Vasculitis por reacción de hipersensibilidad tipo III / Vasculitis for type III hypersensitivity reaction

Los procesos inmunitarios son utilizados por el organismo para defenderse de la agresión de agentes infecciosos; no obstante, en ciertos casos, el organismo reacciona de forma inapropiada o excesiva ocasionando diversos tipos de daño tisular. Estas situaciones, que conocemos como hipersensibilidad, pueden tener aspectos positivos o negativos al poder causar ellos mismos la enfermedad. Se presenta el caso de una niña de 14 años de edad, que acude al Hospital Pediátrico Docente William Soler después de varios ingresos en otros centros de salud, donde se planteó el diagnóstico de un pie de madura. Después de varias investigaciones y con el antecedente de alergia a diferentes medicamentos, los cuadros de amigdalitis a repetición, los datos del laboratorio y la clínica que presentaba la paciente, se estableció el diagnóstico de una vasculitis por reacción de hipersensibilidad tipo III. Por las características tan atípicas del cuadro clínico de esta paciente y la dificultad para llegar a un diagnóstico es importante la presentación de este caso(AU)

The immune processes are used by the body to defend against the aggression of infectious agents; however, in certain cases, the body reacts inappropriately or excessively causing various types of tissue damage. These situations, which we know as hypersensitivity, can have positive or negative aspects by being able to cause the disease themselves. We present the case of a 14-year-old girl who attended the William Soler Pediatric Teaching Hospital after several admissions to other health centers, where the diagnosis of a mature foot was raised. After several investigations and with the history of allergy to different drugs, the recurrent tonsillitis symptoms, the laboratory data and the clinic presented by the patient, the diagnosis of a vasculitis due to type III hypersensitivity reaction was established. Because of the atypical characteristics of this patient's clinical picture and the difficulty in reaching a diagnosis, the presentation of this case is important(AU)

Initial digital vasculitis in a large multicenter cohort of childhood-onset systemic lupus erythematosus / Vasculite digital inicial em uma grande coorte multicêntrica de pacientes com lúpus eritematoso sistêmico de início na infância

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.

Evolución de una vasculitis renal asociada a anticuerpos anti-citoplasma de neutrófilos (ANCA) durante un embarazo: caso clínico / Antineutrophil cytoplasmic antibody (ANCA) associated renal vasculitis and pregnancy: report of one case

ANCA mediated vasculitis mainly occur between the fourth and fifth decade of life; therefore, it is very uncommon to see pregnant patients with the disease. Vasculitis may affect significantly the course of pregnancy; in turn pregnancy can change the course of vasculitis. We report a 20 years old woman with ANCA-mediated renal vasculitis lasting 10 years who consulted with a pregnancy of 15 weeks. She was in remission and had amenorrhea attributed to ovarian toxicity due to cyclophosphamide. Pregnancy had an uneventful course with spontaneous delivery at the 37th week, giving birth to a healthy newborn. Proteinuria increased during the course of pregnancy with a mild deterioration of kidney function. During the year after delivery, she had nephrotic proteinuria and a worsening of renal function.

Vasculitis: an unusual manifestation in an HIV-infected patient

Human immunodeficiency virus (HIV) positive patients may develop vasculitis, either mediated by immunological factors or by direct vascular injury. We describe a patient who developed manifestations suggestive of extremities vasculitis with no identifiable risk factors other than HIV, Epstein Barr and Herpes Simplex Virus (HSV) type 1 co-infection. Physicians should be aware that vasculitis may have a heterogeneous presentation and occur associated with HIV infection. Although unusual, this association should be recognized for early proper treatment and prevention of ischemia.

