Placas purpúricas retiformes / Retiform Purpura in Plaques

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Use of mycophenolate in ANCA-associated renal vasculitis: 13 years of experience at a university hospital.

INTRODUCTION: Standard therapy with corticosteroids (CS) and cyclophosphamide (CYC) followed by azathioprine has been shown to improve renal and patient survival in ANCA-associated renal vasculitis (rAAV). Mycophenolate mofetil (MF) has been progressively introduced for the treatment of rAAV in the last years because of its immunosuppressive efficacy combined with a lower toxicity profile. In this study, we retrospectively analyse the results of the introduction of MF for maintenance and induction therapy in rAAV in our institution from 2001 to 2013. RESULTS: We reported 67 patients treated with MF as a maintenance treatment, divided by baseline serum creatinine (>500 µmol/L: Group 1 and <500 µmol/L: Group 2) and treatment schedule. Twenty-nine of the 67 patients were also treated with MF as induction treatment, mostly in Group 2. During the follow-up (2 years after the diagnosis) creatinine levels for serum glomerular filtration rate, ANCA titres, C-reactive protein and percentage of haematuria decreased in all groups. In Group 2, parameters and also relapse rates were similar at 24 months in patients treated with CYC or MF as an induction treatment (Subgroups 2a and 2b, respectively). Median dose of MF in maintenance treatment was 1000 mg daily and prednisone dose was tapered to 10 mg daily from Month 3. After 24 months, 82% of patients remained on MF therapy, 18% had discontinued the treatment, seven of them due to medical indication and two because of gastrointestinal intolerance. The percentage of patients that started renal replacement therapy was irregular in Group 1 depending on the subgroup (25-100%), and 10% in Group 2. Adverse effects, such as neutropenia, infections and neoplasia, were more prevalent in groups treated with CYC. CONCLUSION: In conclusion, in our patients with rAAV, MF demonstrated to be an effective and well-tolerated option for maintenance treatment. As an induction treatment, MF seems to be similar to CYC for patients with moderate renal failure in the diagnosis.

Outcomes of splenectomy in patients with common variable immunodeficiency (CVID): a survey of 45 patients.

Splenectomy has been used in patients with common variable immunodeficiency disorders (CVID), mainly in the context of refractory autoimmune cytopenia and suspected lymphoma, but there are understandable concerns about the potential of compounding an existing immunodeficiency. With increasing use of rituximab as an alternative treatment for refractory autoimmune cytopenia, the role of splenectomy in CVID needs to be re-examined. This retrospective study provides the largest cohesive data set to date describing the outcome of splenectomy in 45 CVID patients in the past 40 years. Splenectomy proved to be an effective long-term treatment in 75% of CVID patients with autoimmune cytopenia, even in some cases when rituximab had failed. Splenectomy does not worsen mortality in CVID and adequate immunoglobulin replacement therapy appears to play a protective role in overwhelming post-splenectomy infections. Future trials comparing the effectiveness and safety of rituximab and splenectomy are needed to provide clearer guidance on the second-line management of autoimmune cytopenia in CVID.

Clinicopathologic features and outcomes of neuro-Behçet disease in Spain: a study of 20 patients.

BACKGROUND: To describe the clinical characteristics and evolution of a series of adult patients hospitalized for neuro-Behçet disease (NBD). METHODS: Consecutive patients admitted for NBD in a teaching hospital were retrospectively selected. Disability at discharge and during follow-up was graded with the modified Rankin Scale, and outcome classified as good or poor (grades 3-6). RESULTS: Twenty patients were included (M/F, 13/7). Mean age at NBD diagnosis was 36.3 years. Nineteen patients had other manifestations of Behçet disease (BD) before NBD developed, but only 7 met the complete diagnostic criteria for BD. Fever, headache, motor weakness, and cranial nerve palsy were each present in approximately 60% of patients. There was a low prevalence of behavioral changes (5%), seizures (5%), and sphincter incontinence (0%), and a relatively high prevalence of meningism (25%). Non-neurologic manifestations of BD were concurrently detected in 15 patients (75%). 80% had parenchymal involvement. Brain biopsies during 5 attacks showed perivascular lymphocytic infiltration with reactive astrocytosis, but no frank vasculitis. During a mean follow-up of 6.3 years per patient, 12 had at least one relapse. In total, there were 22 relapses; all but two were in the same location and were symptomatically similar in each patient. At the end of follow-up, 7 patients (35%) had a poor outcome, including 4 who died. CONCLUSION: Recording of previous manifestations of BD and a physical examination to detect concomitant systemic manifestations of BD may help establish an early diagnosis of NBD. Relapses frequently occurred in the same location. No frank vasculitis was present in brain biopsies.

