ABSTRACT
Abstract Background: The Henoch-Schonlein Purpura (HSP) or IgA vasculitis is the most common vasculitis of childhood and may occur with renal involvement, with hematuria and / or proteinuria, and may cause severe and non-reversible sequelae. Objectives: To establish the profile of patients with renal involvement due to IgA vasculitisand to describe our experience with the use of azathioprine to treat patients with nephritis. Methods: Clinical data were retrospectively collected from medical records of patients with IgA vasculitiswho attended the pediatric rheumatology unit between 1995 and 2017. Patients were separated into two groups based on whether or notthey weretreated with non-glucocorticoid immunosuppressants. Results: From the178 patients with IgA vasculitis, nephritis was found in67 patients (37.6%), 13 of whom receivedtreatment with non-glucocorticoid immunosuppressants. Ten patients responded well to azathioprine and 1 patient to cyclosporine. Forty patients received oral glucocorticoids, whilst 16received intravenous glucocorticoids. Conclusion: Azathioprine may be beneficial in the treatment of IgA vasculitis with renal involvement.
Subject(s)
Humans , IgA Vasculitis/physiopathology , Azathioprine/therapeutic use , Vasculitis/physiopathology , Nephritis/drug therapy , Health ProfileABSTRACT
Abstract Objectives: To assess clinical digital vasculitis (DV) as an initial manifestation of childhood-onset systemic lupus erythematosus (cSLE) within a large population. Methods: Multicenter cross-sectional study including 852 cSLE patients (ACR criteria) followed in ten Pediatric Rheumatology centers in São Paulo State, Brazil. Results: DV was observed in 25/852 (3%) cSLE patients. Periungual hemorrhage was diagnosed in 12 (48%), periungual infarction in 7 (28%), tip finger ulceration in 4 (16%), painful nodules in 1 (4%) and gangrene in 1 (4%). A poor outcome, with digital resorption, occurred in 5 (20%). Comparison of patients with and without DV revealed higher frequency of malar rash (80% vs. 53%, p = 0.008), discoid rash (16% vs. 4%, p = 0.017), photosensitivity (76% vs. 45%, p = 0.002) and other cutaneous vasculitides (80% vs. 19%, p < 0.0001), whereas the frequency of overall constitutional features (32% vs. 61%, p = 0.003), fever (32% vs. 56%, p = 0.020) and hepatomegaly (4% vs. 23%, p = 0.026) were lower in these patients. Frequency of female gender, severe multi-organ involvement, autoantibodies profile and low complement were alike in both groups (p > 0.05). SLEDAI-2K median, DV descriptor excluded, was significantly lower in patients with DV compared to those without this manifestation [10 (0-28) vs. 14 (0-58), p = 0.004]. Visceral vasculitis or death were not observed in this cSLE cohort. The frequency of cyclophosphamide use (0% vs. 18%, p = 0.014) was significantly lower in the DV group. Conclusion: Our large multicenter study identified clinical DV as one of the rare initial manifestation of active cSLE associated with a mild multisystemic disease, in spite of digital resorption in some of these patients.
