1.73; 95% CI, 1.37-2.18), Crohn's disease (RR, 1.42; 95% CI, 1.20-1.71), ulcerative colitis (RR, 1.45; 95% CI, 1.29-1.63), psoriasis (RR, 1.48; 95% CI, 1.37-1.60), vasculitis (RR, 1.48; 95% CI, 1.33-1.64), scleroderma (RR, 1.65; 95% CI, 1.35-2.02), and sarcoidosis (RR, 1.42; 95% CI, 1.24-1.62) showed a higher risk of developing AMD compared with controls. CONCLUSIONS: The results suggest that there is an increased risk of developing AMD in patients with RA, SLE, Crohn's disease, ulcerative colitis, psoriasis, vasculitis, scleroderma, and sarcoidosis compared with patients with no IMIDs. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
Asunto(s)
Degeneración Macular , Puntaje de Propensión , Humanos , Femenino , Masculino , Estudios Transversales , Anciano , Factores de Riesgo , Degeneración Macular/diagnóstico , Degeneración Macular/epidemiología , Estudios Retrospectivos , Persona de Mediana Edad , Inflamación , Anciano de 80 o más Años , Incidencia , Medición de Riesgo/métodos , Estados Unidos/epidemiologíaRESUMEN
BACKGROUND/AIMS: Anti-tumor necrosis factor (Anti-TNF) agents have proven beneficial for the treatment of chronic non-infectious uveitis, yet rare neurological complications and demyelinating disease can occur with their use. Management of uveitis and neurological disease after developing these rare complications is not well understood. We sought to identify these specific cases and their outcomes through a retrospective observational case series. METHODS: Electronic Medical Record (EMR) chart review of 394 non-infectious uveitis patients on anti-TNF therapy focused on identifying patients seen by uveitis specialists at a single institution who were on anti-TNF therapy and had developed neurological symptoms. Cases were reviewed for subsequent management and outcomes of both their neurologic and ocular inflammatory disease. RESULTS: Five (5) patients were included following complaints of neurological symptoms while on anti-TNF therapy. Subsequent demyelinating diagnosis, acute treatment, and long-term course were described. All five patients continue to be inactive at around three years of anti-TNF discontinuation. CONCLUSION: Unidentified rare neurological symptoms and demyelinating disease associated with the use of anti-TNF agents can be detrimental to patient treatment outcomes. Emphasis is given on possible avoidance and early identification of exacerbating underlying disease through a detailed neurologic history and use of imaging when suspicion is high. Patients may have no evidence of higher neurological risk prior to starting an anti-TNF treatment. Discontinuation of an anti-TNF agent and subsequent control of disease is possible with alternative immunosuppressive treatments.