Antiglomerular basement membrane disease with normal renal function.

Published on Dec 1, 2007in Kidney International10.612
· DOI :10.1038/SJ.KI.5002525
Zhao Cui26
Estimated H-index: 26
(PKU: Peking University),
Ming hui Zhao7
Estimated H-index: 7
(PKU: Peking University)
+ 1 AuthorsHongya Wang20
Estimated H-index: 20
(PKU: Peking University)
Sources
Abstract
CASE PRESENTATION A 23-year-old man was referred to Peking University First Hospital with a 4-month history of intermittent hemoptysis. One month before admission, he experienced severe hemoptysis with cough and respiratory distress. His hemoglobin was 71.0 g/l, urinalysis revealed protein 1 +, and red blood cells 3-5/high-power field (HPF). Arterial blood gas analysis showed pH 7.385, PO 2 58 mm Hg, PCO 2 40 mm Hg, and SaO 2 88%. Chest radiograph showed diffusely parenchymal shadows in both lungs. He was diagnosed as having a pulmonary infection with respiratory failure type I and was treated with ceftazidime and erythromycin, but no improvement was obtained after 3 weeks. One week before admission, an antiglomerular basement membrane (GBM) antibody was detected positive by enzyme-linked immunosorbent assay (ELISA) using purified bovine α(IV)NC1 as solid-phase antigen. He was then referred to our hospital. The patient was a car repairman with a 5-year exposure to gasoline and diesel. He smoked 1-2 packs of cigarettes per day for 4 years. Physical examination revealed a well-nourished man with no edema or rash. The temperature was 36.8°C, blood pressure 130/85 mm Hg, and pulse 90/min. The heart rate and rhythm was regular. Moist rales could be heard in both lungs. The abdomen was soft and non-tender without organomegaly. Laboratory data on admission were as follows: white blood cells 12.8 x 10 9 /l (normal range, 3.5-9.5 x 10 9 /l), hemoglobin 62.0g/l (137-179g/l), platelet 178 x 10 9 /l (100-300 x 10 9 /l). Hepatic function was normal. Serum creatinine was 94.0/μmol/l (44-133 μmol/l) and blood urea nitrogen was 6.3 mmol/l (1.8-7.1 mmol/l). Electrolytes were in the normal range. Serum albumin was 39.6 g/l (35-50 g/l). Urinalysis revealed red blood cells 3-5/HPF (0-3/HPF) and dysmorphic red cells and the 24-h urine protein was 0.87g (<0.15g/24h). Erythrocyte sedimentation rate was 14 mm/1 h (<15mm/1 h). C-reactive protein, rheumatoid factor, antineutrophil cytoplasmic antibodies, and antinuclear antibodies were all negative. Anti-GBM antibodies were positive at 23% tested by ELISA as described above (normal range, <13%), with a titer of 1:400. Although there was a broad differential, including small vessel vasculitis, systemic lupus erythematosus, pulmonary infection, tuberculosis, and heart failure, the clinical presentation and the laboratory workup were most compatible with a diagnosis of anti-GBM disease with Goodpasture's syndrome. After admission, a renal biopsy was performed.
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3 Authors (Haiyan Wang)
References28
Newest
#2Haiyan Wang (PKU: Peking University)H-Index: 55
Autoimmunity to glomerular basement membrane (GBM) could induce Goodpasture disease, and natural autoantibodies against GBM in the sera of normal individuals were not reported. The aim of the study was to identify and characterize natural autoantibodies against GBM in normal human sera. Natural anti-GBM autoantibodies were purified from the sera of five healthy persons by affinity chromatography, using purified bovine α (IV)non-collagenous (NC1) as solid-phase ligands. Antigen specificity, immun...
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#1Zhao Cui (PKU: Peking University)H-Index: 1
#2Ming-Hui Zhao (PKU: Peking University)H-Index: 1
Anti-glomerular basement membrane (GBM) antibody-mediated diseases are characterized by the binding of autoantibodies to GBM, leading to rapidly progressive glomerulonephritis that often results in irreversible loss of renal function. The nephrotoxic potential of anti-GBM antibodies has been demonstrated in animal experiments. We questioned whether high avidity leads to persistent deposition of anti-GBM antibodies, thereby perpetuating inflammation and renal damage. To address the hypothesis, se...
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#1Zhao Cui (PKU: Peking University)H-Index: 26
#2Ming-hui ZhaoH-Index: 1
Last. Hai-yan WangH-Index: 2
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Background: Patients with anti-glomerular basement membrane (GBM) disease were predominantly reported in Caucasian population and reports from Chinese were lacking. The general pict
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#1Mårten Segelmark (Lund University)H-Index: 55
#2Thomas HellmarkH-Index: 30
Last. Jörgen WieslanderH-Index: 52
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Background: The nephrotoxic potential of anti-glomerular-basement-membrane (GBM) antibodies has been demonstrated in numerous animal experiments. However, it is not known to what extent the properties of circulating anti-GBM antibodies in human disease reflect the severity of the disease and predict the outcome. Methods: Clinical data were collected for 79 Swedish patients for whom a positive result had previously been obtained with anti-GBM ELISA. In stored sera from the patients, we measured a...
