RESUMO
INTRODUCTION: Vasculitides are a heterogeneous group of disorders producing inflammation of blood vessels (e.g. arteries or veins). All major vasculitides potentially have ophthalmological symptoms and signs including visual loss. Co-morbidity, multimorbidity, polypharmacy, and geriatric syndromes all play important roles in patient outcomes for these rheumatic conditions in the elderly. This monograph reviews the NCBI PubMed database (Feb 2023) literature on the neuro-ophthalmic and geriatric considerations in vasculitis. AREAS COVERED: Cogan Syndrome, Granulomatosis with Polyangiitis, Giant Cell Arteritis, Polyarteritis Nodosa, Takayasu Arteritis, Vasculitis epidemiology, and neuro-ophthalmological symptoms. EXPERT OPINION: Geriatric patient care for vasculitis with neuro-ophthalmological manifestations can be complicated by the interplay of multiple co-morbidities, polypharmacy, and specific geriatric syndromes. The valuation and treatment of vasculitis and the complications associated with the disease can negatively impact patient care. Advances in noninvasive imaging and updates in diagnostic criteria have enabled increased identification of patients at earlier stages with less severe disease burden. Novel therapeutic agents can be glucocorticoid sparing and might reduce the adverse effects of chronic steroid use. Holistic care models like the 5 M geriatric care model (mind, mobility, medications, multicomplexity, and matters most) allow patients' needs to be in the forefront with biopsychosocial aspects of a patient being addressed.
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Comorbidade , Vasculite , Transtornos da Visão , Idoso , Síndrome de Cogan/epidemiologia , Arterite de Células Gigantes/epidemiologia , Granulomatose com Poliangiite/epidemiologia , Poliarterite Nodosa/epidemiologia , Arterite de Takayasu/epidemiologia , Vasculite/classificação , Vasculite/diagnóstico , Vasculite/epidemiologia , Vasculite/terapia , Transtornos da Visão/epidemiologia , HumanosRESUMO
Abstract The term vasculitis refers to the inflammation of vessel walls. It may range in severity from a self-limited disorder in one single organ to a life-threatening disease due to multiple organ failure. It has many causes, although they result in only a few histological patterns of vascular inflammation. Vessels of any type and in any organ can be affected, a fact that results in a broad variety of signs and symptoms. Different vasculitides with indistinguishable clinical presentations have quite different prognosis and treatments. This condition presents many challenges to physicians in terms of classification, diagnosis, appropriate laboratory workup, and treatment. Moreover, it compels a careful follow-up. This article reviews the Chapel-Hill 2012 classification, etiology, recent insights in pathophysiology, some important dermatological clues for the diagnosis and summarizes treatment of some of these complex vasculitis syndromes.
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Humanos , Masculino , Feminino , Vasculite/diagnóstico , Vasculite/patologia , Dermatopatias Vasculares/diagnóstico , Dermatopatias Vasculares/patologia , Síndrome , Vasculite/classificação , Dermatopatias Vasculares/classificação , NecroseRESUMO
The term vasculitis refers to the inflammation of vessel walls. It may range in severity from a self-limited disorder in one single organ to a life-threatening disease due to multiple organ failure. It has many causes, although they result in only a few histological patterns of vascular inflammation. Vessels of any type and in any organ can be affected, a fact that results in a broad variety of signs and symptoms. Different vasculitides with indistinguishable clinical presentations have quite different prognosis and treatments. This condition presents many challenges to physicians in terms of classification, diagnosis, appropriate laboratory workup, and treatment. Moreover, it compels a careful follow-up. This article reviews the Chapel-Hill 2012 classification, etiology, recent insights in pathophysiology, some important dermatological clues for the diagnosis and summarizes treatment of some of these complex vasculitis syndromes.
