Antitubular basement membrane antibodies in rapidly progressive poststreptococcal glomerulonephritis: Report of a case (original) (raw)
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...
Acta Nephrologica, 2006
We described a 33-year-old man with gross hematuria and acute renal failure, who had suffered from fever for 6 months, multiple cervical lymph node swelling, splenomegaly, left-sided pleural effusion. He also suffered from anemia, thrombocytopenia, hypergammaglobulinemia, and his serum interleukin-6 levels were markedly elevated. Antiglomerular basement membrane antibodies were positive in the patient's serum. Lymph node biopsy results were compatible with Castleman disease of "plasma cell" variant. Renal biopsy revealed cellular crescents in most of the glomeruli. Immunofluorescence studies showed strong deposition of IgG in a linear pattern along the glomerular basement membrane. Pathologic features were compatible with crescentic glomerulonephritis because of antiglomerular basement membrane disease. With intensive plasmapheresis and monthly chemotherapy (cyclophosphamide, oncovin, prednisone regimen), the patient experienced clinical and biochemical remission. Although autoimmune phenomenon had been described frequently in Castleman disease, to the best of our knowledge, this was the first report that the patient with rapid progressive glomerulonephritis mediated by antiglomerular basement membrane antibodies, which might be associated with Castleman disease.
Anti-glomerular basement membrane disease
Kidney International, 2003
A 47-year-old white man who worked as a professional musician presented to his general practitioner with a 3-week history of malaise, loss of appetite, slight weight loss, and dark urine. There was no history of respiratory symptoms and, in particular, no hemoptysis. He had had mild asthma for 10 years, which had been treated with inhaled bronchodilators. He was a lifelong nonsmoker and drank alcohol only occasionally. He had no family history of renal disease. The general practitioner detected proteinuria and hematuria, and blood tests showed an elevated serum creatinine. The patient was referred to our renal unit the same day. On arrival, he looked well and was afebrile with no rash or edema. Pulse was 80 beats/minute and regular, and blood pressure was 145/70 mm Hg. Clinical examination of the heart, lungs, abdomen, and nervous system was normal. Urinalysis showed 2ϩ blood, 3ϩ protein, dysmorphic red cells, and granular and red cell casts. Serum creatinine was 550 mol/L (6.3 mg/dL) and urea 21 mmol/L. Electrolytes were in the normal range, but albumin was reduced at 2.3 g/dL. Blood picture showed hemoglobin 10.7 g/dL; white blood cell count 9.6 ϫ 10 9 /L; and platelets 654 ϫ 10 9 /L. Oxygen saturation with the patient breathing room air was 94%, and a chest radiograph was normal. An enzyme-linked immunosorbent assay (ELISA) for antiglomerular basement membrane (anti-GBM) antibodies was
ISRN nephrology, 2013
To identify differences in treatment and outcome of various types of glomerulonephritis developing in the course of infections triggering antineutrophil cytoplasmic antibody (ANCA) formation, we analyzed published reports of 50 patients. Immunosuppressives were added to antibiotics in 22 of 23 patients with pauci-immune glomerulonephritis. Improvement was noted in 85% of 20 patients with information on outcomes. Death rate was 13%. Corticosteroids were added to antibiotics in about 50% of 19 patients with postinfectious glomerulonephritis. Improvement rate was 74%, and death rate was 26%. Two patients with mixed histological features were analyzed under both pauci-immune and post-infectious glomerulonephritis categories. In 9 patients with other renal histology, treatment consisted of antibiotics alone (7 patients), antibiotics plus immunosuppressives (1 patient), or immunosuppressives alone (1 patient). Improvement rate was 67%, permanent renal failure rate was 22%, and death rate ...
Immunopathologia Persa, 2016
Anti-neutrophil cytoplasmic antibody (ANCA)-associated crescentic glomerulonephritis is an important cause for rapidly progressing glomerulonephritis. It is generally classified under pauci-immune glomerulonephritis. However, 12%-18% of ANCA-associated crescentic glomerulonephritis show immune-complex deposits causing a diagnostic dilemma. We report 2 cases of ANCA-mediated glomerulonephritis with associated immune-complex deposits. First case is a 19-year-old female patient presented with fever and bilateral lower limb purpura since one day. Immunologic work-up was normal except positivity for cytoplasmic or c-ANCA by indirect immunofluorescence (IF). Kidney biopsy showed presence of segmental cellular crescent with fibrinoid necrosis. IF showed strong fine granular positivity for IgG, IgA, C3, C1q, kappa and lambda along the glomerular capillary walls. Second case is a 20-year-old male presented with low grade fever for last one month and vomiting for last two days. Immunologic work-up was unremarkable except positivity for cytoplasmic or c-ANCA by indirect IF. Kidney biopsy showed 14 glomeruli of which 8 glomeruli showed cellular crescents. IF for IgG, IgA, C3, kappa and lambda was done, which showed strong fine granular positivity along the glomerular capillary walls. Both the cases were treated with intravenous methylprednisolone and oral prednisone on a weaning regimen, and monthly therapy of intravenous cyclophosphamide. The maintenance phase consisted of mycophenolate mofetil and oral prednisone administrated in alternate-day low-dose regimen. Both the patients are on regular follow-up and are doing well.These immunecomplexes act synergistically with ANCA to cause more severe damage to the kidneys with a poorer outcome. Thus a prompt diagnosis and management of these patients is crucial.
Curēus, 2024
Anti-glomerular basement membrane (GBM) disease is a form of rapidly progressive glomerulonephritis with acute deterioration of kidney function. Atypical forms of this disease have been described which do not show positive serology for the classical anti-GBM antibody (Ab) but their presence on kidney biopsies. Furthermore, concomitantly any other separate glomerular pathology along with anti-GBM disease has been only rarely seen. A 40-year-old male patient presented with complaints of lower limb swelling and hematuria. Initial blood investigations revealed nephrotic range proteinuria and hypoalbuminemia. The patient underwent a renal biopsy. Initial reports showed the presence of "linear" deposits for immunoglobulin G (IgG) Ab and crescent formation in the majority of glomeruli. Treatment with plasmapheresis was initiated for the same. Electron microscopy, which later revealed subepithelial deposits raised suspicion of concomitant membranous nephropathy (MN). This finding was confirmed with a staining biopsy block with an anti-PLA2R Ab stain. Treatment was initiated to treat both glomerular pathologies, which very rarely present together and do not have standard guidelines for treatment. The patient responded to treatment with a reduction in serum creatinine values and did not require maintenance hemodialysis. There have been only a handful of documented cases, only in the form of a few case series that have described the presence of both anti-GBM disease and MN in the same kidney biopsy.