Behçet’s Disease: A Case Series of 5 Patients in the Department of Dermatology, Hospital Kuala Lumpur, Malaysia

Behcet’s disease (BD) is a variant of systemic vasculitides characterized by recurrent oral aphthous ulcers, recurrent genital ulcers with eyes, cutaneous, gastrointestinal, joints, neurological and others organ involvement. Here we aim to describe the demography, clinical patterns and the treatment of 5 cases of BD presented to the Department of Dermatology Hospital Kuala Lumpur between 2002 and 2016. All the patients had a delay in their diagnosis. The clinical characteristics and the choices of treatment in our patients did not differ greatly compared to the reports from other countries. BD could be under-diagnosed in Malaysia as the presenting symptoms are non-specific. Therefore, a high index of suspicion is needed.

CT Features of Vasculitides Based on the 2012 International Chapel Hill Consensus Conference Revised Classification

Vasculitis, characterized by inflammation of vessel walls, is comprised of heterogeneous clinicopathological entities, and thus poses a diagnostic challenge. The most widely used approach for classifying vasculitides is based on the International Chapel Hill Consensus Conference (CHCC) nomenclature system. Based on the recently revised CHCC 2012, we propose computed tomography (CT) features of vasculitides and a differential diagnosis based on location and morphological characteristics. Finally, vasculitis mimics should be differentiated, because erroneous application of immunosuppressive drugs on vasculitis mimics may be ineffective, even deteriorating. This article presents the utility of CT in the diagnosis and differential diagnosis of vasculitides.

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.

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.

Trends of Childhood Vasculitides in Eastern India.

A prospective follow up for 7 years (2004-2010) revealed 10.2% children (n=158) had vasculitis among all rheumatological cases (n=1544). Henoch-Schonlein Purpura (HSP) (56.9%) and Kawasaki disease (KD) (24%) were major groups.

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.