Double positive Anti-GBM and ANCA-associated glomerulonephritis – A case report (original) (raw)
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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.
Sequential development of anti-GBM nephritis and ANCA-associated pauci-immune glomerulonephritis
American Journal of Kidney Diseases, 1999
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 pauciimmune 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.
Crescentic glomerulonephritis with dual positive anti-GBM and C-ANCA/PR3 antibodies
Clinical Nephrology – Case Studies, 2016
Antiglomerular basement membrane (anti-GBM) antibodies are more often accompanied by myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) than by proteinase 3 antineutrophil cytoplasmic antibody (PR3-ANCA). Both disease processes can affect the kidneys and/ or the lungs. Patients with dual positive disease may have an atypical presentation which may delay diagnosis and treatment. Here we report a case of crescentic glomerulonephritis associated with positive PR3-ANCA and anti-GBM antibodies who underwent both lung and kidney biopsies.
Bulletin of the National Research Centre
Background Anti-glomerular basement membrane (GBM) disease and ANCA-associated vasculitis (AAV) diseases are rare. It is associated with variable renal manifestations and increased mortality, thus requiring early aggressive treatment to minimize adverse outcomes and improve prognosis. Case presentation We present the case of a male patient with 1-month onset of asthenia, adynamia, oliguria, and weight loss. Initial laboratory findings were indicative of severe kidney dysfunction. The urinalysis showed active sediment, but the urinary tract ultrasound was unaltered. As these findings were consistent with rapidly progressive glomerulonephritis, he received steroid pulses, and given the severity of the condition, renal replacement therapy was initiated. Other diagnostic tests revealed MPO-ANCA antibody levels of 26 mg/dl, pANCAs 1/320, and anti-GBM of 8 mg/dl. Kidney biopsy evidenced necrotizing glomerulonephritis with extracapillary proliferation in 90% of the glomeruli. The patient r...
American Journal of Kidney Diseases, 2005
Background: In a substantial proportion of patients with crescentic glomerulonephritis (CGN), both antiglomerular basement membrane (GBM) antibodies and antineutrophil cytoplasmic antibodies (ANCAs) with specificity for myeloperoxidase (MPO-ANCA) are detected. In the present study, we questioned whether histological and clinical features of patients with both ANCA and anti-GBM antibodies differ from those of patients with either ANCA or anti-GBM alone. Methods: We reviewed the Limburg renal biopsy registry (1978 to 2003; n ؍ 1,373) for cases of CGN. The presence of linear fluorescence on renal biopsy and the presence of ANCA and/or anti-GBM antibodies were measured. Subsequently, we assessed patient characteristics and follow-up and compared histological findings among the different groups. Results: We identified 46 MPO-ANCA-positive, 10 doublepositive, and 13 anti-GBM-positive patients. Mean ages were 63, 64, and 52 years (P ؍ 0.04), and serum creatinine levels were 5.0, 10.3, and 9.6 mg/dL (445, 910, and 850 mol/L), respectively (P ؍ 0.01). Granulomatous periglomerular inflammation was found in either MPO-ANCA-or double-positive patients, but not in anti-GBM-positive patients with CGN without MPO-ANCAs. Patient survival among the 3 groups was different, although not statistically significant (log rank P ؍ 0.17, with 75%, 79%, and 100% alive at 1 year, respectively). Renal survival analysis showed significant differences among the 3 groups (P ؍ 0.04, with 65%, 10%, and 15% off dialysis therapy at 1 year, respectively). Conclusion: In patients with both anti-GBM antibodies and MPO-ANCAs, histological findings differ from those of patients with anti-GBM antibodies only. However, renal survival in these patients is not better than that in anti-GBM-positive patients and is worse compared with patients with MPO-ANCAs only. Am J Kidney Dis 46: 253-262.
Glomerular Diseases
Introduction: Anti-glomerular basement membrane (anti-GBM) disease is characterized by rapidly progressive glomerular nephritis with or without pulmonary hemorrhage with disease severity correlating with antibody titer. Following treatment, relapse is rare but has been reported in the literature. The objective of this study was to assess for clinical, serologic, and histologic differences associated with disease relapse in patients with anti-GBM disease. Methods: Patients seen at our facility between 1997 and 2017 were screened for anti-GBM disease by ICD 9/10 codes. They were included if the diagnosis was confirmed by a board-certified rheumatologist or nephrologist and had positive antibodies and/or biopsy results consistent with anti-GBM disease. Relapsing disease was defined as recurrence of pulmonary or renal manifestations after achieving remission following the initial presentation. All charts were reviewed for baseline demographics, clinical manifestations, and antibody posi...
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.
American Journal of Kidney Diseases, 2000
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. 2000 by the National Kidney Foundation, Inc. INDEX WORDS: Anti-glomerular basement membrane antibody (anti-GBM); glomerular basement membrane (GBM); Goodpasture's syndrome; hemodialysis (HD); antineutrophil cytoplasmic antibody (ANCA); myeloperoxidase (MPO).
Journal of Nephropathology
Anti-glomerular basement membrane (anti-GBM) nephritis is uncommon glomerular disease caused by autoantibodies targeting the capillary beds of the kidney. The clinical presentation of the disease is a variable nephritic syndrome, rapidly progressing to glomerulonephritis. Treatment outcomes are dependent on predictors at first diagnosis. We presented a case of 58-year-old man who did not have underlying disease presented with marked abdominal distension and acute kidney injury. He had no evidence of chronic renal disease before admission however, laboratory test showed microscopic haematuria (RBC 30-50 per high-powered field), proteinuria (2.9 g/d), and renal failure (serum creatinine 610 µmol/L) compatible with rapidly progressive glomerulonephritis; hence, a renal biopsy was conducted. The pathology showed 100% crescentic glomerulonephritis with IgG deposits in a linear pattern at the GBM. The initial serum anti-GBM titre was 105.59 RU/mL. This patient had poor renal prognosis fac...