1/8. Alveolar haemorrhage in anti-glomerular basement membrane disease without detectable antibodies by conventional assays.Anti-glomerular basement membrane (anti-GBM) disease represents the spectrum of disease attributable to circulating anti-GBM antibodies. While active anti-GBM disease in the absence of circulating anti-GBM antibodies has been described, it is considered rare with the use of current routinely available assays. We report four subjects with features consistent with active anti-GBM antibody disease without detectable antibodies by routinely available enzyme linked immunosorbent assay (ELISA) and immunoblot techniques. All were smokers who presented with diffuse alveolar haemorrhage, minimal renal involvement, and undetectable anti-GBM antibodies. Seronegative anti-GBM disease with predominant pulmonary involvement may be more common than previously appreciated and should be part of the differential diagnosis for otherwise unexplained diffuse alveolar haemorrhage. Renal biopsy with immunofluorescent studies should be considered in the diagnostic evaluation of such subjects, including those with idiopathic pulmonary haemosiderosis.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
2/8. Goodpasture's syndrome with normal renal function.Two cases of Goodpasture's syndrome with severe pulmonary haemorrhage and normal renal function are described. In spite of minor (patient 2) or even no (patient 1) biological or light microscopic signs of glomerulonephritis, immunofluorescence of immunoglobulin g (IgG) was strongly positive in a linear fashion along the glomerular basement membranes in both patients. We suggest that renal biopsy in patients with apparently idiopathic pulmonary haemorrhage may lead to an early diagnosis of Goodpasture's syndrome. It is not possible in this disease to recognize on presentation those patients who will remit spontaneously and those who will undergo severe disease. The deadly evolution for patient 1 and some cases in the literature lend support to the notion that cytostatics and plasma exchange must be added to corticosteroids, even if pulmonary haemorrhage is not active and renal function is normal at the time.- - - - - - - - - - ranking = 0.5keywords = haemorrhage (Clic here for more details about this article) |
3/8. Idiopathic Goodpasture's syndrome. Fatal pulmonary haemorrhage and crescentic glomerulonephritis in the absence of immune-reactant deposition.A 58-year-old woman was hospitalized with acute renal failure and unilateral pulmonary consolidation. Ten days later she developed massive pulmonary haemorrhage with diffuse pulmonary consolidation. Renal biopsy revealed 100% crescentic nephritis without immunofluorescence (IF) or electron microscopic evidence of immune reactant deposition. Circulating anti-glomerular basement membrane (antiGBM) antibody was not detectable by radioimmunoassay. Despite aggressive therapy pulmonary haemorrhage eventually proved fatal. IF of lung tissue revealed no immune-reactant deposition. This report represents a case of idiopathic Goodpasture's syndrome, both from an aetiological and an immunopathological viewpoint. It emphasizes that Goodpasture's syndrome (i.e. pulmonary haemorrhage and glomerulonephritis) occurs in a variety of situations which are not mediated by antiGBM antibody deposition and that alveolar haemorrhage should be considered in the differential diagnosis of all radiological pulmonary infiltrates, including unilateral opacities, when abnormalities of renal function coexist.- - - - - - - - - - ranking = 1.3333333333333keywords = haemorrhage (Clic here for more details about this article) |
4/8. Goodpasture's syndrome: case report of a survivor.A patient with Goodpasture's syndrome who presented with severe pulmonary haemorrhage and minimal renal involvement is described. The diagnosis was substantiated by immunofluorescence studies on a renal biopsy specimen and by the detection of circulating antiglomerular basement membrane antibody. The patient was treated with corticosteroids and cyclophosphamide. He remains in good health 20 months after diagnosis, although there is a persisting abnormality in single-breath gas transfer for carbon monoxide. Recovery from Goodpasture's syndrome is reviewed in the light of recent advances.- - - - - - - - - - ranking = 0.16666666666667keywords = haemorrhage (Clic here for more details about this article) |
5/8. penicillamine-induced "Goodpasture's syndrome": successful treatment of a fulminant case.Goodpasture's syndrome is a recognised but rare complication of penicillamine therapy. Return of useful renal function has no been recorded with this complication. A patient is presented in whom aggressive management with plasmapheresis, haemodialysis and immunosuppression in the acute stage resulted in rapid control of pulmonary haemorrhage and return of useful renal function without the need for long-term haemodialysis. Immunofluorescence and electron microscopic studies of renal tissue together with the demonstration of circulating immune complexes, established the immune complex pathogenesis of this patient's disease. Microhaematuria is emphasised as an important warning sign of this complication.- - - - - - - - - - ranking = 0.16666666666667keywords = haemorrhage (Clic here for more details about this article) |
6/8. Wegener's granulomatosis exhibiting the clinical features of Goodpasture's syndrome.A case is reported here, the clinical features of which raised the suspicion of Goodpasture's syndrome which was, however, at variance with the absence of antiglomerular basement membrane antibodies. Renal failure improved on immunosuppressive treatment and peritoneal dialysis, but the patient died of gastric haemorrhage from a peptic ulcer related to steroid treatment. Necropsy and microscopic study revealed abnormalities of liver and spleen, consistent with Wegener's granulomatosis. Immunohistologic studies of the kidney confirmed the presence of granular-type IgG deposits in the glomeruli. There were extensive fibrin deposits in the kidney. The ways and means for the differentiation of Wegener's granulomatosis from Goodpasture's syndrome, the potential pathogenetic role of immunocomplexes and fibrin deposits, and the therapeutic possibilities are discussed.- - - - - - - - - - ranking = 0.16666666666667keywords = haemorrhage (Clic here for more details about this article) |
7/8. Ophthalmic features of fourteen cases of Goodpasture's syndrome.Juxtapapillary subretinal neovascular membranes developed in both eyes of a patient who had been treated for Goodpasture's syndrome for 4 years. These lesions caused visual impairment but were successfully treated by laser photocoagulation. Subretinal neovascularisation has not been reported before in association with Goodpasture's syndrome, but diverse ocular abnormalities have been described. It is not certain whether these lesions were caused by anti-basement-membrane auto-antibodies. The eyes of 13 other patients with Goodpasture's syndrome were examined, in order to detect other unsuspected ocular pathology. In 1 further patient, both retinae contained a few unexplained superficial retinal haemorrhages. During follow-up, the original patient developed bilateral peripheral retinoschisis. From this short series and from cases previously described, we conclude that sight-threatening ocular abnormalities are rare in Goodpasture's syndrome. It is, however, particularly important to be aware of the possibility of treatable eye disease in Goodpasture's syndrome, since the introduction of effective treatment with immunosuppression and plasmapheresis has made long-term survival likely.- - - - - - - - - - ranking = 0.16666666666667keywords = haemorrhage (Clic here for more details about this article) |
8/8. Goodpasture's syndrome.This paper describes the case of a patient who combined severe pulmonary haemorrhages with symptoms of nephritis. These severe progressive changes led to a fatal issue. Clinical and postmortem findings led to the diagnosis 'lung purpura with nephritis', which is known in the literature as Goodpasture's syndrome.- - - - - - - - - - ranking = 0.16666666666667keywords = haemorrhage (Clic here for more details about this article) |