Nephrology Book

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Goodpasture's Syndrome

Aka: Goodpasture's Syndrome
  1. Pathophysiology
    1. Antibody to renal and alveolar basement membrane
      1. Intraalveolar Hemorrhage
      2. Glomerulonephritis
  2. Symptoms and Signs
    1. Pulmonary
      1. Hemoptysis
      2. Cough
      3. Exertional Dyspnea
      4. Rales and rhonchi on Lung Exam
    2. Renal
      1. Glomerulonephritis
  3. Labs
    1. Basement Membrane Antibody (anti-GBM) present
    2. Complete Blood Count
      1. Normochromic Anemia
    3. Gastric aspirate or Bloody Sputum exam
      1. Siderophages
    4. Urinalysis
      1. Consistent with Glomerulonephritis
  4. Imaging: Chest XRay
    1. Bilateral perihilar patchy infiltrates
  5. Diagnosis
    1. Renal Biopsy
      1. Linear Immunofluorescence
    2. Lung Biopsy
      1. Linear IgG
  6. Course
    1. Rapidly Progressive Glomerulonephritis is typical
    2. Renal Failure usually ensues
  7. Management
    1. Plasma exchange
    2. Glucocorticoids IV for severe lung Hemorrhage

Anti-Glomerular Basement Membrane Disease (C0403529)

