Résumé
Both anti-glomerular basement membrane (GBM) disease and the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are common causes of pulmonary-renal syndrome. Organizing pneumonia (OP), a special pattern of interstitial lung disease, is extremely rare either in AAV or anti-GBM disease. We report an old woman presented with OP on a background of co-presentation with both ANCA and anti-GBM antibodies.
Sujets)
Femelle , Humains , Anticorps anti-cytoplasme des polynucléaires neutrophiles , Pneumonie organisée , Autoanticorps , Glomérulonéphrite , Maladie des anticorps antimembrane basale glomérulaire , Pneumopathie infectieuse , Vascularites associées aux anticorps anti-cytoplasme des neutrophiles/complicationsRésumé
A 29-year-old male patient was admitted due to his general weakness and poor oral intake for several months. He was diagnosed as having Crohn disease 16 years ago and total colectomy was performed 10 years ago. On the 3rd day after admission, gross hematuria and sudden hemoptysis combined with diffuse infiltration were noted on chest X-ray. His symptoms and the diffusely increased lung opacities improved with administering high-dose steroid therapy. Later, anti-GBM antibody was found to be positive on the laboratory findings. We report here on a rare case of Goodpsture syndrome combined with prolonged Crohn disease along with a review of literature.
Sujets)
Adulte , Humains , Mâle , Maladie des anticorps antimembrane basale glomérulaire , Colectomie , Maladie de Crohn , Hématurie , Hémoptysie , Poumon , ThoraxRésumé
A 63-year-old woman presented to the hospital with gross hematuria and acute renal failure. Kidney function deteriorated rapidly and progressively. A renal biopsy revealed segmental or circumferential crescents associated with linear deposits of immunoglobulin G, typical of anti-glomerular basement membrane disease. Both c-ANCA and anti-GBM antibody were detected in serum. She was treated with hemodialysis, plasmapheresis, high dose steroid and cyclophosphamide. However, she died 7 weeks after treatment because of pneumonia, without recovery of renal function. Serologic positivity of both ANCA and anti-GBM antibody are becoming more frequently recognized in rapidly progressive glomerulonephritis. The influence of c-ANCA on the clinical course of anti-GBM glomerulonephritis remains to be determined.
Sujets)
Femelle , Humains , Adulte d'âge moyen , Atteinte rénale aigüe , Maladie des anticorps antimembrane basale glomérulaire , Anticorps anti-cytoplasme des polynucléaires neutrophiles , Membrane basale , Biopsie , Cyclophosphamide , Cytoplasme , Glomérulonéphrite , Hématurie , Immunoglobuline G , Rein , Plasmaphérèse , Pneumopathie infectieuse , Dialyse rénaleRésumé
To investigate the mechanism of crescent formation, sequential pathologic changes from the New Zealand White rabbits with anti-GBM antibody induced GN by administration of guinea pig anti-GBM IgG were studied by light (LM), immunofluorescent (IF) and electron (EM) microscopy. Although no glomerular changes were observed in LM, swelling of the endothelial cells and the epithelial cells were noted in EM by day 2. By day 7, early and cellular crescents were evident. Proteinaceous materials and fibrins were noted in the glomerular capillary lumina (GCL) and Bowman's space (BS) associated with segmental hypercellularity. The GBM damage became progressively severe, followed by focal detachment of the visceral epithelial cells from the GBM. At day 14, fibrin strands, mononuclear cells and collagen fibrils were present between the proliferating extracapillary cells. At day 31, fibrocellular crescents were predominated. Elongated spindle cells, morphologically resembling myofibroblasts, were noted near the Bowman's capsule (BC). A degree of tubular atrophy, interstitial fibrosis, and inflammatory infiltrates increased as it did with fibrous organization of crescent. Intense linear IF staining for IgG and C3 were seen throughout the experiments along the GBM. In conclusion, the progression of crescent from an early "proteinaceous" stage through cellular, fibrocellular and fibrous stages was well documented in this study. Inflammatory cells and coagulation mechanism may activate the initiation of the GBM damage at the early stage. Activated periglomerular mononuclear cells may also cause disruption of BC which facilitates entry of activated periglomerular cells and fibroblasts into BS leading to progressive fibrous crescent formation.
Sujets)
Animaux , Lapins , Atrophie , Capsule de Bowman , Vaisseaux capillaires , Collagène , Cellules endothéliales , Cellules épithéliales , Fibrine , Fibroblastes , Fibrose , Glomérulonéphrite , Cochons d'Inde , Immunoglobuline G , Microscopie , MyofibroblastesRésumé
AIM To investigate the protective effects and mechanisms of TMP on accelerated anti-glomerulus basement membrane(GBM) antibody nephritis of rats. METHODS Model of accelerated anti-GBM nephritis was established in rats and TMP was administrated 120 mg?kg -1 ?d -1 by ip since the day before the model established, consecutively for 15 days. Content of 24h urine protein was detected after model established and rats were killed on d 7 and d 14. Contents of blood urine nitrogen (BUN) and serum creatinine (SCr) were detected. Renal cortex cytoplasm and mitochondria were produced and change of content of lipid peroxidation products malondialdehyde(MDA), activities of glutathione peroxidase(GSH-Px) and catalase(CAT) and superoxide dismutase(SOD) were investigated. RESULTS Compared with model group, TMP group showed significant inhibition in changes of proteinuria and BUN, SCr(P