Vasculitides in HIV Infection.

PURPOSE OF REVIEW: To review the spectrum of vasculitides in HIV-infected patients and to identify the clinical features that characterize vasculitis in sero-positive HIV. RECENT FINDINGS: Epidemiological studies conducted in the post-HAART era described the rarity of vasculitis in the setting of HIV-infected patients. A study identified histopathological features such as leukocytoclastic vasculitis of the vasa vasorum and adventitial inflammation in the large artery pathology of HIV-positive patients compared with HIV-negative patients with critical lower limb ischemia. A recent retrospective cohort study reported that HIV-positive patients with LVV developed more vascular complications, responded less to antiretroviral therapy, and had worse outcome than HIV-negative patients with LVV. Vasculitides continue to be a rare disease in patients with HIV. The spectrum of vasculitis ranges from life-threatening conditions to relatively mild skin conditions. Recognizing vasculitis in the setting of HIV-positive patients is important because sometimes it require immunosuppressive treatment.

Childhood- Versus Adult-Onset Primary Vasculitides: Are They Part of the Same Clinical Spectrum?

PURPOSE OF THE REVIEW: Most of the primary vasculitis in children and adults has different clinical manifestations for the same disease, which suggests that they might not be part of the same clinical spectrum and requires a different approach in order to reduce the morbidity and mortality of these patients. In this work, we review the most recent literature and the most important studies that describe and compare adult and children primary vasculitides pathogenesis, clinical presentation, and treatment approach. Accordingly, we discuss recent research involving clinical trials, comparison studies, and pathogeny for these vasculitides. RECENT FINDINGS: Clinical manifestations in the different primary vasculitis change in predominance from adults to children. There is a female sex predominance for the ANCA vasculitides in children compared with adults, but the same treatment works in most cases for both groups. Identifying the diverse clinical spectrum in both adults and children primary vasculitides will reduce the need to extrapolate the diagnostic criteria from one group to another and individualize it, which will allow the clinician to establish a better approach.

O rituximabe como uma opção para pacientes com vasculite sistêmica grave refratária à terapia convencional: relato de sete casos e revisão de literatura / Rituximab as an alternative for patients with severe systemic vasculitis refractory to conventional therapy: report of seven cases and literature review

Resumo O maior entendimento das bases fisiopatológicas e do comportamento das vasculites sistêmicas, aliado ao desenvolvimento de regimes terapêuticos com perfil de segurança e eficácia cada vezes melhores, modificou drasticamente o prognóstico dos pacientes diagnosticados com essas entidades clínicas. Recentemente, o emprego do rituximabe no tratamento de pacientes com vasculites ANCA associadas em ensaios clínicos randomizados se mostrou uma opção importante em casos selecionados, especialmente pacientes refratários ou intolerantes à terapia-padrão com ciclofosfamida e corticosteroides. O presente artigo traz o relato de sete casos de vasculites sistêmicas com tratamento bem-sucedido com rituximabe.

Abstract The greater understanding of pathophysiology and behavior of systemic vasculitis, together with the development of therapeutic regimens with increasingly better safety and efficacy profiles, dramatically changed the prognosis of patients diagnosed with these clinical entities. Recently, the use of rituximab in the treatment of patients with ANCA-associated vasculitis in randomized clinical trials showed an important alternative in selected cases, especially patients refractory or intolerant to standard therapy with cyclophosphamide and corticosteroids. This article presents the report of seven cases of systemic vasculitis successfully treated with rituximab.

Rituximab as an alternative for patients with severe systemic vasculitis refractory to conventional therapy: report of seven cases and literature review.

The greater understanding of pathophysiology and behavior of systemic vasculitis, together with the development of therapeutic regimens with increasingly better safety and efficacy profiles, dramatically changed the prognosis of patients diagnosed with these clinical entities. Recently, the use of rituximab in the treatment of patients with ANCA-associated vasculitis in randomized clinical trials showed an important alternative in selected cases, especially patients refractory or intolerant to standard therapy with cyclophosphamide and corticosteroids. This article presents the report of seven cases of systemic vasculitis successfully treated with rituximab.

