RESUMO
Lemierre's syndrome is a rare disease in the antibiotic era which is characterised by oropharyngitis, thrombophlebitis and septic metastases, but it can still occur and early recognition of its characteristic features and commencement of appropriate treatment can be life saving. The case history is described of a middle-aged man with Lemierre's syndrome who made an uneventful recovery following treatment with co-amoxiclav and metronidazole for 6 weeks.
Assuntos
Fusobacterium necrophorum/isolamento & purificação , Síndrome de Lemierre , Humanos , Veias Jugulares/diagnóstico por imagem , Síndrome de Lemierre/complicações , Síndrome de Lemierre/diagnóstico por imagem , Síndrome de Lemierre/terapia , Pulmão/diagnóstico por imagem , Abscesso Pulmonar/diagnóstico por imagem , Abscesso Pulmonar/etiologia , Masculino , Pessoa de Meia-Idade , Radiografia , Trombose/diagnóstico por imagem , UltrassonografiaRESUMO
AIMS: Although pulmonary involvement is a known cause of morbidity in Niemann-Pick disease, histological features in the lung are not well characterized. The purpose of this study is to document the histological features seen in pulmonary involvement by types B and C Niemann-Pick disease and to correlate them with clinical and imaging data. METHODS AND RESULTS: Surgical lung biopsies from six patients (four with type B and two with type C disease) were reviewed and all showed diffuse endogenous lipid pneumonia, with lesser involvement of the interstitium by fibrosis and foamy macrophage accumulation. In type B disease only, there was also fine cytoplasmic vacuolation within the cytoplasm of ciliated epithelial cells. Neither disease showed foamy changes within pneumocytes. One patient had a bronchial cast removed on whole lung lavage. Electron microscopy showed abnormal lamellar inclusions within lysosomes of affected cells in type B disease. In patients with type C disease, biopsies were undertaken as part of investigations into acute respiratory failure in the context of multiorgan systemic presentation. Three patients with type B disease had clinical disease limited to the lung, all adults (mean age of 40 years) with unexplained diffuse parenchymal lung disease and mainly ground-glass shadowing on high-resolution computed tomography. CONCLUSIONS: Niemann-Pick disease should be considered for any patient with unexplained diffuse endogenous lipid pneumonia, even when disease is limited to the lungs and presentation is during adulthood.
