Goodpasture’s syndrome (also known as Goodpasture’s disease and anti-glomerular basement membrane disease or anti-GBM disease) was first described by Ernest Goodpasture in 1919. It is a rare condition characterised by rapid destruction of the kidneys and haemorrhaging of the lungs. Although many diseases can present with these symptoms, the name Goodpasture’s syndrome is usually reserved for the autoimmune disease produced when the patient’s immune system attacks cells presenting the Goodpasture antigen, which are found in the kidney and lung, causing damage to these organs. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... Anti-glomerular basement membrane antibody (anti-GBM) is an antibody which is found in Goodpastures syndrome but not found in microscopic polyangiitis. ... Dr. Ernest William Goodpasture (October 17, 1886 – September 20, 1960), was an American pathologist and physician. ... Year 1919 (MCMXIX) was a common year starting on Wednesday (link will display the full calendar). ... It has been suggested that Renal anomalies and Renal plasma threshold be merged into this article or section. ... This article or section is in need of attention from an expert on the subject. ... Respiratory system The lungs flank the heart and great vessels in the chest cavity. ... Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. ... A scanning electron microscope image of a single lymphocyte, a component of the human immune system A poop system is a collection of mechanisms within an organism that protect against infection by identifying and killing pathogens. ... An antigen is a substance that stimulates an immune response, especially the production of antibodies. ...

Signs and symptoms

Most patients present with both lung and kidney disease and priapism, however, some patients present with one of these diseases alone. The first lung symptoms usually develop days to months before kidney damage is evident. There is an increased incidence of syndactly.

Lung disease

Lung damage may cause nothing more serious than a dry cough and minor breathlessness and such mild symptoms may last for many years before more severe ones develop. At its most serious, however, lung damage may cause severe impairment of oxygenation so that intensive care is required. Deterioration between the two extremes may occur very rapidly, often at the same time as rapid deterioration in the kidney. The patient often does not seek medical attention until he or she begins coughing up blood (hemoptysis). The patient may be anaemic due to loss of blood through lung haemorrhaging over a long period. In Goodpasture’s syndrome, unlike many other conditions that cause similar symptoms, lung haemorrhaging most often occurs in smokers and those with damage from lung infection or exposure to fumes. Hemoptysis (US English) or haemoptysis (International English) is the expectoration (coughing up) of blood or of blood-stained sputum from the bronchi, larynx, trachea, or lungs (e. ... This article discusses the medical condition. ... The cigarette is the most common method of smoking tobacco. ... This false-colored electron micrograph shows a malaria sporozoite migrating through the midgut epithelia. ...

Kidney disease

The kidney disease mostly affects the glomeruli causing a form of nephritis. It is usually not detected until a rapid advance of the disease occurs so that kidney function can be completely lost in a matter of days, a condition known as rapidly progressive glomerulonephritis, or RPGN. Blood leaks into the urine causing hematuria, the volume urinated decreases and urea and other products usually excreted by the kidney are retained and build up in the blood. This is acute renal failure. Renal failure does not cause symptoms until more than 80% of kidney function has been lost. Symptoms include loss of appetite and sickness at first and then, when the damage is more advanced, breathlessness, high blood pressure and edema (swelling caused by fluid retention). The kidney involvement usually presents as nephritic syndrome, i.e. hematuria, a reduced glomerular filtration rate, and high blood pressure. This is in contrast to nephrotic syndrome, a more rare outcome of Goodpasture's, characterized by an abnormally large amount protein in the urine (proteinuria), coupled with severe edema. Glomerulus refers to two unrelated structures in the body, both named for their globular form. ... Nephritis is inflammation of the kidney. ... Human blood smear: a - erythrocytes; b - neutrophil; c - eosinophil; d - lymphocyte. ... This article or section is in need of attention from an expert on the subject. ... In medicine, hematuria (or haematuria) is the presence of blood in the urine. ... Urea is an organic compound of carbon, nitrogen, oxygen and hydrogen, with the formula CON2H4 or (NH2)2CO. Urea is also known as carbamide, especially in the recommended International Non-proprietary Names (rINN) in use in Europe. ... A sphygmomanometer, a device used for measuring blood pressure. ... Edema (American English) or oedema (British English), formerly known as dropsy or hydropsy, is swelling of any organ or tissue due to accumulation of excess lymph fluid, without an increase of the number of cells in the affected tissue. ... Nephritic syndrome is a collection of signs (known as a syndrome) associated with disorders affecting the kidneys, more specifically glomerular disorders. ... Glomerular filtration rate (GFR) is the volume of fluid filtered from the renal glomerular capillaries into Bowmans capsule per unit time. ...