Vasculitis paraneoplásica: presentación de 2 casos clínicos / Paraneoplastic vasculitis: presentation of two clinical case

Las vasculitis constituyen un grupo heterogéneo de enfermedades que se definen por un proceso inflamatorio de la pared vascular, con obstrucción y necrosis distal. Son poco frecuentes y pueden ser primarias o asociadas a infecciones, enfermedades sistémicas o excepcionalmente a neoplasias. La asociación de vasculitis y neoplasia es infrecuente; la vasculitis se presentaantes, de forma concomitante o posteriormente al diagnóstico de la neoplasia. Las más frecuentemente asociadas son las de origen hematooncológico y luego, en menor medida, los tumores sólidos. Las manifestaciones cutáneas por afectación de “pequeño vaso” son las más habituales, y dentro de ellas las leucocitoclásticas. Se presentan 2 casos clínicos excepcionales de vasculitis paraneoplásicas, el primer caso se trata de una paciente de 57 años con lesiones vasculíticas de pequeño vaso asociadas a cáncer broncopulmonar y el segundo caso se trata de una paciente de 79 años con diagnóstico previo de glioblastoma encefálico de alto grado y lesiones en miembros inferiores con características de vasculitis de pequeño vaso.

Vasculitis constitutes a heterogeneous group of diseases that are defined by an inflammatory process of the vascular wall, with occlusion and distal necrosis. They are rare and may be primary or secondary infections, systemic disease or exceptionally to neoplasic disease. The association of vasculitis and neoplasia is uncommon; vasculitis occurs before, concomitantly or subsequently to the diagnosis of neoplasia. The most frequently associated are the hemato-oncology and then to a lesser extent the solid tumors. Cutaneous manifestations by affectation of “small vessel” are the most common. We present 2 exceptional clinical cases of vasculitis associated with cancer, the first case is about a 57 years old patient with lesions of small vessel associated with bronchopulmonary cancer and the second case is a 79 years old patient diagnosed with brain glioblastoma of high-grade and injuries in lower limbs with characteristics of small vessel vasculitis.

Pro-calcitonin and inflammation in chronic hemodialysis / Pro-calcitonina e inflamación en hemodiálisis crónica

Procalcitonin (PCT) has emerged as a marker of infection, a frequent complication in hemodialysis (HD). We analyzed PCT levels in chronic non-acutely infected HD subjects, assessed its correlation with inflammatory and nutritional markers and propose a PCT reference value for non-infected HD patients.In an observational cross-sectional study, 48 chronic HD patients and 36 controls were analyzed. Variables: age, gender, time on HD; diabetes; vascular access, PCT, C-reactive protein (CRP), albumin, malnutrition inflammatory score (MIS), hematocrit, leukocyte count, and body mass index (BMI). Subsequently, control (G1, n = 36, 43%) vs. non-infected patients (G2, n = 48, 57%) groups were compared. In control subjects (G1), age: 54.3 ± 13.7 years, range (r): 30-81; males: 19 (53%); median PCT 0.034 ng/ml (r: 0.02-0.08); median CRP 0.80 mg/dl (r: 0.36-3.9); p95 PCT level: 0.063 ng/ml. In G2, age: 60.2 ± 15.2 years; males 32 (67%), time on HD: 27.0 ± 24.4; diabetics: 19 (32%); median PCT: 0.26 ng/ml (r: 0.09-0.82); CRP: 1.1 mg/dl (r: 0.5-6.2); p95 PCT level: 0.8 ng/ml. In control subjects, PCT and CRP were significantly lower than in G2: PCT: 0.034 vs. 0.26 ng/ml, p = 0.0001; CRP: 0.8 vs. 1.1 mg/dl, p = 0.0004. PCT-CRP correlation in G2: ρ = 0.287, p = 0.048. PCT and CRP concentrations are elevated in chronic non-acutely infected HD subjects, independently of infection, diabetes and vascular access. A p95 PCT level of 0.8 ng/ml may be considered as the upper normal reference value in non-acutely infected HD subjects. The PCT cut-off level in HD is yet to be determined in HD.