Resumo Objetivos: Avaliar a vasculite digital (VD) clínica como uma manifestação inicial do lúpus eritematoso sistêmico de início na infância (LESi) em uma grande população. Métodos: Estudo transversal multicêntrico que incluiu 852 pacientes com LESi (critérios do ACR), acompanhados em dez centros de reumatologia pediátrica do Estado de São Paulo. Resultados: Observou-se VD em 25/852 (3%) pacientes com LESi. Diagnosticaram-se hemorragia periungueal em 12 (48%), infarto periungueal em sete (28%), úlcera de ponta de dígito em quatro (16%), nódulos dolorosos em um (4%) e gangrena em um (4%). Um desfecho ruim, com reabsorção digital, ocorreu em cinco (20%) pacientes. A comparação entre pacientes com e sem VD revelou maior frequência de erupção malar (80% vs. 53%, p = 0,008), erupção discoide (16% vs. 4%, p = 0,017), fotossensibilidade (76% vs. 45% p = 0,002) e outras vasculites cutâneas (80% vs. 19%, p < 0,0001), enquanto a frequência de características constitucionais totais (32% vs. 61%, p = 0,003), febre (32% vs. 56% p = 0,020) e hepatomegalia (4% vs. 23%, p = 0,026) foram menores nesses pacientes. A frequência do gênero feminino, o envolvimento grave de múltiplos órgãos, perfil de autoanticorpos e baixo complemento foram semelhantes nos dois grupos (p > 0,05). A mediana no Sledai-2 K, exclusive o descritor de VD, foi significativamente menor nos pacientes com VD em comparação com aqueles sem essa manifestação [10 (0 a 28) vs. 14 (0 a 58), p = 0,004]. Não foram observadas vasculite visceral nem morte nessa coorte de pacientes com LESi. A frequência de uso de ciclofosfamida (0% vs. 18%, p = 0,014) foi significativamente menor no grupo VD. Conclusão: Este grande estudo multicêntrico identificou a VD clínica como uma rara manifestação inicial do LESi ativo, associada a doença multissistêmica leve, apesar da ocorrência de reabsorção digital em alguns desses pacientes.
Subject(s)
Humans , Female , Child , Adolescent , Vasculitis/epidemiology , Toes , Fingers , Lupus Erythematosus, Systemic/epidemiology , Vasculitis/etiology , Vasculitis/physiopathology , Severity of Illness Index , Brazil/epidemiology , Case-Control Studies , Cross-Sectional Studies , Retrospective Studies , Age of Onset , Lupus Erythematosus, Systemic/physiopathologyABSTRACT
Vasculitides comprise a heterogeneous group of autoimmune disorders, occurring as primary or secondary to a broad variety of systemic infectious, malignant or connective tissue diseases. The latter occur more often but their pathogenic mechanisms have not been fully established. Frequent and varied central and peripheral nervous system complications occur in vasculitides and connective tissue diseases. In many cases, the neurological disorders have an atypical clinical course or even an early onset, and the healthcare professionals should be aware of them. The purpose of this brief review was to give an update of the main neurological disorders of common vasculitis and connective tissue diseases, aiming at accurate diagnosis and management, with an emphasis on pathophysiologic mechanisms.
As vasculites são um grupo heterogêneo de doenças autoimunes primárias ou secundárias a uma grande variedade de doenças infecciosas sistêmicas, malignas ou do tecido conjuntivo. Estas últimas são as que ocorrem com mais frequência, porém os mecanismos patogênicos ainda não foram plenamente determinados. Diversas e frequentes complicações do sistema nervoso central e periférico ocorrem nas vasculites e doenças do tecido conjuntivo. Em muitos casos, os distúrbios neurológicos têm evolução clínica atípica ou mesmo início precoce, ao que todos os profissionais de saúde devem estar cientes. O objetivo desta breve revisão foi atualizar os principais distúrbios neurológicos da vasculite comum e das doenças do tecido conjuntivo, visando ao diagnóstico e ao tratamento corretos, com ênfase nos mecanismos fisiopatológicos.
