Case report: anti-glomerular basement membrane antibody disease with normal renal function

Published on Nov 4, 2015in BMC Nephrology1.913
· DOI :10.1186/S12882-015-0179-1
China Nagano7
Estimated H-index: 7
Yoshimitu Goto1
Estimated H-index: 1
+ 1 AuthorsYoshiyuki Kuroyanagi3
Estimated H-index: 3
Background Anti-glomerular basement membrane (GBM) antibody disease is a rare autoimmune disorder characterized by rapidly progressive glomerulonephritis caused by autoantibodies against the α3-chain of type IV collagen in the GBM.
#1Ricardo SilvariñoH-Index: 5
#2Oscar NoboaH-Index: 16
Last. Ricard CerveraH-Index: 103
view all 3 authors...
Basement membranes form an anatomic barrier that contains connective tissue. They are composed of type IV collagen, laminin and proteoglycans. Anti-basement membrane antibodies bind to the non-collagen site of the α3 chain of type IV collagen. A group of renal diseases, pulmonary diseases and perhaps others affecting different organs have long been associated with the presence of antibodies directed against glomerular basement membrane (GBM), alveolar basement membrane and tubular basement membr...
12 Citations
#1Thomas Hellmark (Lund University)H-Index: 29
#2Mårten Segelmark (Linköping University)H-Index: 53
Goodpasture's disease or anti-glomerular basement membrane disease (anti-GBM-disease) is included among immune complex small vessel vasculitides. The definition of anti-GBM disease is a vasculitis affecting glomerular capillaries, pulmonary capillaries, or both, with GBM deposition of anti-GBM autoantibodies. The disease is a prototype of autoimmune disease, where the patients develop autoantibodies that bind to the basement membranes and activate the classical pathway of the complement system, ...
70 CitationsSource
#1Allan Bayat (AU: Aarhus University)H-Index: 2
#2Konstantinos Kamperis (Aarhus University Hospital)H-Index: 17
Last. Troels Herlin (Aarhus University Hospital)H-Index: 37
view all 3 authors...
Antiglomerular basement membrane antibody disease (aGD) remains a very uncommon entity in the pediatric population, characterized by pulmonary hemorrhage, glomerulonephritis, and antibodies against the glomerular basement membrane. We herein describe the case of a 14-year-old girl with primary pulmonary symptoms diagnosed with aGD. Furthermore, we review 23 cases described in English literature over a 25-year period. Our case is the fourth child that presented with only pulmonary symptoms and no...
19 CitationsSource
#1Sean R. Williamson (IU: Indiana University)H-Index: 30
#2Carrie L. Phillips (IU: Indiana University)H-Index: 25
Last. Corina Nailescu (Riley Hospital for Children)H-Index: 5
view all 4 authors...
Anti-glomerular basement membrane (anti-GBM) disease, which is extremely uncommon in children, is characterized by rapidly progressive glomerulonephritis (RPGN) and autoantibodies against GBM collagen. Pulmonary hemorrhage is the third component in Goodpasture Syndrome. Cigarette smoking and exposure to hydrocarbons have been linked to anti-GBM disease in adults, but such an association has not been established in children. We reviewed renal biopsy and autopsy specimens over 25 years from a majo...
25 CitationsSource
#1Nagasu Hajime (Kawasaki Medical School)H-Index: 1
#2Abe MichikoH-Index: 1
Last. Sakaguchi KatsuhikoH-Index: 1
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Goodpasture's syndrome is characterized by pulmonary hemorrhage, rapid progressive glomerulonephritis and the presence of anti-glomerular basement membrane (anti-GBM) antibodies. Here, we report a case of Goodpasture's syndrome that we treated with double filtration plasmapheresis (DFPP) combined with immunosuppression therapy. The patient was a 32-year-old man with the main complaints of low-grade fever, general fatigue and dyspnea. The clinical diagnosis was renal-pulmonary syndrome based on p...
12 CitationsSource
#1Zhao Cui (PKU: Peking University)H-Index: 25
#2Ming hui Zhao (PKU: Peking University)H-Index: 7
Last. Hongya Wang (PKU: Peking University)H-Index: 17
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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 lun...
36 CitationsSource
#1Zhao Cui (PKU: Peking University)H-Index: 25
#2Haibo Wang (PKU: Peking University)H-Index: 9
Last. Ming-Hui Zhao (PKU: Peking University)H-Index: 56
view all 3 authors...
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...