La procalcitonina (PCT) puede ser un marcador de infección en la hemodiálisis (HD). Analizamos los niveles de PCT en sujetos sin infección aguda en HD crónica, su correlación con marcadores inflamatorios y nutricionales y, de acuerdo a ello, proponemos niveles de referencia de PCT. En un estudio observacional transversal se estudiaron 48 pacientes en HD y 36 controles. Variables: edad; sexo, tiempo en HD; diabetes; acceso vascular, PCT, proteína C-reactiva (PCR), albúmina, score de malnutrición-inflamación, hematocrito, recuento leucocitario, e índice de masa muscular (IMC). En los controles se determinaron PCT y PCR. Se comparó grupo control (G1, n = 36, 43%) vs. pacientes (G2, n = 48, 57%). G1: edad, 54.3 ± 13.7, rango (r): 30-81 años; hombres: 19 (53%); PCT mediana: 0.034 ng/ml (r: 0.020-0.080); PCR mediana: 0.8 mg/dl (r: 0.36-3.9); el nivel p95 de PCT: 0.063 ng/ml. En el G2, edad media 60.2 ± 15.2 años, hombres: 32 (66%), tiempo en HD: 27.0 2 4.4; diabéticos: 19 (32%); PCT: 0.26 ng/ml (r: 0.09-0.82); PCR: 1.1 mg/dl (r: 0.5-6.2); p95 PCT: 0.8 ng/ml. En G1 los niveles de PCT y PCR fueron significativamente más bajos que en G2: PCT: 0.034 vs. 0.26 ng/ml, p = 0.0001; PCR: 0.8 vs 1.1 mg/dl, p = 0.0004. Correlación PCT- PCR en G2: ρ = 0.287, p = 0.048. La PCT y la PCR están elevadas en HD crónica independientemente de infección, diabetes y acceso vascular. Se propone p95 de PCT de 0.8 ng/ml como límite superior del intervalo de referencia en sujetos sin infección aguda en HD. El valor de PCT en HD está por determinarse.

Vasculite testicular: uma manifestação rara de artrite reumatoide / Testicular vasculitis: a rare manifestation of rheumatoid arthritis

A vasculite testicular é uma manifestação extra-articular muito rara da artrite reumatoide (AR). Descrevemos o caso de um homem de 53 anos com diagnóstico de AR por oito anos, sem controle adequado da doença. O paciente desenvolveu vasculite reumatoide, manifestada por úlceras de membros inferiores e neuropatia periférica. Apresentou ainda meningite neutrofílica aguda, tendo sido tratado com antibióticos e posterior pulsoterapia endovenosa com metilprednisolona (500 mg/dia) por três dias, seguida de ciclofosfamida (2 mg/kg/ dia) e prednisona orais. O paciente apresentou melhora do quadro, mas 15 dias após a alta hospitalar, houve reativação da meningite bacteriana. O paciente foi reinternado e tratado novamente com antibióticos. Três dias depois da segunda admissão hospitalar, o paciente apresentou dor, aumento de volume do testículo esquerdo e posteriormente gangrena. Foi realizada orquiectomia unilateral e o exame anatomopatológico revelou vasculite linfocítica. O paciente faleceu dois dias após a cirurgia devido a pneumonia aspirativa. Esse caso ilustra a vasculite testicular como uma manifestação rara e grave da vasculite reumatoide.

Testicular vasculitis is a very rare extra-articular manifestation of rheumatoid arthritis (RA). We describe the case of a 53-year-old man diagnosed with RA for eight years, who was poorly controlled and developed rheumatoid vasculitis, which manifested as leg ulcers and peripheral polyneuropathy. The patient also had acute neutrophilic meningitis and was treated with antibiotics and intravenous pulse therapy with methylprednisolone (500 mg daily) for three days, followed by oral cyclophosphamide (2 mg/kg daily) and prednisone. Overall improvement was observed, and the patient was discharged. But 15 days later, the meningitis recurred, and the patient was readmitted and treated again with antibiotics. Three days later, he developed pain and enlargement of his left testicle with gangrene. Unilateral orchiectomy was performed, revealing lymphocytic vasculitis. The patient died two days later due to aspiration pneumonia. This case illustrates a rare and severe manifestation of rheumatoid vasculitis.

