1/31. 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 = 1keywords = alveolar (Clic here for more details about this article) |
2/31. 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 = 1keywords = alveolar (Clic here for more details about this article) |
3/31. Rapidly progressive antineutrophil cytoplasm antibodies associated with pulmonary-renal syndrome in a 10-year-old girl.CONTEXT: The term pulmonary-renal syndrome has been used frequently to describe the clinical manifestations of a great number of diseases in which pulmonary hemorrhage and glomerulonephritis coexist. The classic example of this type of vasculitis is Goodpastures syndrome, a term used to describe the association of pulmonary hemorrhage, glomerulonephritis and the presence of circulating antiglomerular basement membrane antibodies (anti-GBM). Among the several types of systemic vasculitides that can present clinical manifestations of the pulmonary-renal syndrome, we focus the discussion on two types more frequently associated with antineutrophil cytoplasm antibodies (ANCA), microscopic polyangiitis and Wegener's granulomatosis, concerning a 10 year old girl with clinical signs and symptoms of pulmonary-renal syndrome, with positive ANCA and rapidly progressive evolution. CASE REPORT: We describe the case of a 10-year-old girl referred to our hospital for evaluation of profound anemia detected in a primary health center. Five days before entry she had experienced malaise, pallor and began to cough up blood-tinged sputum that was at first attributed to dental bleeding. She was admitted to the infirmary with hemoglobin = 4 mg/dL, hematocrit = 14 %, platelets = 260,000, white blood cells = 8300, 74 % segmented, 4 % eosinophils, 19 % lymphocytes and 3 % monocytes. Radiographs of the chest revealed bilateral diffuse interstitial alveolar infiltrates. There was progressive worsening of cough and respiratory distress during the admission day, when she began to cough up large quantities of blood and hematuria was noted. There was rapid and progressive loss of renal function and massive lung hemorrhage. The antineutrophil cytoplasm antibody (ANCA) test with antigen specificity for myeloperoxidase (anti-MPO) was positive and the circulating anti-GBM showed an indeterminate result.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
4/31. Goodpasture's syndrome: a case of delayed appearance of autoantibodies and renal disease.Goodpasture's syndrome, or anti-glomerular basement membrane (anti-GBM) disease, is a rare pulmonary-renal syndrome that results from autoantibody-mediated destruction of alveolar and glomerular basement membranes. It is defined by the triad of pulmonary hemorrhage (hemoptysis), glomerulonephritis (hematuria), and circulating anti-GBM antibodies. Although kidney disease can occur with or without lung involvement, isolated alveolar hemorrhage as an incipient manifestation of the syndrome is rare. We report the case of a patient with anti-GBM disease who initially presented with seronegative hemoptysis and normal urine. It was not until relapse of his condition that we found acute glomerulonephritis and circulating antibodies. In this report, we briefly review the pathogenesis and clinical manifestations of anti-GBM disease and discuss the relevance of isolated alveolar hemorrhage.- - - - - - - - - - ranking = 3keywords = alveolar (Clic here for more details about this article) |
5/31. Goodpasture's syndrome: two cases with contrasting early course and management.In 2 patients with hemoptysis and hematuria, Goodpasture's syndrome was documented by clinical presentation, demonstration of circulating anti-glomerular basement membrane antibodies, and demonstration of linear immunofluorescence along glomerular and alveolar basement membranes. One patient was considered to have progressive disease and was treated with prednisone and cyclophosphamide. During the subsequent 18-month follow-up period, clinical remission occurred with eventual disappearance of hematuria and detectable circulating anti-glomerular basement membrane antibodies. The other patient did not have active hemoptysis at the time of referral and no therapy was instituted. After 12 months of follow-up, hemoptysis remains quiescent but hematuria and positive circulating anti-glomerular basement membrane antibodies persist. Although therapy appears to have been particularly effective in one patient, the benign course of the other patient precludes premature enthusiasm for early intervention.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
