Anti-neutrophil cytoplasmic antibodies in new-onset systemic lupus erythematosus

Abstract: Background: The clinical significance of anti-neutrophil cytoplasmic antibodies in patients with new-onset systemic lupus erythematosus, especially in systemic disease accompanied by interstitial lung disease remains to be elucidated. Objectives: This study was designed to investigate the role of anti-neutrophil cytoplasmic antibodies in new-onset systemic lupus erythematosus patients. Methods: A hundred and seven patients with new-onset SLE were enrolled. Presence of anti-neutrophil cytoplasmic antibodies in the sera was assessed by indirect immunofluorescence as well as enzyme linked immunosorbent assay against proteinase-3 and myeloperoxidase. Clinical features and laboratory parameters of patients were also recorded. All patients were subjected to chest X-ray, chest high-resolution computed tomography and pulmonary function test. Results: Forty-five systemic lupus erythematosus patients (45/107, 42%) were seropositive for anti-neutrophil cytoplasmic antibodies. Compared with anti-neutrophil cytoplasmic antibodies-negative patients, the anti-neutrophil cytoplasmic antibodies-positive patients had significantly higher incidence of renal involvement, anemia, and Raynaud's phenomenon as well as decreased serum level of complement 3/complement 4 and elevated erythrocyte sedimentation rate. In addition, there was a positive correlation between serum anti-neutrophil cytoplasmic antibodies level and disease activity of systemic lupus erythematosus. Furthermore, prevalence of interstitial lung disease in the anti-neutrophil cytoplasmic antibodies -positive patients (25/45, 55.6%) was obviously higher than that in the anti-neutrophil cytoplasmic antibodies-negative patients (15/62, 24.2%). Study limitations: The sample size was limited and the criteria for screening new-onset systemic lupus erythematosus patients might produce bias. Conclusions: The level of anti-neutrophil cytoplasmic antibodies in new-onset systemic lupus erythematosus patients correlates positively with the disease activity and the prevalence of interstitial lung disease.

Myelopathy in systemic lupus erythematosus: clinical, laboratory, radiological and progression findings in a cohort of 1,193 patients / Mielopatia no lúpus eritematoso sistêmico: achados clínicos, laboratoriais, radiológicos e evolutivos em uma coorte de 1. 193 pacientes

Abstract Objective To describe clinical, laboratory, radiological and progression characteristics of myelopathy in systemic lupus erythematosus (SLE). Patients and methods A retrospective analysis was performed on a cohort of 1193 patients with SLE (ACR criteria) in order to identify patients with myelopathy (neuropsychiatric ACR). Disease activity was assessed by the SLE activity index (SLEDAI) on the date of the event and functional capacity was assessed by the Expanded Disability Status Scale (EDSS) at the last visit. Results We identified 14 (1.2%) patients with myelopathy. All were women with a mean age of 30 ± 11.5 years. Myelopathy occurred at the diagnosis of SLE in four (28%) patients; and nine (64%) patients had another type of neuropsychiatric manifestation associated. Neurological recurrence was observed in one (7%) patient. Disease activity was observed in 2 (14%) patients. Cerebrospinal fluid presented pleocytosis on 7 (53%) patients; antiphospholipid antibodies were positive in 5 (45%). Magnetic resonance imaging (MRI) showed T2 hyperintensity with a predominance of longitudinal involvement in 6 (86%) patients. Most were treated with intravenous corticosteroids and cyclophosphamide. No patient had full recovery and four (36%) had high EDSS scores. Three (21%) patients died from sepsis early in the course of their myelopathy, during or after immunosuppressive therapy. Conclusions Myelopathy occurred in 14 (1.2%) of the patients in our cohort and this may be the first manifestation of the disease occurring independently of systemic disease activity. Although rare, myelopathy shows great morbidity and mortality, can be recurrent and MRI is critical for diagnosis.

Resumo Objetivo Descrever características clínicas, laboratoriais, radiológicas e evolutivas de mielopatia no lúpus eritematoso sistêmico (LES). Pacientes e métodos Foi feita análise retrospectiva de uma coorte de 1.193 pacientes com LES (critérios ACR) para identificar os pacientes com mielopatia (ACR neuropsiquiátrico). A atividade de doença foi analisada pelo Índice de Atividade do LES (Sledai) na data do evento e a capacidade funcional pela Escala Expandida do Estado de Incapacidade (EDSS) na última consulta. Resultados Foram identificados 14 (1,2%) pacientes com mielopatia. Todas eram mulheres com média de 30 anos (DP ± 11,5 anos). A mielopatia ocorreu no diagnóstico do LES em quatro (28%) e em nove (64%) havia outro tipo de manifestação neuropsiquiátrica associada. Recorrência do quadro neurológico foi observado em uma (7%) paciente. Atividade de doença foi observada em dois (14%) pacientes. O líquido cefalorraquidiano apresentava pleocitose em sete (53%) pacientes anticorpos antifosfolípides eram positivos em cinco (45%). A ressonância magnética (RM) demonstrou hipersinal em T2 com predomínio do comprometimento longitudinal em seis (86%) pacientes. A maioria foi tratada com corticosteroides e ciclofosfamida endovenosos. Nenhuma paciente teve completa recuperação e quatro (36%) tinham escores altos da EDSS. Óbito foi observado em três (21%) durante episódio de mielopatia, por septicemia durante ou após terapia imunossupressora. Conclusões A mielopatia ocorreu em 14 (1,2%) dos pacientes da nossa coorte e pode ser a primeira manifestação da doença e ocorrer independentemente de atividade sistêmica da doença. Embora rara, é de grande morbimortalidade, pode ser recorrente e a RM é fundamental para o diagnóstico.