Assuntos
Dermatopatias Vasculares/diagnóstico , Dermatopatias Vasculares/patologia , Vasculite/diagnóstico , Vasculite/patologia , Feminino , Humanos , Masculino , Necrose , Dermatopatias Vasculares/classificação , Síndrome , Vasculite/classificaçãoRESUMO
The skin is one of the organs most commonly affected by vasculitis and the only one that is open to direct inspection. Cutaneous vasculitis can be part of a systemic vasculitis, a variant restricted to the skin (e.g. systemic and cutaneous IgA1 vasculitis) or an independent cutaneous form (recurrent macular vasculitis in hypergammaglobulinemia, nodular vasculitis). For the nomenclature and classification of cutaneous vasculitides the scheme of the Chapel Hill Consensus Conference can be used and the vessels mainly affected determine the clinical picture of individual forms of vasculitis. Some cutaneous efflorescences and their distribution are so characteristic for certain forms of vasculitis that they provide diagnostic indications or even diagnostic criteria. A palpable purpura on the legs is typical for involvement of postcapillary venules in the context of immune complex vasculitis. If arterioles or venules in the dermis are additionally affected, the clinical presentation is that of plaques with marginal offshoots and central hemorrhagic blisters or necrosis (retiform purpura). In contrast to the purpura in occluding vasculopathies, which shows no or little surrounding erythema, the purpura in vasculitis is accompanied by an inflammatory erythema. In vasculitides of the arterioles and small-caliber arteries in the subcutaneous tissue, visible or only palpable nodules exist surrounded by an irregular livedo or also retiform purpura. Understanding how vasculitis-induced efflorescence arises and the histological manifestations helps their recognition during physical examinations in daily practice and is an important guide in the diagnosis and classification of vasculitides.
Assuntos
Exame Físico/métodos , Dermatopatias Vasculares/diagnóstico , Dermatopatias/diagnóstico , Dermatopatias/etiologia , Pele/patologia , Vasculite/classificação , Vasculite/diagnóstico , Eritema , Humanos , Vasculite por IgA , Púrpura , Terminologia como Assunto , Vasculite/complicações , Vasculite/imunologiaRESUMO
"The vasculitic neuropathies encompass a wide range of disorders characterized by ischemic injury to the vasa nervorum. Patients with vasculitic neuropathies develop progressive, painful sensory or sensorimotor deficits that are typically multifocal or asymmetric. Depending on the underlying etiology, the vasculitis may be confined to the peripheral nervous system; may be one manifestation of a primary systemic vasculitis; or one manifestation of a systemic vasculitis that is secondary to underlying connective tissue disease, drug exposure, viral infection, or paraneoplastic syndrome. This article reviews the classification, clinical presentation, diagnostic approach, etiologies, and treatment of the vasculitic neuropathies."
Assuntos
Doenças do Sistema Nervoso Periférico/classificação , Doenças do Sistema Nervoso Periférico/patologia , Vasculite/classificação , Vasculite/patologia , HumanosRESUMO
OBJECTIVES: The frequency of different vasculitides and their characteristics vary among different regions. The identification of geographic disparities of disease phenotypes helps the development of international criteria, allowing the classification of patients of different ethnicities. This study aimed to describe the frequency, characteristics, course, response to treatment, and outcome of the different adulthood vasculitides in Egypt. METHODS: This was a multicenter study in which the medical records of adult Egyptian patients diagnosed with vasculitis between 2002 and 2018 were retrospectively reviewed. RESULTS: The most frequent vasculitides in Egypt were Behçet's disease (76%), hepatitis C virus vasculitis (13.9%), and granulomatosis with polyangiitis (3.9%). Most patients (73.8%) had a major event at the time of diagnosis. Generalized granulomatosis with polyangiitis was more common than the localized type (90% versus 10%, respectively). The aortic arch and its branches were the most common affected sites of Takayasu arteritis. Of vasculitides, Behçet's disease and giant cell arteritis were associated with the greatest rates of relapse (62.7% and 33.3%, respectively). Delayed diagnosis and permanent organ damage were reported in 69.9% and 68.9% of patients, respectively. A low mortality rate was noted (1.3%). CONCLUSIONS: The most common types of adulthood vasculitides in Egypt are Behçet's disease, hepatitis C virus vasculitis, and granulomatosis with polyangiitis. Major organ involvement is frequent. Delayed diagnosis and permanent organ damage are common.
Assuntos
Vasculite/classificação , Vasculite/epidemiologia , Adulto , Idoso , Síndrome de Behçet/epidemiologia , Egito/epidemiologia , Feminino , Granulomatose com Poliangiite/epidemiologia , Humanos , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
Vasculitis mimics need to be differentiated from primary and secondary vasculitides as described in the Chapel Hill nomenclature. The clinical symptomatology resembles that of vasculitis of small and medium, rarely also large vessels and hence imitates the classical vasculitic disorders. Pathogenetically, the causes are partly genetic mutations, embolization syndromes, infections and substance abuse. Also, Bcell lymphomas can mimic vasculitis. The present manuscript summarizes the vasculitis mimics.