6/31. A case of Goodpasture's syndrome associated with anti-myeloperoxidase antibodies.A case of Goodpasture's syndrome with anti-myeloperoxidase (MPO) antibodies is reported. Histological examination revealed crescentic glomerulonephritis and alveolar hemorrhage with linear deposition of IgG along the glomerular capillary walls and alveolar capillary walls by immunofluorescence microscopy. Not only anti-glomerular basement membrane (GBM) antibodies but also anti-MPO antibodies, an anti-neutrophil cytoplasmic antibody, were simultaneously detected in the serum. Although it is generally accepted that crescentic glomerulonephritis in Goodpasture's syndrome is mediated by anti-GBM antibodies, this case suggested that anti-MPO antibodies might also participate in the pathogenesis of crescentic glomerulonephritis and probably alveolar hemorrhage of Goodpasture's syndrome, especially with vasculitis.- - - - - - - - - - ranking = 3keywords = alveolar (Clic here for more details about this article) |
7/31. Goodpasture's syndrome: a report of an autopsy case and a review of Japanese cases.A 75-yr-old man was admitted because of acute renal failure. On the 9th hospital day, pulmonary hemorrhage occurred. He was treated with a bolus dose of methylprednisolone and plasma exchange, but died from respiratory failure on the 16th hospital day. autopsy findings demonstrated marked intraalveolar hemorrhage and crescentic glomerulonephritis. Linear depositions of IgG along both alveolar and glomerular basement membranes (GBM) were shown by direct immunofluorescent studies. Circulating anti-GBM antibodies were demonstrated by indirect immunofluorescent techniques. This is a confirmatory case of Goodpasture's syndrome, which is rare in japan. A review of the Japanese literature revealed some characteristics of Japanese cases.- - - - - - - - - - ranking = 2keywords = alveolar (Clic here for more details about this article) |
8/31. Pulmonary-renal syndrome in a patient with bacterial endocarditis.Pulmonary-renal syndrome is defined as a combination of pulmonary hemorrhage and glomerulonephritis. We report an unusual case of bacterial endocarditis presenting with pulmonary hemorrhage and rapidly progressive glomerulonephritis as the initial manifestations of the disease. A 37-year-old man was admitted with fever, hemoptysis, and dyspnea. Admission examinations revealed severe renal failure requiring dialysis therapy. Chest radiograph showed extensive pulmonary reticulonodular infiltrates. echocardiography revealed ventricular septal defect. Furthermore, blood cultures grew viridians group streptococci. The kidney and lung biopsies demonstrated diffuse cresentic glomerulonephritis and alveolar hemorrhage, respectively. Bacterial endocarditis was diagnosed according to the Duke criteria and the patient was treated with intravenous antibiotic therapy. The pulmonary infiltrates disappeared gradually. However, renal function did not improve, even after trial of a course of immunosuppressive therapy. The patient survived and remained on regular hemodialysis. We conclude that bacterial endocarditis should be included in the differential diagnosis of pulmonary-renal syndrome.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
9/31. 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 = 2keywords = alveolar (Clic here for more details about this article) |
10/31. Concurrent anti-glomerular basement membrane disease and membranous glomerulonephritis: a case report and literature review.BACKGROUND: anti-glomerular basement membrane disease (anti-GBM) is a relatively rare entity characterized by antibodies to collagen type iv of glomerular and alveolar basement membranes. The sequential or simultaneous presentation of anti-glomerular basement membrane disease with membranous glomerulonephritis has been infrequently described. CASE: We present the case of a 49-year-old man who had fatigue, flank pain, hematuria and renal failure. serology was positive for anti-GBM antibodies; crescentic glomerulonephritis was seen on renal biopsy. Immunofluorescence and electron microscopy demonstrated evidence of both anti-GBM glomerulonephritis and membranous deposits. DISCUSSION: Simultaneous anti-GBM disease and membranous glomerulonephritis is the most common temporal presentation of this rare entity. However, cases of membranous glomerulonephritis preceding or following recovery from anti-GBM disease have been described. Study of such cases provides insight into pathophysiologic mechanisms, including the possibility of increased antigen synthesis, exposure of cryptic epitopes, and/or capping and shedding of antigen-antibody complexes, in analogy to Heymann nephritis.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
| | Next -> |