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1/174. 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. ( info)

2/174. hypotension, bradycardia, and asystole after high-dose intravenous methylprednisolone in a monitored patient.

    We report a case of hypotension, bradycardia, and asystole after intravenous administration of high-dose methylprednisolone in a 73-year-old patient who underwent electrocardiographic (ECG) monitoring throughout the episode. There was a history of ischemic cardiac disease 9 years earlier. The patient was admitted with a pulmonary-renal syndrome with hemoptysis, rapidly progressive renal failure, and hypoxemia that required mechanical ventilation in the intensive care unit. After receiving advanced cardiopulmonary resuscitation, the patient recovered cardiac rhythm. The ECG showed a junctional rhythm without ventricular arrhythmia. This study reviews the current proposed mechanisms of sudden death after a high dose of intravenous methylprednisolone (IVMP). These mechanisms are not well understood because, in most cases, the patients were not monitored at the moment of the event. Rapid infusion and underlying cardiac disease were important risk factors in the case reported here, and the authors discount ventricular arrhythmia as the main mechanism. ( info)

3/174. 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. ( info)

4/174. 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. ( info)

5/174. 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. ( info)

6/174. 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. ( info)

7/174. 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. ( info)

8/174. Pulmonary renal syndrome in childhood: a report of twenty-one cases and a review of the literature.

    In adults, the term specific pulmonary renal syndrome describes disorders with pulmonary and glomerular manifestations and includes Wegener's granulomatosis, Goodpasture disease, and systemic lupus erythematosus. Nonspecific pulmonary renal syndrome refers to either pulmonary disease complicating glomerular disease, or glomerular diseases following pulmonary disease. Since little is known regarding pulmonary renal syndrome in childhood, we reviewed the charts of 21 pediatric patients with pulmonary renal syndromes treated by the Department of pediatrics, University of Bern between 1991 and 1998; we also reviewed the pediatric literature that deals with specific pulmonary renal syndromes. Specific pulmonary renal syndrome was noted in 3 children with systemic vasculitis (wegener granulomatosis, N = 2; microscopic polyangiitis, N = 1) and 2 with systemic lupus erythematosus. Nonspecific pulmonary renal syndrome was observed in 12 patients with pulmonary edema (N = 9), pulmonary thromboembolism (N = 2), and pulmonary infection (N = 1) complicating the course of a glomerular disease, and in 4 children with a pulmonary disease followed by a glomerular disease. review of the literature disclosed 52 cases of specific pulmonary renal syndrome other than systemic lupus erythematosus: wegener granulomatosis (N = 28), Goodpasture disease (N = 13), and Henoch-Schonlein purpura (N = 11). In addition, hemolytic uremic syndrome complicated pneumococcal pneumonia in 32 cases. We conclude that pulmonary renal syndromes need to be looked for in childhood. Apart from wegener granulomatosis, Goodpasture disease, and systemic lupus erythematosus, Henoch-Schonlein purpura and hemolytic-uremic syndrome occasionally have both pulmonary and renal features. ( info)

9/174. 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. ( info)

10/174. Immunoadsorption in Goodpasture's syndrome.

    patients with Goodpasture's syndrome presenting with dialysis-dependent end-stage renal failure at diagnosis almost never regain independent renal function. We report a patient with a 100% crescentic lesion in whom reversal of dialysis dependence was achieved by immunoadsorption together with immunosuppression. In a second patient, early initiation of immunoadsorption was able to completely restore normal renal function as early as 1 month after the start of treatment. These data give evidence of the use of immunoadsorption as a hopeful alternative to conventional plasma exchange in patients with Goodpasture's syndrome showing advanced renal failure. ( info)
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