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#1Yu Yan (PKU: Peking University)H-Index: 2
#2Zhao CuiH-Index: 1
Last. Ming-hui ZhaoH-Index: 1
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Objective: It was reported that major subclasses of anti-golmerular basement membrane (GBM) antibody were IgG1 and IgG4. The IgG1 subclass was mainly found in male patients and IgG4 subclass in female patients with anti-GBM disease. This study investigates the distribution of anti-GBM IgG subclasses and their association with clinical characteristics. Methods: Sera from 50 patients diagnosed as anti-GBM disease during 1991-2003 were collected. The anti-GBM antibodies with IgG1, IgG2, IgG3, and I...
#1Rj FalkH-Index: 1
#1T. Dougan (Hammersmith Hospital)H-Index: 1
#2Jeremy Levy (Hammersmith Hospital)H-Index: 34
Last. Charles D. Pusey (Hammersmith Hospital)H-Index: 92
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Goodpasture’s disease is characterized by the binding of IgG autoantibodies to the glomerular basement membrane, leading to glomerular inflammation. The autoantigen has been identified as the noncollagenous domain of the α3 chain of type IV collagen (α3(IV)NC1). We have used the IAsys resonant mirror biosensor to analyse the extent and affinity of binding of anti-GBM antibodies from sera of patients to purified α3(IV) NC1. α3(IV) NC1 monomers were immobilized to a carboxylate cuvette, with the s...
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#1Alan D. Salama (ICL: Imperial College London)H-Index: 56
#2Jeremy Levy (ICL: Imperial College London)H-Index: 34
Last. Charles D. Pusey (ICL: Imperial College London)H-Index: 92
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Correspondence to: Dr Alan D Salama, Laboratory of Immunogenetics and Transplantation, Brigham and Women’s Hospital, 75 Francis Street, Boston, MA 02115, USA (e-mail: asalama@rics.bwh.harvard.edu) Goodpasture’s disease In 1919, Goodpasture was attempting to define the specific pathological features of influenza infection in diseased lungs, and reported the autopsy findings in two patients who had died at the height of the 1918–19 pandemic. The finding of hyaline membranes overlying dilated alveo...
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#1Jeremy Levy (ICL: Imperial College London)H-Index: 34
#2A. N. TurnerH-Index: 2
Last. Charles D. Pusey (Edin.: University of Edinburgh)H-Index: 92
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Background: Anti-glomerular basement membrane (GBM) antibody disease is an autoantibody-mediated disorder that usually presents as rapidly progressive glomerulonephritis, often with pulmonary hemorrhage (the Goodpasture syndrome). It is reported that patients with severe renal failure do not generally recover renal function. Objective: To examine the long-term outcome of severe anti-GBM antibody disease. Design: Retrospective review of patients treated for confirmed anti-GBM antibody disease ove...
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#1Kailash K. Jindal (Dal: Dalhousie University)H-Index: 12
Management of idiopathic crescentic and diffuse proliferative glomerulonephritis: Evidence-based recommendations. Idiopathic crescentic glomerulonephritis (GN) often presents with a rapid loss of renal function and pathology showing extensive crescent formation. The disease is caused by different immunopathogenetic mechanisms, pauci-immune, often antineutrophil cytoplasmic antibody (ANCA)-positive microvasculitis, anti-glomerular basement membrane (GBM) antibody disease, and immune complex forma...
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Cited By36
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#1Guming Zou (China-Japan Friendship Hospital)H-Index: 7
#2Hai-Tao Lu (China-Japan Friendship Hospital)H-Index: 2
Last. Wenge Li (China-Japan Friendship Hospital)H-Index: 11
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BACKGROUND Anti-glomerular basement membrane (anti-GBM) disease is a rare autoimmune condition responsible for rapidly progressive glomerulonephritis. This disease is usually mediated by IgG autoantibodies against the noncollagenous domain of the α3(IV) collagen chain. In rare cases, IgA or IgM anti-GBM antibodies are involved. This raises the question of whether there are different types of antibody-mediated anti-GBM disease at the same time. CASE REPORT A 37-year-old woman with anti-GBM diseas...
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Atypical anti-glomerular basement membrane (GBM) disease, which is characterized by low levels of or negativity for anti-GBM antibodies in circulation but positivity in the kidney, has been recognized in this decade. However, a therapeutic strategy has not been established to date because its outcome is better than that of classic anti-GBM disease. This case report and literature review highlight atypical anti-GBM disease in infection-related rapidly progressive glomerulonephritis. A 72-year-old...