53 CitationsSource
#1Alan D. Salama (Imperial College London)H-Index: 57
#2Jeremy Levy (Imperial College London)H-Index: 32
Last. Charles D. Pusey (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: 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...
137 CitationsSource
Anti-glomerular basement membrane disease (anti-GBM) is a rare but well-characterized cause of glomerulonephritis. It is defined by the presence of autoantibodies directed at specific antigenic targets within the glomerular and/or pulmonary basement membrane. These antibodies bind to the α3 chain
128 CitationsSource
#1C AngH-Index: 1
#2Judy SavigeH-Index: 39
Last. R S SinclairH-Index: 1
view all 7 authors...
Background. This study compared the clinical and laboratory characteristics of patients with anti-glomerular basement membrane (GBM) disease and normal renal function, with those of patients with anti-GBM disease where there was renal impairment. Methods. The medical records of the 14 patients who had presented with anti-GBM disease to our hospital in the past 20 years were reviewed. Results. Five (36%) had a normal serum creatinine or creatinine clearance at presentation. Other features were ha...
39 CitationsSource
Cited By6
#1Dandan LiangH-Index: 2
#2Shaoshan Liang (NU: Nanjing University)H-Index: 14
Last. Caihong ZengH-Index: 35
view all 8 authors...
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 ...
6 CitationsSource
The respiratory system may be involved in all types of systemic vasculitis with varying significance and frequency. ANCA-associated vasculitis, including granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis, affects the respiratory tract more commonly than other vasculitis types. Granulomatosis with polyangiitis is always associated with upper or lower respiratory tract involvement. Pulmonary and ENT involvements are the hallmark feature o...
7 CitationsSource
#1Emily HoltH-Index: 1
#2Andrew GoughH-Index: 1
Last. Fahmid U. ChowdhuryH-Index: 18
view all 4 authors...
: A 30-year-old woman presented with lethargy, night sweats, and fever with raised inflammatory markers. Anti-neutrophil cytoplasmic antibody was negative. Abdominopelvic CT was unremarkable. Subsequently, she underwent FDG PET/CT showing globally enlarged kidneys with diffuse hypermetabolic activity within the renal parenchyma bilaterally. Renal biopsies showed morphologic features of an active necrotizing crescentic glomerulonephritis, which was confirmed clinically and treated. This case demo...
1 CitationsSource
#1Guillaume Dorval (Paris V: Paris Descartes University)H-Index: 5
#2Mathilde Lion (Paris V: Paris Descartes University)H-Index: 1
Last. Olivia Boyer (Paris V: Paris Descartes University)H-Index: 27
view all 7 authors...
Antiglomerular basement membrane glomerulonephritis (anti-GBM GN) is a rare autoimmune disease that is characterized by rapidly progressive glomerulonephritis that may be associated with pulmonary hemorrhage. Anti-GBM GN is caused by autoantibodies (classically type G immunoglobulin) directed against the α3 subunit of type IV collagen. Without any appropriate treatment, the disease is generally fulminant, and patient and kidney survival is poor. The current guidelines recommend the use of plasma...
6 CitationsSource
#1Guillaume Dorval (Paris V: Paris Descartes University)H-Index: 5
#2S. Guérin (Necker-Enfants Malades Hospital)H-Index: 1
Last. Olivia Boyer (Paris V: Paris Descartes University)H-Index: 27
view all 7 authors...
Resume La maladie des anticorps anti-membrane basale glomerulaire est une maladie auto-immune se manifestant par une glomerulonephrite rapidement progressive, associee ou non a une atteinte pulmonaire de type hemorragie intra-alveolaire (syndrome de Goodpasture). Elle est secondaire a la fixation d’un auto-anticorps de type immunoglobulines G (IgG) dirige contre la sous-unite α3 du collagene de type IV. Cette affection est rare chez l’adulte et extremement rare chez l’enfant (incidence totale es...
1 CitationsSource
#2Diana WarneckeH-Index: 1
Last. Sarah ElhadiH-Index: 1
view all 4 authors...
Goodpasture’s syndrome (GPS) remains a very rare disease entity in the pediatric population characterized by the presence of pulmonary hemorrhage and rapidly evolving glomerulonephritis. We hereby describe the case of a 2-year-old girl who presented with renal failure and was diagnosed with GPS. A brief review of the literature in regard to data on demographics, pathogenesis, clinical features, diagnosis, treatment, and prognosis for renal recovery is also provided.
2 CitationsSource