Definition (NCI) An autoimmune disorder characterized by pulmonary hemorrhage and glomerulonephritis. It is a hypersensitivity reaction resulting in the formation of antibodies against the pulmonary alveoli and the basement membrane of the glomeruli.
Definition (NCI) An autoimmune disorder characterized by the production of autoantibodies against type IV collagen of the glomerular basement membrane of the kidney. In the majority of patients the immune reaction also extends to the alveolar capillary membrane of the lungs. The latter cases are referred to as Goodpasture Syndrome.
Definition (CSP) combination of pulmonary hemorrhage and glomerulonephritis; known also as the lung purpura glomerulonephritis complex; considered by some to be a form of hypersensitivity reaction.
Definition (MSH) An autoimmune disease of the KIDNEY and the LUNG. It is characterized by the presence of circulating autoantibodies targeting the epitopes in the non-collagenous domains of COLLAGEN TYPE IV in the basement membranes of kidney glomeruli (KIDNEY GLOMERULUS) and lung alveoli (PULMONARY ALVEOLI), and the subsequent destruction of these basement membranes. Clinical features include pulmonary alveolar hemorrhage and glomerulonephritis.
Concepts Disease or Syndrome (T047)
MSH D019867
ICD9 446.21
ICD10 M31.0
SnomedCT 50581000, 236506009, 195351002, 155445002, 266323005
English GOODPASTURE'S SYNDROME, Anti GBM Disease, Anti GBM dis - Anti GBM dis, Anti GBM disease, Anti-GBM Disease, Goodpasture Syndrome, Goodpasture's Syndrome, Lung Purpura with Nephritis, Syndrome, Goodpasture's, Goodpastures Syndrome, Syndrome, Goodpasture, GOODPASTURE SYNDROME, ANTI GLOMERULAR BASEMENT MEMBRANE DIS, ANTIGBM DISEASE, ANTIGLOMERULAR BASEMENT MEMBRANE DISEASE, ANTI GBM DIS, Goodpasture's syndrome (diagnosis), Syndrome Good pasture, Syndrome Good postures, Syndrome Goodpasture's, Goodpasture syndrome, Goodpasture disease, Anti-Glomerular Basement Membrane Disease [Disease/Finding], good pastures syndrome, pulmonary renal syndromes, goodpastures syndrome, good posture syndrome, pneumorenal syndrome, goodpasture's syndrome, anti gbm disease, goodpasture's disease, goodpasture syndrome, good pasture syndrome, goodpastures's syndrome, Anti Glomerular Basement Membrane Disease, Anti-Glomerular Basement Membrane Disease, Pulmonary-renal syndrome, Lung purpura with nephritis syndrome, Goodpasture's syndrome, Anti-GBM nephritis with pulmonary hemorrhage, Pulmonary hemorrhage with glomerulonephritis, Pulmonary hemosiderosis with glomerulonephritis, Anti GBM - Antiglomerular basement membrane disease, Anti-GBM nephritis with pulmonary haemorrhage, Antiglomerular basement membrane disease, Goodpasture's disease, Pulmonary haemorrhage with glomerulonephritis, Pulmonary haemosiderosis with glomerulonephritis, Goodpasture's disease (disorder), Goodpasture's syndrome (disorder), Haemorrhagic pneumonia AND glomerulonephritis, Hemorrhagic pneumonia AND glomerulonephritis, Goodpasture, pulmonary; renal syndrome (Goodpasture), syndrome; pulmonary-renal (Goodpasture), Hemorrhagic pneumonia and glomerulonephritis, Lung Purpura with Glomerulonephritis, lung purpura with nephritis
Portuguese SINDROME DE GOODPASTURE, Síndrome de Goodpasture, Doença de Anticorpo Antimembrana Basal Glomerular, Doença de Anticorpo (Anti-GBM) Antiglomerular a Membrana Basal, Doença Glomerular de Anticorpo Antimembrana Basal, Doença Anti-GBM, Doença Antimembrana Basal Glomerular
Spanish SINDROME DE GOODPASTURE, Síndrome de Goodpasture, neumonía hemorrágica y glomerulonefritis, enfermedad antimembrana basal glomerular, enfermedad de Goodpasture (trastorno), enfermedad de Goodpasture, glomerulonefritis y neumonía hemorrágica, hemorragia pulmonar con glomerulonefritis, hemosiderosis pulmonar con glomerulonefritis, nefritis con anticuerpos anti - membrana basal glomerular con hemorragia pulmonar, púrpura pulmonar con síndrome nefrítico, síndrome de Goodpasture (trastorno), síndrome de Goodpasture, Enfermedad Anti-GBM, Enfermedad por Anticuerpos Antimembrana Basal Glomerular
Italian Sindrome di Goodpasture, Malattia degli anticorpi anti-membrana basale glomerulare
Dutch syndroom van Goodpasture, pulmonaal; renaal syndroom (Goodpasture), syndroom; pulmonaal-renaal (Goodpasture), Goodpasture-syndroom, Anti-GBM-glomerulonefritis, Anti-glomerulaire basale-membraanglomerulonefritis, C-membraan-glomerulonefritis, Glomerulonefritis, C-membraan-, Glomerulonefritis, anti-GBM-
German Goodpasture-Syndrom, GOODPASTURESYNDROM, ANTIGBM KRANKHEIT, Goodpasture Syndrom, Anti-GBM-Krankheit, Antiglomeruläre Basalmembrankrankheit
Russian ГУДПАСЧЕРА СИНДРОМ, ЛЕГОЧНО-ПОЧЕЧНЫЙ НАСЛЕДСТВЕННЫЙ СИНДРОМ, ЛЕГОЧНАЯ ПУРПУРА ГЕМОРРАГИЧЕСКАЯ С НЕФРИТОМ, LEGOCHNO-POCHECHNYI NASLEDSTVENNYI SINDROM, AUTOIMMUNNYI GLOMERULONEFRIT, GLOMERULONEFRIT S ANTITELAMI K BAZAL'NOI MEMBRANE, LEGOCHNAIA PURPURA GEMORRAGICHESKAIA S NEFRITOM, GLOMERULONEFRIT AUTOIMMUNNYI, GUDPASCHERA SINDROM, АУТОИММУННЫЙ ГЛОМЕРУЛОНЕФРИТ, ГЛОМЕРУЛОНЕФРИТ АУТОИММУННЫЙ, ГЛОМЕРУЛОНЕФРИТ С АНТИТЕЛАМИ К БАЗАЛЬНОЙ МЕМБРАНЕ
French Syndrome de Goodpasture, SYNDROME DE GOODPASTURE, Glomérulonéphrite extracapillaire par anticorps antimembrane basale glomérulaire, Maladie avec anticorps antimembrane basale glomérulaire, Maladie des anticorps antimembrane basale glomérulaire, Maladie par auto-anticorps anti-membrane basale glomérulaire, Maladie à anticorps anti-GBM, Maladie à anticorps anti-membrane basale, Maladie à anticorps antimembrane basale glomérulaire
Swedish Anti-GBM-sjukdom
Japanese グッドパスチャーショウコウグン, Goodpasture症候群, 腎炎合併肺性紫斑病, 抗糸球体基底膜抗体症, グッドパスチャー症候群, 肺腎症候群, 抗糸球体基底膜抗体病, 抗GBM抗体症
Czech antirenální glomerulonefritida, autoimunitní glomerulonefritida, anti-GBM nemoc, Goodpastureův syndrom
Finnish Tyvikalvovasta-aineglomerulonefriitti
Polish Choroba z przeciwciałami przeciwko kłębuszkom nerkowym, Choroba przeciw błonie podstawnej kłębuszków nerkowych, Choroba anty-GBM
Hungarian Goodpasture-syndroma, Goodpasture syndroma
Norwegian Anti-GBM-sykdom, Goodpastures syndrom
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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