Vasculite mesentérica em paciente com lúpus eritematoso sistêmico juvenil / Mesenteric vasculitis in a juvenile systemic lupus erythematosus patient

A vasculite mesentérica lúpica (VML) é uma rara causa de dor abdominal aguda. Há poucos relatos de caso demonstrando VML em adultos e, particularmente, em crianças e adolescentes. No entanto, para o nosso conhecimento, a prevalência dessa grave vasculite em uma população pediátrica com lúpus ainda não foi estudada. Portanto, dados de 28 anos consecutivos foram revisados e incluídos 5.508 pacientes em seguimento no Hospital da Faculdade de Medicina da Univesidade de São Paulo (FMUSP). Identificamos 279 (5,1%) casos que preencheram critérios de classificação diagnóstica do American College of Rheumatology para lúpus eritematoso sistêmico (LES) e um (0,4%) desses apresentou VML. Este paciente recebeu diagnóstico de LES aos 11 anos de idade. Aos 13 anos foi hospitalizado com dor abdominal difusa e aguda, náuseas, vômitos biliosos, distensão e rigidez abdominal, com descompressão brusca positiva. O paciente foi prontamente submetido à laparotomia exploradora, identificando isquemia intestinal segmentar, com edema de parede intestinal e aderências. Foi realizada ressecção parcial de intestino delgado, com lise das aderências e pulsoterapia com metilprednisolona. A análise histopatológica identificou arterite de vasos mesentéricos. Após 13 dias, apresentou recorrência de dor abdominal difusa intensa, sendo novamente submetido à laparotomia exploradora, identificando obstrução em intestino delgado por aderências, com gangrena intestinal. Nova ressecção intestinal foi realizada, além de pulsoterapia com metilprednisolona e infusão de imunoglobulina. Portanto, VML é uma rara e grave manifestação abdominal na população com lúpus pediátrico, e pode ser a única manifestação de atividade da doença. Além disso, este estudo reforça a importância do diagnóstico precoce e do tratamento imediato.

Lupus mesenteric vasculitis (LMV) is a rare cause of acute abdominal pain. Few cases of LMV have been reported in adults, children and adolescents. However, to our knowledge, the prevalence of that severe vasculitis in a pediatric population with lupus is yet to be studied. This study reviewed data from 28 consecutive years and included 5,508 patients being followed up at the hospital of the Faculdade de Medicina of the Univesidade de São Paulo (FMUSP). We identifi ed 279 (5.1%) patients meeting the American College of Rheumatology classifi cation criteria for the diagnosis of systemic lupus erythematosus (SLE), one of whom (0.4%) had LMV. That male patient was diagnosed with SLE at the age of 11 years. At the age of 13 years, he was hospitalized with diffuse and acute abdominal pain, nausea, bilious vomiting, abdominal distension, rebound tenderness, and abdominal muscle guarding. The patient underwent laparotomy immediately, and segmentary intestinal ischemia with intestinal wall edema and adhesions were identifi ed. Partial small bowel resection with lysis of the adhesions was performed, as were pulses of intravenous methylprednisolone. The histopathologic analysis evidenced mesenteric arteritis. After 13 days, the diffuse and intense abdominal pain recurred, and the patient underwent a new laparotomy, during which adhesive small bowel obstruction with intestinal gangrene was identifi ed. New intestinal resection was performed, and the patient received pulses of intravenous methylprednisolone and infusion of immunoglobulin. Thus, LMV is a rare and severe abdominal manifestation of the pediatric population with lupus, and can be the only manifestation of disease activity. In addition, this study stresses the importance of the early diagnosis and immediate treatment.

Sudden natural death due to takayasu’s arteritis.

A young male aged 23 yrs with alleged history of chest pain was brought to the emergency medicine department of St John’s Medical college hospital. On arrival was declared brought dead. There was no previous significant medical history. A Medico-legal autopsy was done which revealed left anterior descending coronary artery lumen to be occluded by grey white material. On Histo-pathological examination of the heart, it was diagnosed as Takayasu’s arteritis. Takayasu arteritis, also known as Pulseless disease, occlusive thromboaortopathy, and Martorella syndrome, It is a Granulomatous inflammation of unknown aetiology affecting medium and large arteries leading to vessel wall thickening and occlusion . Females are more likely to be affected than males. Patients often notice the disease symptoms between 15- 30 years of age. Symptoms range from malaise, fever, night sweats, weight loss, arthalagia, fatigue and can present with absent pulses, limb claudication, blood pressure, discrepancies, Hypertension, retinopathy Ischemia, postural dizziness, seizures, hemi paresis and many more. Sudden death due to Takayasu’s arteritis affecting coronary artery is rarely reported during medico-legal autopsy, hence this case is reported.