18F-FDG PET/CT in the evaluation of POEMS syndrome.

In POEMS syndrome the identification and biopsy of an osteosclerotic lesion or a lymph node typical of Castleman's disease (CD) is essential to establish the diagnosis and plan appropriate treatment. We report four patients in whom the localisation and identification of diagnostic bone lesions or lymphadenopathies were guided by fluorodeoxyglucose positron emission tomography integrated with computerised tomography (FDG PET/CT). FDG PET/CT identified bone lesions not detected with other techniques in one patient, and revealed hypermetabolic characteristics in bone lesions or adenopathies in the others, thus guiding the diagnostic biopsy in those with hypermetabolism. In conclusion, FDG PET/CT may be useful in detecting and selecting bone lesions and lymph nodes for biopsy in patients with suspected POEMS syndrome.

Clinical efficacy and safety of Flebogammadif, a new high-purity human intravenous immunoglobulin, in adult patients with chronic idiopathic thrombocytopenic purpura.

Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by a low platelet count and bleeding, whose incidence is approximately 6.2 for each 100,000 adults per year. Intravenous immunoglobulins (IVIG) can be useful in patients with ITP to prevent bleeding or prior to surgery. In this study, the efficacy and safety of Flebogammadif, a new high-purity IVIG, were assessed by an open, multicentre, non-controlled, prospective study in adult patients with chronic ITP. A total of 20 patients (enrolled if experiencing chronic ITP since at least 6 months before recruitment and if platelet count <20 x 10(9)L(-1) before treatment) received 0.4 g kg(-1)-bw of Flebogammadif for 5 consecutive days and were followed-up for 3 months. Efficacy endpoints were three: proportion of patients who reached a platelet count > or = 50 x 10(9)L(-1), time for the platelet count to reach that level and duration of response. Safety parameters [adverse events (AE), laboratory determinations and vital signs] and viral markers were regularly monitored. A total of 14 patients achieved a platelet count of > or = 50 x 10(9)L(-1). The median time to platelet response was or = 50 x 10(9)L(-1) was > or = 7 days. A regression of haemorrhages was reported for 17 patients on day 14. Eight patients presented 21 AEs (mostly mild) potentially related to the study drug. Neither abnormalities in laboratory values nor in viral markers were registered during the follow-up period. Flebogammadif was well tolerated and succeeded in providing a haemostatic platelet count in patients with ITP.

Unidad de diagnóstico rápido en un hospital de tercer nivel. Estudio descriptivo del primer año y medio de funcionamiento / Rapid diagnosis unit in a third level hospital. Descriptive study of the first year and a half

Fundamento y objetivo. Presentamos el estudiodescriptivo de una unidad de diagnóstico rápido(UDR) en régimen ambulatorio como alternativa alingreso hospitalario tradicional.Material y métodos. La UDR ha estado constituidadurante el tiempo que abarca este estudio por unmédico internista, un radiólogo y una enfermera.Para llevar a cabo el estudio estadístico descriptivo,revisamos la historia clínica de 1.132 pacientesvisitados en el primer año y medio.Resultados. Hemos atendido a 1.132 pacientes, el71% procedentes de Urgencias y el 26%, de AtenciónPrimaria. El estudio se llevó a cabo en unos 9 días demediana. Los principales motivos de consulta fueronanemia, síndrome constitucional y adenopatíasperiféricas. El 38% de los pacientes fue dado de alta,el resto requirió seguir el estudio o bien en consultasexternas (52%) o bien bajo ingreso hospitalario (10%).Hasta 199 pacientes (18%) fueron diagnosticados decáncer, los más frecuentes colon, linfoma y pulmón.Estimamos que se han podido evitar 4,5 camas/día enel Servicio de Medicina Interna.Conclusiones. La UDR ha demostrado ser ennuestro centro una alternativa eficiente de lahospitalización en pacientes en estudio de patologíapotencialmente grave, pero en los cuales el estadogeneral permite una atención ambulatoria

