1/115. IgA antiglomerular basement membrane disease associated with bronchial carcinoma and monoclonal gammopathy.Antiglomerular basement membrane (anti-GBM) disease is characterized by a linear deposition of immunoglobulins along the glomerular basement membrane. A 67-year-old man with a recently discovered monoclonal gammopathy of unknown significance (MGUS) presented with microscopic hematuria, nephrotic-range proteinuria, and rapidly deteriorating renal function after a pneumonia. Renal histology showed a crescentic glomerulonephritis; immunohistology showed intense linear staining of the GBM with immunoglobulin a (IgA) and moderate linear staining with kappa and lambda light chains. Screening for systemic disease, including diabetes mellitus, lupus erythematodes disseminatus, cryoglobulinemia, was negative. Serological tests for detection of anti-GBM antibodies were positive for IgA class and negative for IgG. Further examination indicated a bronchial carcinoma T2N2M0. This clinical report adds new information to the spectrum of anti-GBM disease and suggests that neoplasia may be associated with unusual exposure of and/or immune response to epitopes in the GBM.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
2/115. Antiglomerular basement membrane antibody-mediated glomerulonephritis after intranasal cocaine use.We report a case of rapidly progressive glomerulonephritis due to antiglomerular basement membrane (anti-GBM) antibodies that progressed to end-stage renal disease in a 35-year-old man who used intranasal cocaine on an occasional basis. In contrast to many prior reports of acute renal failure occurring with cocaine-associated rhabdomyolysis, this patient did not have any evidence of acute muscle damage and myoglobin release. Circulating anti-GBM antibodies and renal biopsy with linear IgG and C3 deposits confirmed the diagnosis of anti-GBM disease. The possibility of anti-GBM must be considered in the differential diagnosis of acute renal failure in cocaine addicts. This unusual combination raises complex questions regarding the pathogenesis of this type of renal injury.- - - - - - - - - - ranking = 0.83333333333333keywords = membrane (Clic here for more details about this article) |
3/115. Sequential development of anti-GBM nephritis and ANCA-associated Pauci-immune glomerulonephritis.The medical history is presented of a 23-year-old man experiencing three episodes of pulmonary-renal syndrome. On the first occasion, a diagnosis of anti-glomerular basement membrane (GBM) disease (with linear deposition of immunoglobulin g [IgG] along the GBM) was made, whereas anti-neutrophil cytoplasmic autoantibodies were also present in serum. On the third occasion, 5 years later, p-ANCA-associated vasculitis (with pauci-immune crescentic glomerulonephritis) was diagnosed, whereas anti-GBM antibodies were absent. The current literature on ANCA-positive anti-GBM disease is briefly reviewed. A substantial proportion (20% to 30%) of patients with histologically and serologically proven anti-GBM nephritis display the presence of ANCA as well. In this group of patients with dual antibodies, clinical and histological findings suggest that ANCA are not merely epiphenomena, but are of pathogenetic importance and might be responsible for an initial vasculitic insult to the kidney with resultant secondary anti-GBM nephritis. The clinical course in our patient lends further support to this concept. Histological demonstration of anti-GBM nephritis followed by ANCA-associated pauci-immune glomerulonephritis in a single patient has not been reported before.- - - - - - - - - - ranking = 0.16666666666667keywords = membrane (Clic here for more details about this article) |
4/115. Recurrent Goodpasture's disease due to a monoclonal IgA-kappa circulating antibody.We describe the case of a 54-year-old man who first presented with a clinical syndrome manifested by recurrent pulmonary hemorrhage, hematuria, and mild renal insufficiency. Direct immunofluorescence of renal biopsy sections showed linear deposition of IgA-kappa in the glomerular (GBM) and tubular basement membranes. serum protein immunoelectrophoresis was positive for a monoclonal immunoglobulin A (IgA)-kappa protein. serum analysis showed circulating IgA anti-GBM antibodies. Treatment with high-dose steroids, cyclophosphamide, and plasma exchange resulted in resolution of the clinical picture. To the best of our knowledge, this is the first report of Goodpasture's disease associated with the presence of a circulating monoclonal IgA-kappa antibody.- - - - - - - - - - ranking = 0.16666666666667keywords = membrane (Clic here for more details about this article) |
5/115. Goodpasture's syndrome: a nursing challenge.Goodpasture's syndrome is an uncommon occurring disorder that causes hemorrhage in the basement membrane lining of the kidneys and the lungs. By recognizing early signs and symptoms of pulmonary-renal syndromes that may lead to a diagnosis of Goodpasture's syndrome, critical care and advanced practice nurses can play a key role in ensuring successful patient outcomes and preventing complications. Expert nursing care and emotional support is essential for the patient and family to cope with this usually fatal disease.- - - - - - - - - - ranking = 0.16666666666667keywords = membrane (Clic here for more details about this article) |