Hemorragia alveolar difusa asociada a vasculitis ANCA positiva, manejo con plasmaféresis. reporte de un caso / ANCA vasculitis associated diffuse alveolar hemorrhage treated with plasmapheresis: a case report

Presentamos el caso de una paciente femenina de 43 años con vasculitis tipo ANCA con severo compromiso pulmonar por desarrollo de hemorragia alveolar difusa (HAD) e insuficiencia respiratoria. Debido al rápido deterioro clínico y pobre respuesta a inmunosupresores (ciclofosfamida y metilprednisolona) se le practicó plasmaféresis resultando en rápida mejoría, permitiendo la liberación en la ventilación mecánica y mejoría radiológica. En la actualidad se puede sugerir esta terapia en pacientes con diagnóstico de vasculitis tipo ANCA con compromiso pulmonar severo.

We describe a 43 years old woman who was diagnosed of ANCAassociated vasculitides (AAV) with onset of diffuse alveolarhemorrhage and poor pulmonary function. She developed a clinicaldeterioration in spite of cyclophosphamide and methylprednisolonetreatment, and plasmapheresis was performed. She was successfullytreated with timely plasma exchange and immunosuppressivetreatment. Early plasmapheresis with immunosuppressant therapycan rescue this fatal complication.

Doença de Behçet / Behçet's disease

A síndrome de Behçet é uma desordem multissistêmica inflamatória crônica caracterizada por uma vasculitede vasos de grande (artéria pulmonar), médio (artérias cerebrais, veias superficiais) e pequeno calibre. Possui distribuição geográfica heterogênea, não sendo rara nos países do Mediterrâneo e no Japão, contrapondo-se à ínfima prevalência no norte da Europa e nas Américas. Não tem predileção por sexo, sendo a idade médio de início em torno de 20 a 35 anos; não se conhece a taxa de incidência anual. A etiologia e a patogenia continuam obscuras. Contudo, estão envolvidos fenômenos imunológicos, demonstrados pela presença de anticorpos contra células endoteliais a e mucosa oral. Dentre os possíveis fatores etiológicos ambientais, cita-se o Streptococcus sanguis, HSV-1, proteína de choque térmico bacteriana de 65kd. Parece haver ainda um componente genético, pois em certas populações há marcante relação entre o HLA-B51 e a doença. Possui características histopatológicas de vasculite, sem especificidade, sendo diagnosticada clínicamente, de acordo com os critérios elaborados pelo Grupo Internacional de Estudos para Síndrome de Behçet. Cursa com manifestações mucocutâneas, com típicas exacerbações e recorrências, além de acometimento do SNC, TGI, musculoesquelético e, sobretudo, ocular. Sintomas inespecíficos, como cansaço, perda de peso e febre baixa, também estão presentes. Devem ser considerados no diagnóstico diferencial: doença de Crohn, Stevens-Johnson, Reiter, pênfigos, líquen plano, mielodisplasias e Aids. No que concerne ao tratamento observam-se dúvidas e desafios, seja pelo curso irregular, ora benigno, ora grave, seja pela polissintomatologia, que requer tratamentos distintos, ou pelo fato de que os muitos esquemas terapêuticos utilizados carecem de indicações precisas; pode-se lançar mão de corticoides tópicos e sistêmicos, imunossupressores, talidomida, metotrexato, colchicina, dentre outros.