Background and aim. We show a descriptive studyof a Rapid Diagnosis Unit as an alternative of thetraditional hospital admission.Material and methods. An Internist, a Radiologistand a Nurse have constituted Rapid Diagnosis Unit.A retrospective and descriptive study of 1132patients visited in the first and a half- year isdescribed.Results. 1132 patients have been attended, 71%coming from Emergency Department and 26% fromPrimary Health Care. Mean stay was 9 days.Anaemia, wasting syndrome and lymph nodesenlargement were the most likely complaints. Theoutcome of these patients was diverse: 38% havebeen discharged, 52% carried on as outpatientsin our hospital and 10% required hospitalization. In199 patients (18%) cancer was diagnosed. Colon,lymphoma and lung cancer were the most commonmalignant disorders involved. About 4,5 beds perday have been saved from the Internal MedicineDepartment.Conclusions. Rapid Diagnosis Unit is an efficientalternative to conventional hospital admissions forpatients in study of potential and serious disordersallowing an out-patient management

Mycophenolate mofetil in anti-MPO renal vasculitis: an alternative therapy in case of cyclophosphamide or azathioprine toxicity.

BACKGROUND: Standard therapy with corticosteroids and cyclophosphamide followed by azathioprine has improved renal and patient survival in renal vasculitis. However, this regimen is associated with high toxicity. Mycophenolate mofetil (MMF), a less toxic immunosuppressive drug, has been proposed as a therapeutic alternative. METHODS: We report 12 patients (4 males, 8 females, aged 65.6 A+/- 12.1 years) with anti-MPO renal vasculitis who were switched from standard therapy to MMF because of drug-related adverse effects: leukopenia, toxic hepatitis, nausea, hair loss or appearance of carcinoma. MMF was introduced at a dose of 500 mg/8 h, after 83 A+/- 56 days under standard therapy. RESULTS: After 354 A+/- 195 days of MMF therapy, all patients maintained clinical remission. Mean values of serum anti-MPO, disease activity markers and serum creatinine decreased when these values were compared from pre-therapy to the time of switching to MMF, and then to the end of the study anti-MPO: 204 A+/- 144 U, 54 A+/- 85 U and 12 A+/- 5 U. Serum-reactive C protein 97 A+/- 82 mg/l, 13 A+/- 10 mg/l and 4 A+/- 2 mg/l. Erythrocyte sedimentation rate 88 A+/- 40, 41 A+/- 28 and 26 A+/- 15 mm. Serum creatinine 415 A+/- 238, 202 A+/- 93 and 169 A+/- 104 micromol/l. In one case there was a relapse of vasculitis under MMF and a low dose of prednisone after 9 months of therapy. Side effects were herpes infection in four cases and chickenpox in one. Neither leukopenia nor anemia was observed. CONCLUSIONS: These results indicate that MMF could be an alternative therapy for anti-MPO renal vasculitis associated with cyclophosphamide or azathioprine-related toxicity.

Neuro-Behçet y neurotoxicidad por ciclosporina / Neuro-Behçet and neurotoxicity due to cyclosporine

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[Rapid diagnosis unit in a third level hospital. Descriptive study of the first year and a half]. / Unidad de diagnóstico rápido en un hospital de tercer nivel. Estudio descriptivo del primer año y medio de funcionamiento.