Lupus eritematoso sistémico en escolar de 9 años, reporte de un caso / Systemic lupus erythematosus in a 9 years old student, case report

INTRODUCCIÓN: El Lupus eritematoso sistémico (LES) es una enfermedad que afecta a múltiples sistemas corporales, caracterizada por la presencia de autoanticuerpos. El inicio del LES en edad pediátrica corresponde aproximadamente al 10-20 por ciento del total de pacientes con LES. PRESENTACIÓN DEL CASO: Escolar de sexo femenino, nueve años y seis meses de edad. Cuadro caracterizado por gonalgia bilateral y eritema malar polimorfo que desapareció espontáneamente. Luego de un mes de evolución se agregó poliartralgia además de compromiso progresivo del estado general, anorexia, pérdida ponderal importante, fiebre, gingivorragia y epistaxis. Dentro de los exámenes realizados destacaban: pancitopenia y transaminasas elevadas. Se descartó leucemia aguda con realización de mielograma. Se continuó estudio con la hipótesis diagnóstica de mesenquimopatía. En exámenes realizados destacaban: Anti DNA (+), ANA (+). Amilasa: 422 UI/L, Hematocrito: 31 por ciento, Hemoglobina: 10,7 g/dl, Tiempo de Tromboplastina Parcial Activado: 71,8 segundos, Protrombina: 10,9 segundos. Ante el diagnóstico de LES se inició tratamiento con corticoides sistémicos. Se agregó posteriormente tratamiento con Hidroxicloroquina y Azatioprina de mantención. Evolucionó favorablemente, con mejoría de los parámetros clínicos y de laboratorio. DISCUSIÓN: El diagnóstico de LES pediátrico no es fácil. La mayoría de los niños debutan con síntomas inespecíficos por lo que se requiere tener un alto nivel de sospecha diagnóstica, además de exámenes de laboratorio para comprobar o descartar la patología. Respecto al tratamiento del LES pediátrico existe consenso de que éste debe incluir Hidroxicloroquina ya que disminuye las complicaciones derivadas de la enfermedad y mejora la sobrevida y el pronóstico.

INTRODUCTION: Systemic lupus erythematosus (SLE) is a pathology that affects multiple corporal systems, it is characterized by the presence of autoantibodies. Pediatric onset lupus accounts for about 10-20 percent of all patients with SLE. CASE REPORT: Female student, normal weight, nine years and six months old. Clinical picture characterized by bilateral knee ache and malar rash that disappeared spontaneously. One month later appeared polyarthralgia, fatigue, anorexia, important weight loss, fever, gums bleeding and epistaxis. Laboratory tests showed pancytopenia and elevated transaminases. The diagnosis of acute leukaemia was excluded by the realization of a myelogram. By the probable diagnostic of mesenchymal pathology, more laboratory tests were done to continue the study of the case. These tests showed: Anti DNA antibodies (+), ANA test (+). Amylases: 422 UI/L, Hematocrit: 31 percent, Haemoglobin: 10.7 g/dl, Activated Partial Thromboplastin time: 71.8 seconds, Prothrombin: 10.9 seconds. The treatment started with systemic steroids after SLE was diagnosed. Later Hydroxychloroquine and Azathioprine was added permanently. The patient evolved favorably with improvement of clinical and laboratory parameters. DISCUSSION: Diagnosing SLE in children is not always easy. Most children present non-specific symptoms, that is why is required an accurate clinical suspicion. Furthermore, laboratory tests are needed to confirm or exclude the pathology. There is consensus about pediatric SLE treatment, it must include Hydroxychloroquine because it decreases complications of SLE and improves the prognosis

Inter- and intraobserver variation between radiologists in the detection of abnormal parenchymal lung changes on high-resolution computed tomography

Radiological and histological evaluations are affected by subjective interpretation. This study determined the level of inter- and intraobserver variation among radiologists for detection of abnormal parenchymal lung changes on high resolution computed tomography [HRCT]. HRCT images of 65 patients known to have systemic lupus erythematosus [with clinical pulmonary involvement] were retrospectively reviewed by four nonthoracic radiologists [two with expertise in magnetic resonance [MR] and two general radiologists]. Each radiologist read the scans twice, with an interval between readings of at least 6 months. The interobserver variation among the first and second readings of the four radiologists and the intraobserver variation of each radiologist's two readings were assessed by the kappa statistic. There was good agreement between the first and second readings of each radiologist. There was moderate agreement between the two readings of one MR radiologist [kappa=0.482]; the other three radiologists had kappa values that were good to excellent [0.716, 0.691, and 0.829]. There was a clinically acceptable level of interobserver variability between all radiologists. The agreement was fair to moderate between the MR radiologist and the other observers [kappa range: 0.362-0.519] and moderate to good between the other three radiologists [0.508-0.730]. The interpretation of imaging findings of abnormal parenchymal lung changes on HRCT is reproducible and the agreement between general radiologists is clinically acceptable. There is reduced agreement when the radiologist is not involved on a regular basis with thoracic imaging. Difficult or indeterminate cases may benefit from review by a chest radiologist