Neurosífilis meningovascular con trombosis de la arteria basilar / Meningovascular neurosyphilis with basilar artery thrombosis, a case report and literature review

Se presenta el caso clínico de un paciente de 54 años, negativo para VIH, con enfermedad cerebrovascular por trombosis de la arteria basilar, secundaria a neurosífilis meningovascular. La neurosífilis es el compromiso del sistema nervioso central por Treponema pallidum subespecie pallidum en cualquier estadio de la entidad e incluye las formas asintomáticas y sintomáticas de la infección; sus formas de presentación son diversas y dependen de la localización y la extensión de las lesiones. La recomendación actual es el tratamiento con 4 millones de unidades de penicilina cristalina cada 4 horas por 14 días.

Herein a case is described of a 54-years old patient, HIV negative, with cerebro-vascular disease by basilar artery thrombosis secondary to meningovascular neurosyphilis. Neurosyphilis is the impairment at any stage of the central nervous system by Treponema pallidum subspecies pallidum and includes asymptomatic and symptomatic forms of infection. The presentation can take many forms, depending on the location and extent of tissue damage. The currently recommended treatment is crystalline penicillin, 4 million units every 4 hours for 14 days.

Vasculitis gingival como complicación de sepsis de punto de partida enteral / Vasculitis gingival sepsis complicating enteral starting point

Vasculitis es la inflamación de vasos sanguíneos, con isquemia, necrosis y compromiso sistémico. se describe el manejo multidisciplinario de niña de 4 meses quien ingresó al hospital universitario de Maracaibo con fiebre, diarrea, deshidratación, consciente, con períodos de somnolencia, abdomen distendido, ausencia de ruidos hidroaéreos. Posteriormente presenta convulsión, deterioro del estado general, lesiones eritematosas múltiples y en rodetes gingivales, de color blanco parduzco, necróticas, manifestaciones de shock séptico. Antecedente de ingestión de n-butilbromuro de hioscina. laboratorio: anemia, leucocitosis, proteína c reactiva elevada, cultivos negativos. Biopsia del rodete gingival: vasos sanguíneos con paredes infiltradas por células inflamatoriasmononucleares y polimorfonucleares dispersas y necróticas, destrucción y oclusión del endotelio, confirmando la vasculitis gingival. Recibió antibioticoterapia, soporte hemodinámico, cirugía de zonas necrosadas. egresó a los 15 días con diagnóstico de vasculitis gingival por sepsis de origen enteral. el manejo interdisciplinario médico-odontológico facilita la resolución de situaciones generales con compromiso bucodental

Vasculitis is the inflammation of blood vessels, with ischemia, necrosis and systemic involvement. this paper describes the multidisciplinary management of a 4 month old female infant that was admitted to the hospital universitario de Maracaibo with fever,diarrhea, dehydration, periods of somnolence, distended abdomen, and absence of bowel sounds. later, the patient presented seizures, deterioration of her general condition, multiple erythematous and brownish white, necrotic lesions of the gingival border mucosa, and septic shock manifestations. the patient had a history of ingestion of n-butylbromid hyoscine. laboratory tests: anemia, leukocytosis,elevated c-reactive protein, negative cultures, biopsy of the gingival border: blood vessel walls infiltrated by scattered and necrotic polymorphonuclear and mononuclear inflammatory cells, destruction and occlusion of the endothelium confirming gingival vasculitis. treatment included antibiotics, surgery of necrotic areas and hemodynamic support. she was discharged after 15 days with the diagnosis of gingival vasculitis secondary to enteral sepsis. Medical and dentistry interdisciplinary management facilitates the resolution of generalsituations with oral involvement

Frosted branch angiitis, neuroretinitis as initial ocular manifestation in Behçet disease.