BACKGROUND AND AIM: We show a descriptive study of a Rapid Diagnosis Unit as an alternative of the traditional hospital admission. MATERIAL AND METHODS: An Internist, a Radiologist and a Nurse have constituted Rapid Diagnosis Unit. A retrospective and descriptive study of 1132 patients visited in the first and a half- year is described. RESULTS: 1132 patients have been attended, 71% coming from Emergency Department and 26% from Primary Health Care. Mean stay was 9 days. Anaemia, wasting syndrome and lymph nodes enlargement were the most likely complaints. The outcome of these patients was diverse: 38% have been discharged, 52% carried on as outpatients in our hospital and 10% required hospitalization. In 199 patients (18%) cancer was diagnosed. Colon, lymphoma and lung cancer were the most common malignant disorders involved. About 4,5 beds per day have been saved from the Internal Medicine Department. CONCLUSIONS: Rapid Diagnosis Unit is an efficient alternative to conventional hospital admissions for patients in study of potential and serious disorders allowing an out-patient management.

[Endovascular treatment of carotid and pulmonary aneurysms in Behçet's disease]. / Tratamiento endovascular de aneurismas carotídeo y pulmonar en la enfermedad de Behçet.

Arterial aneurysm formation is a rare manifestation in Behçet's disease (BD). Aneurysm rupture is the main cause of death among BD patients. In recent years, endovascular treatment has been proposed as an effective and less invasive procedure compared with classical surgical treatment. We report the case of a 29-year-old man with BD who had two syncopes precipitated by pressure on a left cervical mass and hemoptysis. Cerebral magnetic resonance imaging (MRI) showed acute infarction of left basal ganglia. Other examinations demonstrated carotid and pulmonary aneurysms that were treated by endovascular methods. The patient received treatment with corticosteroids and immunosuppressive therapy. First of all, the pulmonary aneurysm was treated by means of coil embolization. After anticoagulant therapy was initiated, a covered stent was placed to exclude the carotid aneurysm at the level of extracranial internal carotid artery (ICA), with no immediate complications. After twenty-seven months of follow-up the patient remains asymptomatic. Endovascular treatment may be a definite therapeutic option for extracranial carotid aneurysms in BD, although longer term follow-up studies are needed.

Tratamiento endovascular de aneurismas carotídeo y pulmonar en la enfermedad de Behçet / Endovascular treatment of carotid and pulmonary aneurysms in Behçet's disease

La formación de aneurismas arteria les en la enfermedad de Behçet (EB) es rara aunque la rotura de éstos representa la principal causa de mortalidad en los pacientes con EB. En los últimos años se ha propuesto el tratamiento endovascular como una opción terapéutica más segura y menos invasiva que la cirugía convencional. Presentamos un varón de 29 años diagnosticado de EB que presentó dos síncopes desencadenados tras la presión de una masa laterocervical izquierda y hemoptisis. La imagen por resonancia magnética (RM) craneal mostró un infarto agudo en los ganglios basales izquierdos. Otras exploraciones complementarias demostraron la presencia de dos aneurismas, uno carotídeo y otro pulmonar, que se trataron por vía endovascular. El paciente recibió tratamiento con corticoides e inmunodepresores. Primero se embolizó el aneurisma pulmonar mediante coils. Posteriormente, y tras iniciar tratamiento anticoagulante, se excluyó el aneurisma de la arteria carótida interna (ACI) en su porción extracraneal mediante la colocación de un stent (endoprótesis vascular) cubierto, sin complicaciones inmediatas. Después de 27 meses de seguimiento el paciente se encuentra asintomático. A pesar de que son necesarios más estudios a largo plazo, el tratamiento endovascular puede ser una opción terapéutica segura para los aneurismas carotídeos extracraneales en la EB

Arterial aneurysm formation is a rare manifestation in Behçet's disease (BD). Aneurysm rupture is the main cause of death among BD patients. In recent years, endovascular treatment has been proposed as an effective and less invasive procedure compared with classical surgical treatment. We report the case of a 29-year-old man with BD who had two syncopes precipitated by pressure on a left cervical mass and hemoptysis. Cerebral magnetic resonance imaging (MRI) showed acute infarction of left basal ganglia. Other examinations demonstrated carotid and pulmonary aneurysms that were treated by endovascular methods. The patient received treatment with corticosteroids and immunosuppressive therapy. First of all, the pulmonary aneurysm was treated by means of coil embolization. After anticoagulant therapy was initiated, a covered stent was placed to exclude the carotid aneurysm at the level of extracranial internal carotid artery (ICA), with no immediate complications. After twenty-seven months of follow-up the patient remains asymptomatic. Endovascular treatment may be a definite therapeutic option for extracranial carotid aneurysms in BD, although longer term follow-up studies are needed