Subject(s)
Humans , Autoimmune Diseases/complications , Connective Tissue Diseases/complications , Vasculitis/complications , Arthritis, Rheumatoid/complications , Arthritis, Rheumatoid/diagnosis , Arthritis, Rheumatoid/physiopathology , Autoimmune Diseases/diagnosis , Autoimmune Diseases/physiopathology , Behcet Syndrome/complications , Behcet Syndrome/diagnosis , Behcet Syndrome/physiopathology , Churg-Strauss Syndrome/complications , Churg-Strauss Syndrome/diagnosis , Churg-Strauss Syndrome/physiopathology , Connective Tissue Diseases/diagnosis , Connective Tissue Diseases/physiopathology , Giant Cell Arteritis/complications , Giant Cell Arteritis/diagnosis , Giant Cell Arteritis/physiopathology , Granulomatosis with Polyangiitis/complications , Granulomatosis with Polyangiitis/diagnosis , Granulomatosis with Polyangiitis/physiopathology , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/physiopathology , Polyarteritis Nodosa/complications , Polyarteritis Nodosa/diagnosis , Polyarteritis Nodosa/physiopathology , Scleroderma, Systemic/complications , Scleroderma, Systemic/diagnosis , Scleroderma, Systemic/physiopathology , Sjogren's Syndrome/complications , Sjogren's Syndrome/diagnosis , Sjogren's Syndrome/physiopathology , Takayasu Arteritis/complications , Takayasu Arteritis/diagnosis , Takayasu Arteritis/physiopathology , Vasculitis, Central Nervous System/complications , Vasculitis, Central Nervous System/diagnosis , Vasculitis, Central Nervous System/physiopathology , Vasculitis/diagnosis , Vasculitis/physiopathologyABSTRACT
A extensa rede vascular pulmonar e brônquica, associada à grande quantidade de células imunocompetentes, torna os pulmões importante alvo de vasculites imunomediadas. O envolvimento destes vasos sanguíneos pode provocar qualquer sintoma respiratório, dependendo do tipo, local e extensão da lesão no sistema vascular respiratório. Algumas situações ameaçadoras da vida podem resultar, abrindo ou acompanhando o quadro destas patologias e, se não prontamente identificadas e tratadas, podem resultar em risco elevado de morbimortalidade. Destacamos duas destas condições especiais - estenoses de grande vias aéreas e hemorragia alveolar disusa - e descrevemos seus aspectos fisiopatológicos, clínicos e terapêuticos
The extensive pulmonary and bronchial vascular system, associated to large amount of immunocompetent cells, makes the respiratory system an important target of immune mediated vasculitis. These blood vessels involvement can cause any respiratory symptoms, depending on the type, location, and degree of lesion in the pulmonary vascular system. Some life-thereatening conditions may ensue, opening or following the course of those deseases and, if not identified and treated promptly, result in increased risk of morbitidy and mortality. We highlight two of those special situations: large airway stenosis and diffuse alveolar hemorrhage, describing their pathophysiological clinical and therapeutic aspects
Subject(s)
Humans , Male , Female , Tracheal Stenosis/physiopathology , Tracheal Stenosis/therapy , Vasculitis/diagnosis , Vasculitis/physiopathology , Vasculitis/therapy , Lung DiseasesABSTRACT
Oxidative low-density lipoprotein (Ox-LDL) is a key risk factor for the development of atherosclerosis, and it can stimulate the expression of a variety of inflammatory signals. As a new and highly sensitive inflammation index, OX40L may be a key to understanding the mechanisms that regulate interactions between cells within the vessel wall and inflammatory mediators during the development of atherosclerosis. To investigate whether Ox-LDL regulates OX40L expression through an oxidized LDL-1 receptor (LOX-1)-mediated mechanism, we investigated the effect of different concentrations of Ox-LDL (50, 100, 150 µg/mL) on endothelial cell proliferation and apoptosis. Stimulation with Ox-LDL increased OX40L protein 1.44-fold and mRNA 4.0-fold in endothelial cells, and these effects were inhibited by blocking LOX-1. These results indicate that LOX-1 plays an important role in the chronic inflammatory process in blood vessel walls. Inhibiting LOX-1 may reduce blood vessel inflammation and provide a therapeutic option to limit atherosclerosis progression.