Behçet’s disease is a chronic multissistemic inflammatory disorder, characterized by a vasculitides of large (pulmonary artery), medium (cerebral arteries, superficial veins) and small-calibre vases. It has a heterogeneous geographic distribution, not being occasional in Mediterranean countries and in Japan, which is the opposite of the undermost prevalence in North Europe and in the Americas. The illness doesn’t present a sex predilection, and the medium starting age is about 20 to 35 years old, the annual incidence rate is not known. The etiology and pathogeny are still obscure; however, immunologic phenomenons are involved, being demonstrated by the presence of antibodies against endothelial cells and oral mucosa; among the possible etiologic factors, can be mentioned the Streptococcus sanguis, HSV-1 and thermic shock bacterial protein of 65kd. The existence of a genetic component is probable, because there is a marking connection in some populations with HLA-B51 and the disease. Is owns histopathologic characteristics of vasculitides without specification, being clinical diagnose according to the criterions developed by International Studying Group for Behçet’s Disease. The syndrome course presents mucocutaneous manifestation, with typical exacerbations and recurrences, beyond central nervous system, gastrointestinal tract, musculoskeletal and above all, ocular compromising. Inespecific symptoms, as tiredness, weight loss and small fever are also present. Must be considered in the differential diagnose: Crohn disease, Stevens-Johnson, Reiter, pemphigus, plane lichen, mielodisplasias and Aids. Concerning the treatment, doubts and challenges are observed, considering the irregular course of the disease, sometimes benign, and sometimes grave; besides the multiple symptoms, which request distinct treatments, or for the fact that the multiple therapeutic schemes used lacks exact indications, being mostly based in personal self opinions and experiences...

Vasulitis primarias en la infancia: estudio clínico-epidemiológio / Primary vasculitis in children: a clinical-epidemiology study

Introducción. La clasificación de las vasculitis primarias es dificultosa y su prevalencia en la edad pediátrica, poco conocida. Objetivo. Determinar la frecuencia, los datos epidemiológicos y las características clínicas de las vasculitis primarias en pacientes pediátricos entre mayo de 2000 y mayo de 2008. Diseño. Estudio observacional, retrospectivo y descriptivo. Materiales y métodos. Revisión de la base de datos de las historias clínicas de vasculitis primarias en pacientes pediátricos de 0 a 16 años que cumplían los criterios de inclusión establecidos. Resultados. Se encontraron 47 pacientes, 29 varones y 18 mujeres. La edad promedio de presentación fue de 4 años (rango: 7 meses a 13 años). La vasculitis más frecuente fue la púrpura de Schõnlein-Henoch, con 33 registros (70%), seguida por la enfermedad de Kawasaki 9 (19%), el edema agudo hemorrágico del lactante 3 (6%), la panarteritis nodosa cutánea 1 (2%) y el síndrome de Churg-Strauss 1 (2%). Conclusión. Las vasculitis primarias son poco frecuentes en la edad pediátrica. La púrpura de Schõnlein-Henoch fue la más frecuente en nuestra población. La enfermedad de Kawasaki fue la vasculitis de mayor morbilidad. El edema agudo hemorrágico del lactante se presentó en niños menores de 2 años, fue menos frecuente y con escasa morbilidad. Pudimos también detectar 2 casos de vasculitis menos frecuentes como la panarteritis nodosa cutánea y el síndrome de Churg-Strauss.

Introduction.The classifi cation of primary vasculitis is diffi cult and the prevalence in childhood is unknown. Objective. To determine the frequency, epidemiological data and the clinical features of the primary vasculitis in pediatric patients seen in our Hospital since May 2000 to may 2008. Design. Observational, retrospective and descriptive study. Materials and methods. We reviewed the data base of medical records of the pediatric patients with primary vasculitis diagnose. We included patients from 0 to 16 years old that met the established inclusion criteria. Results. Forty-seven patients were found, 29 were boys and 18 girls. The average age of onset was 4 years old (range: 7 months to 13 years old). The most frequent vasculitis was the Henoch Schönlein purpura with 33 cases(70%), followed by the Kawasaki disease with 9 patients (19%); the acute hemorrhagic edema of young children with 3 cases (6%) and the cutaneous polyarteritis nodosa and the Churg-Strauss syndrome with 1 case each. Conclusions. Primary vasculitis is not frequent in childhood. The He-noch Schönlein purpura was the most frequent in our patients. The Kawasaki disease was the vasculitis with major morbidity. The acute hemorrhagic edema of infancy was less frequent, showed less morbidity and presented in children younger of two years of age. We had also the opportunity to observed 2 cases of very low-frequency vasculitis such us cutaneous polyarteritis nodosa and Churg-Strauss syndrome.