[Cerebral vasculitis]. / Vasculitis cerebrales.

INTRODUCTION: Cerebral vasculitis embraces a wide range of conditions that are characterised by involvement of the vessels of the central nervous system (CNS) due to inflammation of their walls, which in turn leads to occlusion or the formation of aneurysms with the ensuing ischaemic-haemorrhagic disorders this produces. DEVELOPMENT: In cases of cerebral vasculitis perhaps only the vessels of the CNS (isolated angiitis of the CNS) are involved, or they may be just another of the affected territories to be found in primary or secondary systemic angiitis (due to infection, collagen diseases, drugs, tumours). Neurological symptoms and lab tests are usually rather unspecific. The latest neuroimaging techniques are more useful, and the gold standard among them is digital angiography, although its sensitivity and specificity are limited. Brain tissue biopsy allows for confirmation of the diagnosis and is the gold standard for the diagnosis of isolated angiitis of CNS. A large group of conditions (which may be metabolic, demyelinating, vascular, infectious, and other peripheral vascular diseases) have similar clinical and imaging features, which makes it necessary to consider the differential diagnosis. CONCLUSIONS: Involvement of the nervous system casts a shadow over the prognosis in most cases of vasculitis and can be severe, as in isolated vasculitis of large vessels or in Takayasu's disease, or more benign, as in isolated vasculitis of small vessels and in other primary vasculitis. Treatment with corticoids and immunosuppressant agents, as well as anticoagulant and/or antiaggregating therapy, must be considered in each particular case according to the clinical condition and the progression of each patient.

Vasculitis cerebrales / Cerebral vasculitis

Introducción. Las vasculitis cerebrales comprenden un amplio grupo de entidades caracterizadas por la afectación de los vasos del sistema nervioso central (SNC) ocasionado por la inflamación de su pared, que conduce a la oclusión o a la formación de aneurismas, con las consiguientes alteraciones isquémicas-hemorrágicas. Desarrollo. En las vasculitis cerebrales pueden afectarse exclusivamente los vasos del SNC (vasculitis aislada del SNC), o ser otro de los territorios afectados en las vasculitis sistémicas primarias o secundarias (infecciosas, enfermedades del colágeno, por drogas, tumorales). Los síntomas neurológicos y el laboratorio suelen ser poco específicos. De mayor utilidad son las nuevas técnicas de neuroimágenes, y la angiografía digital es el patrón oro dentro de ellas; sin embargo, su sensibilidad y especificidad es limitada. La biopsia cerebral permite confirmar el diagnóstico y esel patrón oro para el diagnóstico de las vasculitis aisladas del SNC. Un amplio grupo de entidades (metabólicas, desmielinizantes, vasculares, infecciosas y otras vasculopatías) tienen manifestaciones clínicas e imaginológicas similares, por lo que se necesita plantear el diagnóstico diferencial. Conclusiones. La afectación del sistema nervioso ensombrece el pronóstico en la mayoría de las vasculitis y puede ser grave, como en las vasculitis aisladas de grandes vasos o en la enfermedad de Takayasu, o más benigno, como en la vasculitis aislada de pequeños vasos y en otras vasculitis primarias. El tratamiento con corticoides e inmusupresores debe considerarse encada caso particular según la entidad clínica y la evolución de cada paciente, así como el tratamiento con anticoagulantes y/o antiagregantes (AU)

Introduction. Cerebral vasculitis embraces a wide range of conditions that are characterised by involvement of the vessels of the central nervous system (CNS) due to inflammation of their walls, which in turn leads to occlusion or the formation of aneurysms with the ensuing ischaemic-haemorrhagic disorders this produces. Development. In cases of cerebralvasculitis perhaps only the vessels of the CNS (isolated angiitis of the CNS) are involved, or they may be just another of the affected territories to be found in primary or secondary systemic angiitis (due to infection, collagen diseases, drugs, tumours).Neurological symptoms and lab tests are usually rather unspecific. The latest neuroimaging techniques are more useful, and the gold standard among them is digital angiography, although its sensitivity and specificity are limited. Brain tissue biopsy allows for confirmation of the diagnosis and is the gold standard for the diagnosis of isolated angiitis of CNS. A large group of conditions (which may be metabolic, demyelinating, vascular, infectious, and other peripheral vascular diseases) have similar clinical and imaging features, which makes it necessary to consider the differential diagnosis. Conclusions. Involvement of the nervous system casts a shadow over the prognosis in most cases of vasculitis and can be severe, as in isolated vasculitis of large vessels or in Takayasu's disease, or more benign, as in isolated vasculitis of small vessels and in other primary vasculitis. Treatment with corticoids and immunosuppressant agents, as well as anticoagulant and/or antiaggregating therapy, must be considered in each particular case according to the clinical condition and the progression of each patient (AU)

Use of infliximab in patients with systemic juvenile idiopathic arthritis refractory to etanercept.

