Antitubular basement membrane antibodies in rapidly progressive poststreptococcal glomerulonephritis: Report of a case (original) (raw)
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Kidney International, 2007
A 49-year-old African-American female presented with acute renal failure. Her serum creatinine had increased from 0.8 to 2.8 mg/dl over 11 weeks. She had a 1-month history of anorexia, 20 lb weight loss, nausea, and vague abdominal discomfort. An outpatient evaluation included a renal ultrasound and magnetic resonance imaging of the abdomen, which were normal. Her past medical history was significant for a 12-year history of hypertension. There was no recent non-steroidal anti-inflammatory drug usage or radiocontrast study. Her medications included losartan 100 mg daily, amlodipine 2.5 mg daily, hydrochlorothiazide 25 mg daily, and potassium chloride 20 mEq daily.
Therapeutic Apheresis and Dialysis, 2009
Antineutrophil cytoplasmic antibodies (ANCA) and antibodies against glomerular basement membrane (anti-GBM) rarely coexist. Both antibodies may be associated with rapidly progressive glomerulonephritis and pulmonary hemorrhage. We describe the clinical, serological and histological features of our patients with dual antibodies. From 1977 to 2008, 48 patients with anti-GBM antibody-associated renal disease were observed. Eight out of the 30 tested patients (26.7%), all females, had positive myeloperoxidase (MPO)-ANCA coexistent with anti-GBM antibodies. The patients' mean age was 63.4 +/- 7.8 years. Five presented with pulmonary-renal syndrome, all but one were dialysis-dependent on admission. They had constitutional symptoms and different organ involvement. The kidney biopsies revealed intense linear staining for immunoglobulin G and C3 along the glomerular and distal tubular basement membrane associated with irregular diffuse or focal extracapillary crescentic glomerulonephritis with necrosis of varying extent. Lesions of varying ages were characteristically expressed. Seven patients were treated with methylprednisolone and plasma exchange, four with cyclophosphamide, and one with intravenous immunoglobulin. After 28-74 months, there were three dialysis-dependent survivors and one patient with stable chronic renal disease. Two clinical relapses with pulmonary involvement and MPO-ANCA positivity without anti-GBM antibodies occurred in two dialysis-dependent patients. In summary, screening for ANCA and anti-GBM antibodies should be undertaken in patients with clinical signs of systemic vasculitis. In dialysis-dependent patients, the goal of treatment is to limit the damage of other involved organs and not to preserve renal function. Careful follow-up is necessary due to the relapsing nature of the ANCA component of the disease.
A Case of Anti-glomerular Basement Disease Without Pulmonary Involvement
Cureus, 2019
Anti-glomerular basement membrane disease is a rare but classic example of an antibody-mediated disease. The scale of injury that it entails depends on the site where the antibodies are deposited, with some patients presenting with a composite of pulmonary and renal damage. In other scenarios, the renal system is the main site of affliction with patients deteriorating to a status of acute renal failure within days of diagnosis. Due to the paucity of its incidence, we present our findings of anti-glomerular basement disease with pulmonary sparing. Herein, we also review the array of different physical findings, different forms of perpetrating antibodies, the diagnostic tools at our disposal, and the treatment modalities utilized to prevent catastrophic tissue injuries.
American Journal of Kidney Diseases, 2014
Autoantibodies against a constituent of the glomerular basement membrane (GBM), the α3-chain of type IV collagen, can cause both rapidly progressive glomerulonephritis and alveolar hemorrhage, referred to as anti-GBM disease or Goodpasture´s disease. Anti-GBM antibodies are generally of immunoglobulin G subclass 1 (IgG1) and can in most cases readily be detected in the circulation using enzyme linked immunosorbent assays (ELISA). Here we report 4 cases where anti-GBM ELISA yielded negative or borderline results despite lifethreatening disease. All four patients were positive in IgG4 anti-GBM ELISA and all were ANCA negative. All cases were confirmed with kidney biopsy. Two of the patients exhibited higher results in anti-GBM ELISA when using a non-denaturing coating buffer. All four were young women with severe alveolar hemorrhage and favorable renal outcome suggesting that patients with predominance of IgG4 autoantibodies may constitute a distinct subgroup of anti-GBM disease. We conclude that patients with idiopathic alveolar hemorrhage can have anti-GBM disease detected only by IgG subclass specific tests or by kidney biopsy. * Sample sent to the reference laboratory at the time of the second kidney biopsy **Normal ranges in the assays are: total IgG <10; IgG1 <3; IgG2<8; IgG3 <27; IgG4<7 ELISA units. *** PLEX = plasma exchange, CYC iv = inter mitten intravenous cyclophosphamide, pred = prednisolone
2010
A 61-year-old Japanese woman with rapidly progressive glomerulonephritis exhibited both anti-glomerular basement membrane (GBM) antibodies (920 EU) and myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA; 66 EU). Multiple plasma exchanges with fresh frozen plasma preceded by 500 mg/day intravenous methylprednisolone and 30 mg/day oral prednisolone decreased anti-GBM antibody and MPO-ANCA antibody titers to 106 EU and below 10 EU (normal ranges), respectively. Thrombotic thrombocytopenic purpura (TTP) manifests itself as a moderate decrease in the activity of disintegrin-like and metalloproteinase with thrombospondin type 1 motif, 13 (ADAMTS13) protein levels to 35% of normal; ADAMTS13 deficiency is only symptomatic when levels are less than 50%. This patient's disease was resistant to extensive plasma exchange, leading to her death from respiratory distress and perforation of the alimentary tract secondary to cytomegalovirus infection. Autopsy demonstrated severe crescentic glomerulonephritis associated with linear IgG, IgA, IgM, and C3 deposits along the glomerular capillary walls. This case is an uncommon example of combined double antibody-positive crescentic glomerulonephritis and refractory TTP.
Nefrología : publicación oficial de la Sociedad Española Nefrologia
Anti-basement membrane antibody mediated disease is an unfrequent entity but with a high mortality and morbidity. We present a revision of 32 patients diagnosed of anti-basement membrane antibody mediated disease between 1983 and 1997, and their evolution at one year of the diagnosis. The clinical pattern of presentation was as a Goodpasture's syndrome (glomerulonephritis and lung haemorrhage) in 15 patients and glomerulonephritis without lung involvement in 17. We reviewed retrospectively the features at the clinical presentation, the different treatments, and the delay of the starting of it since the beginning of the symptoms, in order to evaluate a prognosis dats of the disease. After the retrospective study we deduce that anti-basement membrane antibody mediated disease has a high mortality although the different regimes of treatment applied (25%), and the need of renal replacement therapy at one year of diagnosis is also high (70.8%). The renal survival at one year of the d...