Diagnosis

Because of the vagueness of early symptoms and rapid progression of the disease, diagnosis is often not reached until very late in the course of the disease. Kidney biopsy is often the fastest way to secure the diagnosis and gain information about the extent of the disease and likely effect of treatment. Tests for anti-GBM antibodies may also be useful, combined with tests for antibodies to neutrophil cytoplasmic antigens, which are also directed against the patient’s own proteins. Brain biopsy A biopsy (in Greek: bios = life and opsy = look/appearance) is a medical test involving the removal of cells or tissues for examination. ... Schematic of antibody binding to an antigen An antibody or immunoglobulin is a large Y-shaped protein used by the immune system to identify and neutralize foreign objects like bacteria and viruses. ...

Pathophysiology

As with many autoimmune conditions, the precise cause of Goodpasture’s Syndrome is not yet known. It is believed to be a type II hypersensitivity reaction to Goodpasture’s antigens on the cells of the glomeruli of the kidneys and the pulmonary alveoli, whereby the immune system wrongly recognizes these cells as foreign and attacks and destroys them, as it would an invading pathogen. Hypersensitivity is an immune response that damages the bodys own tissues. ... Glomerulus refers to two unrelated structures in the body, both named for their globular form. ... Detailed drawing of the alveoli from Grays Anatomy, 1918 - Schematic longitudinal section of a primary lobule of the lung (anatomical unit); r. ... A pathogen or infectious agent is a biological agent that causes disease or illness to its host. ...

Treatment

Like many autoimmune diseases, Goodpasture’s syndrome responds well to treatment with corticosteroids and immunosuppressants, however, the side effects of these can be serious, including as they do increased risk of infection, which may accelerate the progression of the disease. The concentration of anti-GBM antibodies in the blood may be reduced by apheresis to remove blood plasma and its replacement with an isotonic salt and protein solution. This course of treatment usually lasts between three and six months. In physiology, corticosteroids are a class of steroid hormones that are produced in the adrenal cortex. ... Immunosuppression is the medical suppression of the immune system. ... Whole blood enters the centrifuge on the left and separates into layers so that selected components can be drawn off on the right. ...

Antibiotic treatment of lung infection and stopping smoking may also help to reduce lung haemorrhaging. Staphylococcus aureus - Antibiotics test plate. ...

However, none of these can reverse permanent kidney damage and so for patients who have suffered this, renal transplant once the disease has subsided may be the only option. An organ transplant is the moving of a whole or partial organ from one body to another (or from a donor site on the patients own body), for the purpose of replacing the recipients damaged or failing organ with a working one from the donor site. ...

Epidemiology

Goodpasture’s syndrome is rare. In European populations between half and one case presents per million people per year. It is rarer than this in non-European populations. While cases have occurred in patients between the ages of 4 and 80, it is most common between ages 18 and 30 and again between 50 and 65. Men and women are equally affected.

Prognosis

In the 1970s, Goodpasture’s syndrome was most often fatal, but due to advances in diagnosis and treatment deaths are less common now. Death from lung haemorrhage may occur before the diagnosis has been made or in the initial stages of treatment before it has been properly controlled. With treatment, however, the patient can usually recover completely from lung damage. Kidneys, though, are less able to repair themselves and patients with kidney damage must often resort of a life on dialysis or kidney transplantation. Even with the best management there is still a significant mortality from renal failure, particularly if the patient is otherwise in poor health. It must also be remembered that the immunosuppressive treatment many patients are put on increases their risk of infection with a number of serious or fatal diseases. In medicine, dialysis is a type of renal replacement therapy which is used to provide an artificial replacement for lost kidney function due to renal failure. ...

External links

• synd/634 at Who Named It
• GBM antibodies: immunofluorescence image