6/115. A case of Goodpasture's syndrome with massive pulmonary hemorrhage.We present a typical case of Goodpasture's syndrome with massive pulmonary hemorrhage and acute deterioration of renal function. A 20-year-old male was admitted due to severe azotemia (blood urea nitrogen 214.7 mg/dL, serum creatinine 30.2 mg/dL) and was treated with emergency hemodialysis. On the 4th hospital day, a sudden onset of pulmonary hemorrhage developed. The circulating level of anti-glomerular basement membrane antibody was then elevated highly, and the kidney biopsy showed crescentic glomerulonephritis and linear deposition of IgG along the glomerular capillary. The patient was treated with intravenous high dose-steroid, oral cyclophosphamide and plasma exchanges. The pulmonary hemorrhage improved with the therapy, however, his renal function did not improve. He is currently on a regular schedule of hemodialysis.- - - - - - - - - - ranking = 0.16666666666667keywords = membrane (Clic here for more details about this article) |
7/115. Sequential development of pulmonary hemorrhage with MPO-ANCA complicating anti-glomerular basement membrane antibody-mediated glomerulonephritis.We report a case of rapidly progressive glomerulonephritis caused by anti-glomerular basement membrane (anti-GBM) antibodies that progressed to end-stage renal disease in a 67-year-old woman with diabetes. Intensive combined immunosuppressive therapy with methylprednisolone bolus, oral prednisone, and cyclophosphamide led to negativity of anti-GBM antibodies but was not able to restore renal function. After 28 months of hemodialysis, the patient suddenly presented with pulmonary hemorrhage. In this setting, high levels of myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA) and negative anti-GBM antibodies were found. Therapy with oral prednisone and cyclophosphamide led to resolution of pulmonary hemorrhage and negativity of MPO-ANCA.- - - - - - - - - - ranking = 0.83333333333333keywords = membrane (Clic here for more details about this article) |
8/115. Serial complement studies in a patient with Goodpasture's syndrome treated with bilateral nephrectomy and renal transplantation.A young male patient with Goodpasture's syndrome was treated with bilateral nephrectomy and when antiglomerular basement membrane antibodies could no longer be detected he received a cadaveric renal homograft. Fifteen months later he is in good health and without signs of pulmonary disease. Renal function is satisfactory, and there are no findings indicating recurrence of the nephritis. Serial complement studies during the entire course revealed varying degrees of activity in the sequence in the different phases: a high degree of in vivo activation of complement was found in the period before the nephrectomy, there was a moderate degree of activation in the period between the nephrectomy and transplantation and, finally, there were no signs of activity in the system after transplantation. This investigation strongly suggests that the complement system is of definite pathogenetic significance in this human equivalent to experimental nephrotoxic nephritis.- - - - - - - - - - ranking = 0.16666666666667keywords = membrane (Clic here for more details about this article) |
9/115. Alveolar capillary basement membrane lesions in Goodpasture's syndrome and idiopathic pulmonary hemosiderosis.An ultrastructural study of lung distinguised between lesions of the alveolar capillary basement membrane in a case of Good pasture's syndrome and in three cases of idiopathic pulmonary hemosiderosis. In Goodpasture's syndrome, diffuse vascular injury with wide endothelial gaps, diffusely fragmented basement membranes and an electron dense layer on the basement membrane was found. In idiopathic pulmonary hemosiderosis, focal ruptures of the basement membrane were associated with hydropic changes in pneumocytes and, although fibroblasts were not seen, collagen deposition occurred within the basement membrane. immunofluorescent studies failed to show deposition of immunoglobulins G (IgG), A (IgA), M (IgM) or C3 in the lung in either disease. The ultrastructural lesions appear to separate these clinically similar entities.- - - - - - - - - - ranking = 1.5keywords = membrane (Clic here for more details about this article) |
10/115. IgA antibasement membrane nephritis with pulmonary hemorrhage.Goodpasture's syndrome has characteristically been described as being mediated by IgG antibodies. We have recently seen a 55-year-old man who developed renal failure and hemoptysis; a renal biopsy showed linear deposits of IgA and C3 involving glomerular and tubular basement membrane. serologic tests for detecting (IgG) antiglomerular basement membrane antibodies were negative. Elution studies of kidney and lung showed the presence of an IgA antibasement membrane antibody only. The patient's serum contained IgA, but not IgG, antibodies reactive with glomerular and tubular basement membrane of normal human kidney and alveolar basement membrane of normal human lung. Attempts to transfer disease with the patient's IgA antibody to a monkey and to Lewis and Brown-norway rats were unsuccessful. immunoglobulin a antibasement membrane antibody must be considered in the design of immunoserologic procedures for the diagnosis of Goodpasture's syndrome.- - - - - - - - - - ranking = 1.6666666666667keywords = membrane (Clic here for more details about this article) |
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