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Vasculite , Diagnóstico Diferencial , Humanos , Vasculite/classificação , Vasculite/diagnósticoRESUMO
Connective tissue diseases often prominently affect the skin, requiring dermatologists to play an important role in diagnosis and treatment of these patients. Herein we describe updates on the pathogenesis, clinical features, and treatment of 4 major connective tissue diseases: dermatomyositis, cutaneous lupus erythematosus, limited scleroderma (morphea), and cutaneous vasculitis. Many of these updates promise to improve clinical care of patients who suffer from dermatologic involvement of these diseases and are the result of research performed by dermatologists who have expertise in these conditions.
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Dermatomiosite/classificação , Dermatomiosite/tratamento farmacológico , Lúpus Eritematoso Cutâneo/tratamento farmacológico , Esclerodermia Localizada/terapia , Antimaláricos/uso terapêutico , Biomarcadores/sangue , Humanos , Vasculite por IgA/diagnóstico , Lúpus Eritematoso Cutâneo/fisiopatologia , Esclerodermia Localizada/sangue , Esclerodermia Localizada/classificação , Dermatopatias Vasculares/classificação , Dermatopatias Vasculares/diagnóstico , Dermatopatias Vasculares/tratamento farmacológico , Vasculite/classificação , Vasculite/diagnóstico , Vasculite/tratamento farmacológicoAssuntos
Aterosclerose/classificação , Displasia Fibromuscular/classificação , Terminologia como Assunto , Varizes/classificação , Vasculite/classificação , Adulto , Fatores Etários , Aterosclerose/diagnóstico , Feminino , Displasia Fibromuscular/diagnóstico , Displasia Fibromuscular/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Fatores Sexuais , Varizes/diagnóstico , Vasculite/diagnóstico , Vasculite/epidemiologiaRESUMO
PURPOSE OF REVIEW: Cutaneous vasculitis reflects a spectrum ranging from skin limited to severe systemic forms. To date, there is still no generally acknowledged nomenclature for cutaneous vasculitis. This review aims to summarize the recent advances in the nomenclature of cutaneous vasculitis. RECENT FINDINGS: The most widely adopted vasculitis classification system is the one of 2012 Revised Chapel Hill Consensus Conference (CHCC) which represent not such a classification but a nomenclature system that name vasculitis on the basis of the size of the vessel affected. The CHCC 2012 did not deal with the special features of cutaneous vasculitis and did not explicitly discuss the presence of skin-limited or skin-dominant forms of vasculitis. Therefore, a consensus group was formed to propose an Addendum to CHCC 2012, focusing on cutaneous vasculitis. The Addendum better clarify the main aspects of some single-organ vasculitis, including IgM/IgG vasculitis, nodular vasculitis, erythema elevatum et diutinum and recurrent macular vasculitis in hypergammaglobulinemia. Moreover, it differentiated normocomplementemic from hypocomplementemic urticarial vasculitis. Finally, it recognized cutaneous polyarteritis nodosa as a distinct subtype of polyarteritis nodosa. SUMMARY: Classification criteria are useful tools to standardize names and definitions for cutaneous vasculitis; however, they do not represent diagnostic criteria. Collaborative efforts are still needed to get a shared classification and valid diagnostic criteria for cutaneous vasculitis.
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Dermatopatias Vasculares/diagnóstico , Pele/patologia , Vasculite/diagnóstico , Humanos , Dermatopatias Vasculares/classificação , Dermatopatias Vasculares/patologia , Terminologia como Assunto , Vasculite/classificação , Vasculite/patologiaRESUMO
What's new in internal medicine will be dedicated to three topics: i) inflammatory myopathies constituting a heterogenous group of diseases whose clinical manifestations, immunological abnormalities, treatment response and outcomes vary widely; ii) alterations of gut microbiota contributing to the occurrence or development of a range of conditions, including autoimmune diseases for which further work is necessary to understand the correlation of dysbiosis with these diseases; iii) the reciprocal relationship between obesity, metabolic syndrome, atherosclerosis and autoimmune diseases. New data concerning systemic sclerosis, cutaneous vasculitis, adult Still's disease, autoantibodies anti DFS70, Epstein Barr virus and autoimmune diseases were also highlighted.