OBJECTIVE: To analyse the effectiveness and safety of Infliximab in a group of patients with systemic juvenile idiopathic arthritis (SJIA) who had failed treatment with etanercept in a single paediatric rheumatology clinic. METHODS: Patients with SJIA with active polyarthritis refractory to methotrexate (MTX) [> or = 20 mg/m2/week] for at least 3 months and to etanercept (up to 1 mg/kg twice weekly) for at least 6 months were included. All children received infliximab 3-10 mg per kg of body weight intravenously concomitantly with MTX 7.5-10 mg/week for 19 (2-113) weeks. Evaluation included ACR paediatric 30 criteria and presence of signs of systemic activity (fever, rash). RESULTS: Six patients were included. Three patients met ACR paediatric 30 criteria at 2 weeks (2 patients) and 10 weeks after initiation of infliximab. Improvement lasted for 4, 12, and 84 weeks respectively. The presence of fever/rash was not modified by the treatment. Infliximab was discontinued due to moderate side effects in 4 patients. No serious side effects were observed. CONCLUSIONS: Most patients with SJIA who fail to respond to etanercept may not reach sustained improvement when switched to infliximab. The only patient in our group who improved sustainedly with infliximab did not show any systemic features at the beginning of therapy. Further controlled studies are needed in order to assess efficacy of infliximab in children with refractory SJIA.

Etanercept in systemic juvenile idiopathic arthritis.

OBJECTIVE: To evaluate the effectiveness of etanercept in patients with systemic juvenile idiopathic arthritis (SJIA) refractory to methotrexate (MTX) therapy in a pediatric rheumatology practice. METHODS: Fifteen patients with SJIA with active polyarthritis refractory to higher dose MTX (> or = 20 mg/m2/week) for at least 3 months were included. Patients received etanercept 0.4 mg/Kg twice weekly concomitantly with MTX. Observed period of treatment ranged from 5 to 12 months (median 9 months). RESULTS: Improvement of ESR, swollen and limited joint counts, functional capacity, and general wellbeing was achieved by 14/15 patients. The most significant impact on these variables was observed 3 to 5 months after treatment onset. Mean time to improvement was 2 months. In the 4 patients who presented fever and rash, these signs disappeared after the beginning of etanercept treatment and reappeared during flares. Three patients showed sustained clinical and biochemical remission on low dose MTX (< or = 5 mg/m2/week). Thirteen relapses were observed in 9 (60%) patients at a mean of 7.6 months after therapy was begun. Etanercept was discontinued due to lack of efficacy in 7 patients, only after higher dose (1 mg/kg/dose) was used. MTX and corticosteroid doses were decreased during the observation period. No serious side effects were observed. CONCLUSIONS: Etanercept, in combination with MTX, demonstrated benefit soon after initiation of treatment in patients with refractory SJIA, but flares and progressive loss of effectiveness were observed with continued treatment in most patients. Sharp decreases in the dose of MTX and corticosteroids may have contributed to subsequent occurrence of flares. Changes in MTX and corticosteroids doses should probably need to be made gradually, and it is possible that patients on SJIA should continue on therapeutic doses of MTX while being on etanercept in order to maintain therapeutic benefit.

Juvenile rheumatoid arthritis-like polyarthritis in Nijmegen breakage syndrome.

Nijmegen breakage syndrome (NBS) is a rare autosomal recessive disease (8q21) from the family of the genetically determined chromosomal instability syndromes. The disorder is characterized by microcephaly, growth retardation, immunodeficiency, and high incidence of cancer. Several noninflammatory anomalies of the musculoskeletal system have been described in patients with this syndrome. We describe an Argentinian girl with all the clinical, immunological, and cytogenic characteristics described for NBS plus a juvenile rheumatoid arthritis-like syndrome. To our knowledge this is the first report of a patient with the NBS who presented with a symmetric chronic polyarthritis resembling JRA.

