An update of neurological manifestations of vasculitides and connective tissue diseases: a literature review / Atualização das manifestações neurológicas das vasculitides e das doenças do tecido conjuntivo: revisão de literatura

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.

Fiebre de origen desconocido. Forma de la poliarteritis nodosa con hepatitis crónica a virus B / Fever of unknown origin. Form of polyarteritis nodosa related to chronic hepatitis B virus

Se expuso el caso de una paciente de 60 años que presentó un cuadro de fiebre de origen desconocido y el antecedente de haber sido operada de fractura del olécranon, recientemente, lo que hizo sospechar la posibilidad de fiebre inducida por cuerpo extraño, lo cual se descartó por persistencia del cuadro febril, a pesar de la retirada del material de osteosíntesis metálica. En el análisis de hemoquímica se encontraron niveles ligeramente elevados de aminotransferasas; se confirmó la existencia de hepatitis crónica por virus B mediante la laparoscopia, biopsia hepática, antigenemia de superficie y prueba de la reacción en cadena de la polimerasa (PCR) de virus de hepatitis B (VHB). Por la confirmación de antígeno de superficie positivo, a la presencia de lesiones vasculíticas y a la aparición de una mononeuropatía múltiple se tuvo la sospecha diagnóstica de PAN, la cual se confirmó mediante biopsia del nervio sural. Se destacó la evolución satisfactoria con tratamiento antiviral de interferón y régimen inmunosupresor: prednisona y ciclofosfamida oral. Se concluyó que la PAN-VHB es una asociación frecuente que requiere de tratamiento combinado para mejorar pronóstico y se enfatizó en la necesidad de la búsqueda diagnóstica de la entidad ante todo paciente con FOD, sobre todo si tienen asociado antigenemia B positiva , vasculitis y mononeuropatía.

The case of 60-year-old female patient with a picture of unknown fever that had been recently operated on of olecranon fracture that made suspect the possibility of fever induced by a foreign body is reported. This possibility was discarded due to the persistance of fever after the removal of the material of metallic osteosynthesis. In the hemochemical analysis, slightly elevated levels of aminotranspherases were found. The existance of chronic hepatitis B virus was confirmed by laparoscopy, liver biopsy, surface antigenemia and polymerase chain reaction (PCR) of hepatitis B virus (HBV). Due to the confirmation of the positive surface antigen, to the presence of vasculitic lesions, and to the appearance of a multiple mononeuropathy, there was a diagnostic suspicion of PAN, which was corroborated by biopsy of the sural nerve. It was stressed the satisfactory evolution with antiviral interferon treatment and immunosuppressive regime: oral prednisone and cyclophosphamide. It was concluded that PAN-HBV is a frequent association that requires combined treatment to improve the prognosis. Emphasis was made on the need of the diagnostic search of the entity in patients with FUO, mainly if there is an association with positive hepatitis B antigenemia , vasculitis and mononeuropathy.

Hemobilia from Ruptured Hepatic Artery Aneurysm in Polyarteritis Nodosa

Hemobilia, in patients with the diagnosis of polyarteritis nodosa, is rare at clinical presentation and has a grave prognosis. We describe a case of massive hemobilia, due to aneurysmal rupture, in a patient with polyarteritis nodosa. A 39-year-old man was admitted to the hospital with upper abdominal pain. The patient had a history of partial small bowel resection, for intestinal infarction, about 5 years prior to this presentation. Abdominal computed tomography demonstrated multiple high attenuation areas in the bile duct and gallbladder. Hemobilia with blood seepage was visualized on endoscopic retrograde cholangiopancreatography; this bleeding stopped spontaneously. The following day, the patient developed a massive gastrointestinal bleed with resultant hypovolemic shock. Emergent hepatic angiogram revealed multiple microaneurysms; a communication was identified between a branch of the left hepatic artery and the bile duct. Hepatic arterial embolization was successfully performed. The underlying disease, polyarteritis nodosa, was managed with prednisolone and cyclophosphamide.

Manifestações neurológicas da poliarterite nodosa: aspectos etiológicos, imunopatogênicos, clínicos e terapêuticos / Neurologic symptoms of Polyarteritis nodosa: etiology, immunopathology and therapeutic

A poliarterite nodosa (PAN) é uma vasculite necrotizante sistêmica que afeta artérias de pequeno e médio calibre e que tem grande variedade de formas clínicas, dependendo do território vascular comprometido, gravidade e distribuição das lesões dos vasos. O processo imunopatogênico determinante é o de lesão induzida por imunocomplexo. O diagnóstico correto advém da integração dos achados clínicos, laboratoriais e de imagem. A terapêutica baseia-se na imunoexpressão com o uso de corticoesteróides e agentes citotóxicos

Poliarteritis nodosa / Polyarteritis nodosa

El sindrome de poliarteritis nodosa (PAN) durante décadas se consideró una enfermedad única. Recientemente se ha subdividido, esta entidad, en PAN clásica y PAN microscópica, en base a criterios clínicos, serológicos, arteriográficos y anatomopatológicos. La PAN puede presentarse como una forma cutánea o sistémica, teniendo por lo general, la forma cutánea una evolución benigna con ausencia de lesiones viscerales

Poliarteritis nodosa

Se estudiaron 9 pacientes portadores de Poliarteritis Nodosa diagnosticada durante un período de 9 años en el Hospital del Salvador de Santiago. Los síntomas de presentación fueron inespecíficos en la mayoría e inclcuyeron compromiso del estado general, fiebre, baja de peso y nódulos cutáneos. También hubo compromiso renal y ocular. El tiempo promedio entre el inicio de la enfermedad y el diagnóstico fue de 9.8 meses. El diagnóstico tardío de la enfermedad en esta serie parece estar relacionado con la inespecificidad de los síntomas clínicos. La mortalidad fue 22% que es similar a la descrita en la literatura