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Doenças Autoimunes/etiologia , Microbioma Gastrointestinal , Miosite/diagnóstico , Aterosclerose/complicações , Antígenos Nucleares do Vírus Epstein-Barr/genética , Antígenos Nucleares do Vírus Epstein-Barr/imunologia , Humanos , Medicina Interna/tendências , Síndrome Metabólica/complicações , Miosite/classificação , Miosite/terapia , Obesidade/complicações , Terminologia como Assunto , Vasculite/classificação , Vasculite/diagnóstico , Proteínas Virais/genética , Proteínas Virais/imunologia , Deficiência de Vitamina D/complicaçõesRESUMO
Primary vasculitides that affect children are a challenging and complex group of disorders that may involve any system of the body and lead to significant morbidity and mortality. In recent years, there have been significant advances in the field of childhood vasculitides, including the development of classification criteria and outcome assessment. Although some forms of vasculitis occur in both children and adults, considerable differences exist between childhood and adult vasculitides; we review childhood vasculitides, thus highlighting their differences with the adult forms of the disease. We will also discuss monogenic forms of vasculitis, such as deficiency of adenosine deaminase type 2 (DADA2) and haploinsufficiency of A20 (HA20).
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Vasculite/classificação , Criança , Humanos , Vasculite/patologiaRESUMO
The range of pathologies that are related to primitive vasculitis is broad, complex and not as typical as we would expect. Clinicians should be aware that several forms of primitive and systemic vasculitis, regardless of the size of the affected vessel, may exhibit identical histological alterations. This observation has important clinical implications as it means that cases of vasculitis do not correspond clinically and histologically. Thus, while histology remains the diagnostic gold standard, it can be used only as part of the most complete clinical assessment possible. Another point worth of the clinician's attention is that vasculitis histology changes over time, as do disease evolution and activity, even without considering the masking effects of treatment and the possibility of sampling error due to the patchy occurrence of vasculitis. The purpose of this review is to identify the most common forms of vasculitis in clinical practice, and to provide guidance to the clinician on the pathology of the vessels.
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Vasculite/patologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/patologia , Vasos Sanguíneos/ultraestrutura , Eritema Nodoso/patologia , Arterite de Células Gigantes/classificação , Arterite de Células Gigantes/patologia , Humanos , Tamanho do Órgão , Especificidade de Órgãos , Fibrose Retroperitoneal/patologia , Arterite de Takayasu/patologia , Vasculite/classificação , Vasculite/diagnósticoRESUMO
The proposal by the 1994 International Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitides (CHCC1994) and by the CHCC2012 markedly influenced the classification and way of considering cutaneous vasculitis. In the proposal by the CHCC1994, hypersensitivity angiitis was defined as an equivalent pathological condition to microscopic polyangiitis or cutaneous leukocytoclastic angiitis (CLA), and it was not adopted as a disease name. However, CLA which was positioned as a type of small-vessel vasculitis is only a pathological name. In the proposal by the CHCC2012, a new category of single-organ vasculitis included CLA and cutaneous arteritis. Vasculitis allergica cutis (Ruiter) corresponded to CLA and cutaneous polyarteritis nodosa corresponded to cutaneous arteritis. The Japanese Dermatological Association (JDA) prepared guidelines for the management of vasculitis and vascular disorders in 2008 based on the proposal by the CHCC1994 and their original viewpoint of dermatology. The JDA subsequently revised the 2008 edition guidelines in 2016 following publication of the proposal of the CHCC2012 in Japanese. We presented the outline of the 2016 edition guidelines and propose a treatment algorithm for primary vasculitides based on the evaluation of the cutaneous symptoms for cases suspected as primary cutaneous vasculitides, which integrates the 2008 JDA guideline and CHCC2012 classification. This is the secondary English version of the original Japanese manuscript for the guideline for management of vasculitis and vascular disorders published in the Japanese Journal of Dermatology 127(3); 299-415, 2017.