Chronic infantile neurological cutaneous and articular syndrome: two new cases with rare manifestations.

UNLABELLED: CINCA/IOMID is a systemic inflammatory disorder of unknown aetiology that resembles congenital infection and systemic juvenile chronic arthritis (JCA). This disorder is characterized by neonatal onset, persistent rash, ocular inflammatory lesions, and progressive articular and neurological involvement. We report two new patients with this syndrome. Both children presented periodic bouts of cutaneous rash, fever, organomegaly, articular involvement with typical radiological features, and developmental delay. One of the patients presented neonatal jaundice and elevation of liver enzymes; inflammatory infiltrates were observed in the liver biopsy. The other patient showed retinal vasculitis detected at age 18 mo on fundoscopy and fluorescent angiography. Therapy with azathioprine was associated with prolonged remission of this complication. In both cases, the disease was diagnosed after some delay. CONCLUSION: Early hepatitis and retinal vasculitis are rare features of CINCA/IOMID that may help differentiate this syndrome from JRA. Azathioprine may have induced the remission of vasculitis in one case.

Cholestasis in juvenile dermatomyositis: report of three cases.

This report describes 3 cases of juvenile dermatomyositis (juvenile DM) complicated by cholestasis. All 3 patients had typical features of juvenile DM, and all developed a cholestatic syndrome within the initial months of their disease. Liver biopsy revealed mixed (cytoplasmic and ductal) cholestasis with no abnormalities in the intrahepatic ducts in all 3 cases. Cholestasis improved or was completely reversible upon treatment with prednisone. In the 2 patients who could be followed up long term, no sequelae remained. The possible role of inflammation in the pathogenesis of cholestasis in juvenile DM is discussed.

Chronic infantile neurological cutaneous and articular/neonatal onset multisystem inflammatory disease syndrome: ocular manifestations in a recently recognized chronic inflammatory disease of childhood.

OBJECTIVE: To report on the ocular manifestations of the Chronic Infantile Neurological Cutaneous and Articular/Neonatal Onset Multisystem Inflammatory Disease (CINCA/NOMID) syndrome, a rare, recently identified, pediatric multisystem inflammatory disease with chronic cutaneous, neurological, and articular manifestations. DESIGN: Descriptive case-report study. SETTING: International collaborative study based on a questionnaire. RESULTS: We included 31 patients. The mean age at onset of eye manifestations was 4.5 years. Optic disc changes were the most common feature, occurring in 26 patients (83%), including optic disc edema, pseudopapilledema, and optic atrophy. Anterior segment manifestations varying from mild to severe were seen in 13 patients (42%); chronic anterior uveitis, in 17 patients (55%). Moderate to severe visual acuity loss in at least 1 eye was seen in 8 patients (26%) as a consequence of the disease. Posterior synechia, glaucoma, and white iritis were not observed in any patient. CONCLUSION: Ocular manifestations with potentially sight-threatening complications occur commonly in the CINCA/NOMID syndrome. The distinctive nature of these complications may assist the ophthalmologist in recognizing this rare disorder and distinguishing it from juvenile rheumatoid arthritis.

Influence of hepatitis G virus coinfection on the clinical course of chronic hepatitis C.

Hepatitis G virus (HGV) is a parenterally transmitted virus, frequently associated with hepatitis C virus infection. Hepatitis G virus RNA was detected by reverse transcription-polymerase chain reaction in the serum of 40 patients with chronic hepatitis C. Nine (22.5%) patients had evidence of hepatitis G virus viraemia. No significant epidemiological or virological differences could be demonstrated between subjects infected with both hepatitis G virus and hepatitis C virus and subjects infected with hepatitis C virus alone. Aminotransferase values were comparable between the two groups, whereas higher levels of cholestatic enzymes (P< 0.001) were reported in the hepatitis G virus/hepatitis C virus-positive patients. A liver biopsy was performed on all 40 patients no later than 6 months before recruitment. The mean histological activity index did not differ between hepatitis G virus-positive and hepatitis G virus-negative patients, whereas specific histological features such as macrovesicular steatosis, portal granulomas, and bile duct damage were more commonly observed among the coinfected patients. The results indicate that coinfection with hepatitis G virus probably does not have a significant effect on hepatitis C virus-induced hepatic damage.