Parámetros inmunológicos basales en pacientes nonagenarios / Basal immunological parameters in nonagenarian patients

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Morir en el hospital por demencia en fase terminal: análisis de la toma de decisiones después de un programa educativo / Dying in hospital from end-stage dementia: analysis of decision-making after an educational programme

Objetivo: el objetivo de nuestro estudio fue analizar si la toma de decisiones respecto al manejo paliativo de los pacientes ancianos que fallecían en el hospital por demencia en fase terminal mejoraba después de un programa educativo. Material y método: se evaluó a 365 pacientes que fallecieron por demencia en fase terminal en el hospital. En la primera fase se incluyó a 230 pacientes y en la segunda, después de la implantación de una campaña educativa, a otros 135 pacientes. Se investigó si hubo una mejoría en las órdenes escritas sobre reanimación cardiopulmonar, graduación de actuaciones, información a la familia, retirada de la medicación habitual e inicio de tratamiento farmacológico paliativo. Resultados: se ha observado un bajo porcentaje de órdenes de no reanimación cardiopulmonar y graduación de actuaciones, sin diferencias entre ambos períodos evaluados. Respecto a la información proporcionada a la familia sobre el pronóstico, la retirada de la medicación habitual previamente al fallecimiento y el inicio del tratamiento paliativo, el abordaje se consideró adecuado en alrededor del 70% de los casos, sin que se observaran diferencias antes o después de la intervención. Conclusiones: en aproximadamente un tercio de pacientes, tal vez por la dificultad para identificarlos como afectados de demencia en fase terminal, no se aplican las medidas paliativas adecuadas, incluso después de una campaña educativa

Objective: to analyse whether decision-making on the palliative management of elderly patients dying in hospital from end-stage dementia could be improved by an educational intervention. Material and method: a total of 365 patients dying at hospital and suffering from end-stage dementia were analysed (230 patients preintervention and 135 patients post-intervention). We evaluated written information about do not resuscitate orders, gradation of therapeutic decisions, information provided to relatives about prognosis, total withdrawal of routine drug therapy and the provision of terminal care. Results: a low percentage of do no resuscitate orders and gradation of therapeutic and diagnostic procedures was found. There were no statistically significant differences between the two periods analysed. Information given to relatives, drug-withdrawal, and initiation of palliative management was appropriate in 70% of the patients, although no differences were detected between either period. Conclusions: approximately one-third of the patients did not receive adequate palliative care, possibly because of the difficulty of identifying end-stage dementia. This occurred even after the educational intervention

[Geriatric information in the hospital discharge reports of patients over 65 years of age admitted to an Internal Medicine Department]. / Información geriátrica en los informes de alta hospitalaria del servicio de Medicina Interna en pacientes mayores de 65 años.

CONTEXT: The hospital discharge report (HDR) is a document that serves as a link between the hospital clinician and the primary care team. Our objective has been to evaluate the level of geriatric information in the HDR developed in the Internal Medicine Department of an tertiary care university hospital. Besides, the possible changes along the last 5 years were also evaluated. METHODS: HDRs of 1,000 patients over 65 years (500 in 1996 and 500 in 2001) and cared in the Bellvitge University Hospital were evaluated. Data relative to social, functional (qualitative and quantitative), and cognitive (qualitative and quantitative) assessment were collected. RESULTS: In 269 patients (27%) there was some kind of geriatric information in the HDRs. In the 2001 HDRs a significant increase in the percentage of social, qualitative functional and qualitative cognitive information was observed compared to 1996 HDRs. There were no significant differences upon comparing the subgroup of 269 patients with geriatric data in their HDRs with the subgroup of 731 remaining patients. CONCLUSIONS: The level of geriatric information in the HDRs is low. Care of elderly patients admitted to the Internal Medicine Department requires an additional evaluation on the part of all the professionals involved in their assistance, carrying out a global assessment that let a better knowledge of the patient. These evaluations should be part of the HDR.