Subject(s)
Humans , Apoptosis/drug effects , Cell Proliferation/drug effects , Human Umbilical Vein Endothelial Cells/drug effects , Lipoproteins, LDL/pharmacology , /metabolism , Scavenger Receptors, Class E/metabolism , Atherosclerosis/etiology , Atherosclerosis/prevention & control , Cell Cycle , Cells, Cultured , Human Umbilical Vein Endothelial Cells/cytology , Human Umbilical Vein Endothelial Cells/metabolism , Immunoblotting , Lipoproteins, LDL/metabolism , Lipoproteins, LDL/physiology , /genetics , Real-Time Polymerase Chain Reaction , Signal Transduction , Vasculitis/physiopathology , Vasculitis/prevention & controlABSTRACT
Behçet's disease (BD) is a multi-system inflammatory disorder which presents with recurrent orogenital ulceration, uveitis, and erythema nodosum. Medium vessel vasculitis of upper limb is extremely rare and it is only reported in patients with Behçet's disease on long follow up. Mean duration from diagnosis of disease to development of vasculitis is 5.8 years. We present a patient who presented with gangrene of fingers with absent radial pulse and during course of his illness he developed features of Behçet's disease. Diagnosis was established by clinical features and histopathology and patient was treated with steroids and colchicine.
Subject(s)
Adrenal Cortex Hormones/therapeutic use , Arterial Occlusive Diseases/diagnosis , Behcet Syndrome/diagnosis , Colchicine/therapeutic use , Erythema Nodosum/physiopathology , Humans , Inflammation , Male , Middle Aged , Radial Artery/pathology , Tubulin Modulators/therapeutic use , Uveitis/physiopathology , Vasculitis/physiopathologyABSTRACT
Foram analisados 50 pacientes com diagnóstico de granulomatose de Wegener (GW) atendidos no Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, entre 1985 e 2000, de acordo com sexo e idade, sinais e sintomas. Destes 50 pacientes, 14 foram selecionados com material adequado para análise histológica, ensaio de TUNEL e imunohistoquímica para CD34. Como controles, biópsias de pacientes com vasculite leucocitoclástica e tuberculose. Apoptose foi positiva em 11 das 14 amostras dos pacientes com GW e ausente nos controles. As células foram confirmadas como endotélio pela imunohistoquímica para CD34. Apoptose pode ter um papel na patogênese da granulomatose de Wegener / We analyzed 50 patients with the diagnosis of Wegeners granulomatosis (WG) from Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, from 1985 to 2000 regarding clinical presentation, sites of involvement and diagnostic procedures. Fourteen patients were selected with adequate tissue samples for histological analysis, TUNEL assay and CD34-immunohistochemistry. Biopsies from patients with leukocytoclastic vasculitis and tuberculosis were used as controls. Apoptosis was present in 11 of 14 patients with WG and in none of the controls, confirmed as endothelium by immunohistochemistry for CD34. Apoptosis may play a role in the pathogenesis of Wegeners granulomatosis...