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#1Inga Koneczny (Medical University of Vienna)H-Index: 13
Abstract Antibodies of IgG4 subclass are exceptional players of the immune system, as they are considered to be immunologically inert and functionally monovalent, and as such may be part of classical tolerance mechanisms. IgG4 antibodies are found in a range of different diseases, including IgG4-related diseases, allergy, cancer, rheumatoid arthritis, helminth infection and IgG4 autoimmune diseases, where they may be pathogenic or protective. IgG4 autoimmune diseases are an emerging new group of...
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#1Cong-Rong ShenH-Index: 2
#2Xiao-yu JiaH-Index: 12
Last. Ming-Hui ZhaoH-Index: 56
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Background: Atypical cases of anti-glomerular basement membrane (GBM) disease had absent circulating antibodies but linear IgG deposits along GBM in the kidneys. Herein, we reported the clinical-pathological features and outcome of these rare cases. Methods: Linear IgG deposit along GBM were examined by immunofluorescence on renal specimens, with exclusion of diabetic kidney disease. Circulating anti-GBM antibodies were tested by commercial ELISA assay. Clinical, pathological and follow-up data ...
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#1Mårten Segelmark (Lund University)H-Index: 55
#2Thomas Hellmark (Lund University)H-Index: 30
Most patients with anti-glomerular basement membrane (anti-GBM) disease present with rapidly progressive glomerulonephritis with or without pulmonary haemorrhage; however, there are several variants and vigilance is necessary to make a correct diagnosis. Such variants include overlap with anti-neutrophil cytoplasm antibodies-associated vasculitis and membranous nephropathy as well as anti-GBM occurring de novo after renal transplantation. Moreover, patients can present with isolated pulmonary ha...
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#1Maria Prendecki (ICL: Imperial College London)H-Index: 12
#2Charles D. Pusey (ICL: Imperial College London)H-Index: 92
Summary Anti-glomerular basement membrane (GBM) disease is a rare autoimmune vasculitis characterised by antibodies directed against the non collagenous (NC1) domain of the α3 chain of type 4 collagen (α3(IV)NC1). Clinical features are typically of a rapidly progressive glomerulonephritis (RPGN) with or without pulmonary haemorrhage. Treatment aims to rapidly remove circulating autoantibodies with plasma exchange and prevent further antibody production and suppress inflammation using immunosuppr...
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#1Dandan LiangH-Index: 2
#2Shaoshan Liang (NU: Nanjing University)H-Index: 16
Last. Caihong ZengH-Index: 36
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Aim To explore the clinicopathological characteristics of patients with anti-GBM antibody-negative anti-GBM disease. Methods The clinical and renal pathological findings were retrospectively studied in 19 patients. All patients met the following inclusion criteria: linear GBM IgG deposition on immunofluorescence(IF); and lack of serum anti-GBM antibodies by ELISA and indirect immunofluorescence assay. Results There were 17 male and two female patients, with a median age of 36 years (range 15–61 ...
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#1Stephen P. McAdoo (ICL: Imperial College London)H-Index: 22
#2Charles D. Pusey (ICL: Imperial College London)H-Index: 92
Antiglomerular basement membrane (anti-GBM) disease is a rare but life-threatening autoimmune vasculitis that is characterized by the development of pathogenic autoantibodies to type IV collagen antigens expressed in the glomerular and alveolar basement membranes. Once deposited in tissue, these autoantibodies incite a local capillaritis which manifests as rapidly progressive glomerulonephritis (GN) in 80 to 90% of patients, and with concurrent alveolar hemorrhage in ∼50%. A small proportion of ...
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#1Vadim Pedchenko (VUMC: Vanderbilt University Medical Center)H-Index: 21
#2A. Richard Kitching (Monash University)H-Index: 57
Last. Billy G. Hudson (VUMC: Vanderbilt University Medical Center)H-Index: 78
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Abstract Goodpasture's (GP) disease is an autoimmune disorder characterized by the deposition of pathogenic autoantibodies in basement membranes of kidney and lung eliciting rapidly progressive glomerulonephritis and pulmonary hemorrhage. The principal autoantigen is the α345 network of collagen IV, which expression is restricted to target tissues. Recent discoveries include a key role of chloride and bromide for network assembly, a novel posttranslational modification of the antigen, a sulfilim...
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#2Helena PintoH-Index: 4
Last. Carol MarinhoH-Index: 6
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The antiglomerular basement membrane (anti-GBM) antibody disease is marked by the presence of specific antibodies against the non-collagenous domain of the type IV collagen’s α3 chain. We describe a case of a 24-year-old Caucasian man, who may have had an atypical presentation of anti-GBM (slow progressive renal insufficiency, massive proteinuria and no detectable circulating anti-GBM antibody). The patient was treated with steroids and cyclophosphamide. This approach failed to attenuate the dis...
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