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Dermatologia/normas , Dermatopatias Vasculares/terapia , Vasculite/terapia , Dermatologia/métodos , Humanos , Japão , Pele/irrigação sanguínea , Pele/patologia , Dermatopatias Vasculares/classificação , Dermatopatias Vasculares/patologia , Vasculite/classificação , Vasculite/patologiaRESUMO
OBJECTIVE: To prepare a dermatologic addendum to the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides (CHCC2012) to address vasculitides affecting the skin (D-CHCC). The goal was to standardize the names and definitions for cutaneous vasculitis. METHODS: A nominal group technique with a facilitator was used to reach consensus on the D-CHCC nomenclature, using multiple face-to-face meetings, e-mail discussions, and teleconferences. RESULTS: Standardized names, definitions, and descriptions were adopted for cutaneous components of systemic vasculitides (e.g., cutaneous IgA vasculitis as a component of systemic IgA vasculitis), skin-limited variants of systemic vasculitides (e.g., skin-limited IgA vasculitis, drug-induced skin-limited antineutrophil cytoplasmic antibody-associated vasculitis), and cutaneous single-organ vasculitides that have no systemic counterparts (e.g., nodular vasculitis). Cutaneous vasculitides that were not included in the CHCC2012 nomenclature were introduced. CONCLUSION: Standardized names and definitions are a prerequisite for developing validated classification and diagnostic criteria for cutaneous vasculitis. Accurate identification of specifically defined variants of systemic and skin-limited vasculitides requires knowledgeable integration of data from clinical, laboratory, and pathologic studies. This proposed nomenclature of vasculitides affecting the skin, the D-CHCC, provides a standard framework both for clinicians and for investigators.
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Dermatopatias Vasculares/diagnóstico , Vasculite/diagnóstico , Consenso , Diagnóstico Diferencial , Humanos , Pele/irrigação sanguínea , Pele/patologia , Dermatopatias Vasculares/classificação , Terminologia como Assunto , Vasculite/classificaçãoRESUMO
Nonsystemic vasculitic neuropathy (NSVN) is an under-recognized single-organ vasculitis of peripheral nerves that can only be diagnosed with a nerve biopsy. A Peripheral Nerve Society guideline group published consensus recommendations on the classification, diagnosis and treatment of NSVN in 2010, and new diagnostic criteria for vasculitic neuropathy were developed by the Brighton Collaboration in 2015. In this Review, we provide an update on the classification, diagnosis and treatment of NSVN. NSVN subtypes include Wartenberg migratory sensory neuropathy and postsurgical inflammatory neuropathy. Variants include diabetic radiculoplexus neuropathy and - arguably - neuralgic amyotrophy. NSVN with proximal involvement is sometimes termed nondiabetic lumbosacral radiculoplexus neuropathy. Cutaneous polyarteritis nodosa and other skin-nerve vasculitides overlap with NSVN clinically. Three patterns of involvement in NSVN have been identified: multifocal neuropathy, distal symmetric polyneuropathy, and overlapping multifocal neuropathy (asymmetric polyneuropathy). These patterns lack standard definitions, resulting in inconsistencies between studies. We propose definitions and provide an up-to-date differential diagnosis of multifocal neuropathy. Available evidence suggests that NSVN and neuropathy-predominant systemic vasculitis might be controlled better by treatment with corticosteroids and an immunosuppressive agent than with corticosteroids alone. Treated NSVN rarely spreads to other organs, but 30% of patients experience a relapse. Long-term neurological outcome is favourable, but chronic pain is common.
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Polineuropatias/diagnóstico , Vasculite/diagnóstico , Humanos , Polineuropatias/classificação , Polineuropatias/etiologia , Polineuropatias/terapia , Vasculite/classificação , Vasculite/complicações , Vasculite/terapiaAssuntos
Amiloidose/classificação , Pneumopatias/classificação , Neoplasias Epiteliais e Glandulares/classificação , Patologia Clínica , Patologia Cirúrgica , Neoplasias do Timo/classificação , Vasculite/classificação , Amiloidose/patologia , Anticorpos Anticitoplasma de Neutrófilos , Humanos , Pneumopatias/patologia , Neoplasias Epiteliais e Glandulares/patologia , Neoplasias do Timo/patologia , Vasculite/patologiaAssuntos
Imunização/efeitos adversos , Doenças do Sistema Nervoso Periférico , Vasculite , Coleta de Dados , Humanos , Doenças do Sistema Nervoso Periférico/classificação , Doenças do Sistema Nervoso Periférico/diagnóstico , Doenças do Sistema Nervoso Periférico/imunologia , Estatística como Assunto , Vasculite/classificação , Vasculite/diagnóstico , Vasculite/imunologia , Vasculite/terapiaRESUMO
Not available.