Subject(s)
Humans , Male , Female , Adolescent , Adult , Apoptosis , Granulomatosis with Polyangiitis/physiopathology , Vasculitis/physiopathology , /analysis , Biopsy/methods , Endothelium, Vascular/physiopathology , Immunohistochemistry , In Situ Nick-End Labeling/methodsABSTRACT
Distintos tipos infecciosos como bacterias, virus, parásitos y hongos pueden actuar como factores causales o desencadenantes de las vasculitis cutáneo-sistémicas. Los mecanismos involucrados abarcan desde el ataque directo a los tejidos hasta la alteración de la respuesta inmune. La infección como factor precipitante es vasculitis es poco frecuente. La presencia de lesiones sospechosas de vasculitis requiere medir la extensión del proceso, lo cual significa descartar patología sistémica. Las lesiones cutáneo-mucosas características son púrpura palpable, ronchas, ampollar, úlceras y nódulos. Estas dependen de la extensión y profundidad del infiltrado inflamatorio. Las lesiones nodulares y ulceraciones señalan mayor profundidad y compromiso de vasos de mayor calibre. El diagnóstico diferencial de estos cuadros se hará con todos los rash eritematopurpúricos debidos a cuadros infecciosos, vasculitis primarias o secundarias a colangiopatías, drogas o neoplasias y con las púrpuras. El tratamiento de las vasculitis cutáneo-sistémicas asociadas a infecciones dependerá del germen causal, del mecanismo de acción del mismo y del estado general del paciente. Se tendrá en consideración las situaciones epidemiológicas particulares de los pacientes con SIDA e inmunodeprimidos que así lo requieran
Subject(s)
Humans , Vasculitis/microbiology , Purpura/etiology , Vasculitis/diagnosis , Vasculitis/physiopathologyABSTRACT
El fenómeno de Lucio es una leprorreacción necrotizante infreceunte que ocurre en la forma de lepra lepromatosa difusa e infiltrativa conocida como lepra de Lucio. Se presenta el caso de un paciente con lepra de Lucio quien en el transcurso de su enfermedad desarrolló glomerulonefritis proliferaliva, fenómeno de Lucio y eritema nodoso leproso. se consiguió una evolución terapéutica satisfactoria con farmacoterapia triconjugada convencional asociada con esteroides sintémicos
Subject(s)
Humans , Kidney Diseases/diagnosis , Kidney Diseases/etiology , Kidney Diseases/physiopathology , Leprosy, Lepromatous/diagnosis , Leprosy, Lepromatous/drug therapy , Leprosy, Lepromatous/epidemiology , Leprosy, Lepromatous/etiology , Leprosy, Lepromatous/physiopathology , Vasculitis/complications , Vasculitis/etiology , Vasculitis/physiopathologySubject(s)
Leprosy, Lepromatous/diagnosis , Leprosy, Lepromatous/epidemiology , Leprosy, Lepromatous/etiology , Leprosy, Lepromatous/physiopathology , Leprosy, Lepromatous/drug therapy , Kidney Diseases/diagnosis , Kidney Diseases/etiology , Kidney Diseases/physiopathology , Vasculitis/complications , Vasculitis/etiology , Vasculitis/physiopathologyABSTRACT
La poliarteritis o poliangeitis microscópica es considerada como una vasculitis necrotizante sistémica que se asocia al Sindrome Pulmón-riñon cursando con hemorragia alveolar difusa y glomerulonefritis necrotizante segmentaria con compromiso multisistémico. Se presenta un caso de esta entidad, en un paciente de 50 años con su compromiso clínico, hallazgos imagenológicos, de laboratorio e histopalógicos, así como criterios diagnósticos
Subject(s)
Humans , Male , Adult , Polyarteritis Nodosa/diagnosis , Polyarteritis Nodosa/ethnology , Polyarteritis Nodosa/etiology , Vasculitis/physiopathologySubject(s)
Humans , Vasculitis/classification , Vasculitis/physiopathology , Vasculitis/drug therapy , Granulomatosis with Polyangiitis/drug therapy , Adrenal Cortex Hormones/administration & dosage , Adrenal Cortex Hormones/therapeutic use , Critical Illness/therapy , Polyarteritis Nodosa/drug therapy , Cryoglobulinemia/drug therapy , IgA Vasculitis/drug therapy , Behcet Syndrome/drug therapy , Mucocutaneous Lymph Node Syndrome/drug therapyABSTRACT