Behçet disease is an idiopathic, multisystem disorder characterized by recurrent episodes of orogenital ulceration and vasculitis of the veins and arteries of all calibers. Ocular involvement may affect the conjunctiva, sclera, uveal tract, vitreous, blood vessels, and retina. Many theories have pointed toward an autoimmune response behind its pathogenesis, which may be triggered by exposure to an infectious agent. Frosted branch angiitis is characterized by vascular inflammation, sheathing, retinal edema, and retinal hemorrhages. The disease may be idiopathic in a majority of the cases or may be associated with ocular and systemic pathology. Association between Behηet disease, Frosted branch angiitis, and neuroretinitis is not reported in literature. This uncommon combination reflects the varied systemic and ocular manifestations in Behηet disease, especially in patients who are not diagnosed and treated in time. We hereby report a case of bilateral frosted branch angiitis and neuroretinitis in a young male from Middle-east, suffering from Behçet disease.

Lúpus eritematoso sistêmico complicado por vasculite intestinal e pneumatose intestinal / Systemic lupus erythematosus complicated by intestinal vasculitis and pneumatosis intestinalis

As manifestações gastrointestinais no lúpus eritematoso sistêmico (LES) não são incomuns. Frequentemente são encontrados sintomas inespecíficos, como dor abdominal, náuseas, vômitos e diarreia. Por outro lado, a pneumatose intestinal, caracterizada por múltiplos cistos preenchidos por ar na parede intestinal, é uma condição raramente associada ao LES. Descreve-se a seguir o caso de um homem de 20 anos que foi internado por febre, perda ponderal, cefaleia e artrite, cuja investigação mostrou tratar-se de LES. Na evolução, apresentou quadro abdominal sugestivo de vasculite intestinal, com tomografia computadorizada de abdome revelando sinal do duplo halo ou do alvo e pneumatose intestinal. Realizado tratamento conservador com antibioticoterapia endovenosa, repouso intestinal e nutrição parenteral total, com resolução do quadro abdominal.

Gastrointestinal manifestations in systemic lupus erythematosus (SLE) are not uncommon. Non specific symptoms are often observed, such as abdominal pain, nausea, vomiting and diarrhea. On the other hand, pneumatosis cystoides intestinalis, which is characterized by multiple gas-filled cysts located throughout the intestinal wall, is a rare condition in SLE. We describe a case of a 20-year-old man who was admitted with fever, weight loss, headache and arthralgia and had a diagnosis of systemic lupus erythematosus. During his hospital stay, he developed abdominal symptoms that suggested intestinal vasculitis. The computed tomography of the abdomen showed the double halo sign, or target sign and pneumatosis cystoides intestinalis. The patient presented complete recovery after conservative treatment, with intestinal rest and total parenteral nutrition.

Vasculites dos médios e grandes vasos: [revisão] / Medium and large vessel vasculitis: [revision]

As vasculites são constituídas por um grande grupo de síndromes caracterizadas por inflamação e necrose da parede dos vasos sangüíneos, resultando em estreitamento ou oclusão do lúmen. A distribuição dos vasos envolvidos varia consideravelmente e serve de base para a classificação das síndromes vasculíticas: grandes vasos (arterite de Takayasu, arterite temporal); pequenos e médios vasos (poliarterite nodosa, síndrome de Churg-Strauss, granulomatose de Wegener, vasculites nas doenças reumáticas); e pequenos vasos (vasculite de hipersensibilidade, púrpura de Henoch-Schonlein, poliangeíte microscópica, crioglobulinemia). Este artigo revisa as vasculites dos médios e grandes vasos e opções terapêuticas atuais; além de oferecer orientação sobre abordagem diagnóstica e tratamento dos pacientes com suspeita de vasculite cutânea.

Vasculitis comprises a broad group of syndromes characterized by inflammation and necrosis on thewalls of blood vessels, resulting in narrowing or occlusion of the lumen. The distribution of involved blood vesselvaries considerably and serves as the basis for one classification of vasculitic syndromes: large vessels (Takayasu arteritis, giant cell arteritis); medium and small muscular arteries (polyarteritis nodosa, Churg-Strausssyndrome, Wegener's granulomatosis, vasculitis in rheumatic diseases); and small vessels ("hypersensitivity" vasculitis, Henoch-Schonlein purpura, microscopic polyangiitis, cryoglobulinemia). This article reviews medium and large vessel vasculitis and current treatment options; it also presents a comprehensive approach to diagnosing and treating patients with suspected cutaneous vasculitis.