Objetivo: realizar una revisión de la literatura reciente con respecto a la utilidad de marcadores de actividad de las vasculitis. Fuente de datos: se realizó una búsqueda en la base de datos Medline (1989 a 1996), que comprendía tópicos relacionados con criterios de actividad clínica y de laboratorio de las vasculitis, además de los estudios que se han adelantado con respecto a las moléculas de adhesión, las citoquinas, los ANCAs, los anticuerpos anti-célula endotelial, el factor reumatoideo, la proteína C reactiva y los marcadores de daño endotelial en lo que respecta a su participación patogénica y su posible aplicabilidad clínica. Selección del estudio: se estudiaron 450 resúmenes de los cuales, de los cuales 90 informaban sobre tópicos relacionados con el tema. Extracción de los datos: los artículos se clasificaron de acuerdo con sus objetivos y estrategias de ejecución, según se trataran de revisiones, artículos originales o informes de casos. Recopilamos y analizamos los artículos originales y luego elaboramos la revisión, los resúmenes y las conclusiones. Síntesis de datos: los estudios recientes con respecto a los niveles séricos de moléculas de adhesión, citoquinas y diversos anticuerpos, que permiten entender la patogénesis de la vasculitis, también hacen plantear al clínico y a los investigadores su posible utilidad como elementos clasificatorios de diagnóstico, actividad y daño, en una forma más precisa, con un enfoque creíble, estandarizado, reproducible y de fácil ejecución. Conclusión: los criterios de actividad y daño de las vasculitis, son elementos que facilitan la labor del médico que trata este tipo de pacientes, dado que le permiten definir el tipo y grado de inmunosupresión, la diferenciación de recaídas y de reacciones adversas medicamentosas o infecciones concomitantes. También son útiles al investigador que evalúa las respuestas de medicamentos o los aspectos biológicos y bioquímicos de estas enfermedades
Subject(s)
Humans , Vasculitis/classification , Vasculitis/complications , Vasculitis/diagnosis , Vasculitis/drug therapy , Vasculitis/epidemiology , Vasculitis/etiology , Vasculitis/physiopathology , Vasculitis/therapyABSTRACT
La asociación de vasculitis con enfermedades del tejido conectivo es bien conocida y con frecuencia producen eventos oclusivos vasculares; informamos los casos de un paciente con lupus eritematoso sistémico y otro con artritis reumatoidea, con vasculitis reumatoidea y síndrome antifosfolípido secundario, que desarrollaron oclusión arterial en miembros inferiores y que requirieron amputación en quienes se documentó la presencia de vasculitis, vasculopatía y trombo organizado. La pérdida dramática de tejido es rara para vasculitis aislada o síndrome antifosfolípido primario. Sugerimo en pacientes con estas enfermedades que cursen con compromiso vascular rápidamete progresivo sospechar la asociación de vasculitis y trombosis e iniciar manejo agresivo con inmunosupresores, antiagregantes plaquetarios y vasodilatadores
Subject(s)
Humans , Female , Adult , Antiphospholipid Syndrome/surgery , Antiphospholipid Syndrome/classification , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/diagnosis , Antiphospholipid Syndrome/epidemiology , Antiphospholipid Syndrome/etiology , Antiphospholipid Syndrome/physiopathology , Antiphospholipid Syndrome/pathology , Antiphospholipid Syndrome/drug therapy , Antiphospholipid Syndrome/therapy , Vasculitis/classification , Vasculitis/complications , Vasculitis/diagnosis , Vasculitis/drug therapy , Vasculitis/epidemiology , Vasculitis/etiology , Vasculitis/pathology , Vasculitis/physiopathology , Vasculitis/surgery , Vasculitis/therapyABSTRACT
Objetivo: conocer el rendimiento diagnostico de varios marcadores bioquimicos utilizados para conocer la actividad de vasculitis, en la diferenciacion entre vasculitis activa y vasculopatias no inflamatorias (VNI), con evento trombotico isquemico agudo. Metodo: se estudiaron 79 sueros (57 pacientes con vasculitis activa y 22 con VNI), para determinar el factor reumatoideo (FR), la proteina C reactiva (PCR), los anticuerpos anticitoplasmaticos del neutrofilo clasicos (cANCA), la molecula-1 de adhesion intercelular (ICAM-1), la molecula-1 de adhesion celular vascular (VCAM-1), selectinas E, P y L,interleuquina (IL)-2, IL-4, IL-10 y el interferon gamma (INF-Y). Se realiza el analisis de rendimiento diagnostico de vasculitis frente a VNI de cada una de las pruebas, basados en el estudio de razones de probabilidad positivas ("likelihood ratio positivo-LR+"). Resultados: los 57 casos de vasculitis, 19 hombres (33.33 porciento) y 38 mujeres (66.66 porciento), cumplieron criterios del Colegio Americano de Reumatologia (ACR), para la clasificacion de las vasculitis, y de criterios de actividad segun el puntaje de actividad de vasculitis de Birmingham (Birmingham Vasculitis Activity Score, BVAS). La edad promedio fue de 35,5 años con una desviación estandar de 10.9 años. El factor reumatoideo fue positivo en 13 pacientes con vasculitis (22.8 porciento), y en ningun caso de VIN (p= 0.015). La PCR fue positiva en 34 pacientes con vasculitis (59,6 porciento) y en 9 casos de VNI (41 porciento) (chi cuadrado de 2.25; p=0.8111). Los LR(+) de las otras pruebas para puntos de corte de sus concentraciones al nivel percentil 75: para ICAM-0.87,IL-4:1,08,IL-10:0,94 e INF-g:1,08. Conclusion: la ICAM-1 fue la mejor prueba para diferenciar vasculitis activa de VNI con evento isquemicotrombotico agudo
Subject(s)
Humans , Male , Female , Biomarkers/analysis , Biomarkers/chemistry , Vasculitis/diagnosis , Vasculitis/classification , Vasculitis/physiopathologyABSTRACT
La determinación de anticuerpos contra neutrófilos (ANCA) ha sido de gran utilidad en la comprensión de diversas vasculitis sistémicas principalmente granulomatosis de Wegener (GW), poliangeítis microscópica (PM), síndrome de Churg-Strauss, glomerulonefritis idiopática necrotizante progresiva (GNN) y otras. Su determinación se realiza por inmunofluorescencia o ELISA. Hay dos patrones de tinción inmunofluorescencia: citoplásmico (c-ANCA) y perinuclear (p-ANCA); el primero se asocia a GW y el segundo a PM y GNN. Estos anticuerpos pueden tener un papel importante en la patogenia de esta enfermedades, asociados a la acción de citocina e interacción con los polimorfonucleares que resulta en daño al endotelio vascular. Se han asociado a recaídas o exacerbaciones del padecimiento, lo que les confiere importancia como marcadores de actividad de la enfermedad y pueden ser útiles para modificar el tratamiento. Se reconoce su importancia en el diagnóstico diferencial de la hemorragia pulmonar masiva y de la glomerulonefritis rápidamente progresiva. Otras enfermedades en las que se pueden encontrar ANCA positivos son: síndrome de Goodpasture, nefropatía lúpica, enfermedad inflamatoria intestinal y hepatopatías autoinmunes. Aunque el tratamiento es variable, éste depende de la gravedad de la enfermedad y la afección renal; los inmunosupresores (esteroides y citotóxicos) son los más frecuentemente empleados. Se ha informado la potencial eficacia de la globulina antitimocito y de los anticuerpos monoclonales, pero no existe actualmente un consenso para hablar de la terapia idónea
Subject(s)
Vasculitis/classification , Vasculitis/physiopathology , Vasculitis/drug therapy , Enzyme-Linked Immunosorbent Assay , Granulomatosis with Polyangiitis/diagnosis , Granulomatosis with Polyangiitis/physiopathology , Granulomatosis with Polyangiitis/drug therapy , Antibodies, Antineutrophil Cytoplasmic , Biomarkers , Churg-Strauss Syndrome/physiopathologyABSTRACT
This is a case report a of hepatitis C-positive 43-year-old male who presented with small vessel involvement resulting in infarction of the testis, kidney and fingers in addition to multiple and painful aneurysms of arteries. The diagnosis, features and management of the patient are discussed briefly