Goodpasture syndrome

Goodpasture 's syndrome is a very rare serious autoimmune disease in which mainly the kidneys and lungs are involved.

The American pathologist Ernest William Goodpasture (1886-1960) described the combination of glomerulonephritis ( certain form of inflammation of the kidneys ) with pulmonary hemorrhage 1919.

According to current understanding, the disease is caused by autoantibodies formed against the basement membrane of blood vessels, particularly in the renal glomeruli and alveoli. According to the classical classification of Coombs and Gell there is thus an autoimmune disease type II. The pulmonary involvement caused bleeding with bloody sputum ( hemoptysis) that lead to the lungs siderosis ( iron deposits ).

The glomerulonephritis is a rapidly progressive glomerulonephritis which is counted to the group of diffuse extracapillary glomerulonephritis - there is Schlingennekrosen and strong crescent formation. The antibodies directed against the non-collagenous domain of collagen type IV basement membrane. They can be detected in blood serum.

Occurrence

Very rare (incidence 0.5-1 case per million population per year ). The syndrome affects men twice as often as women. The disease usually manifests between 20 to 40 years of age.

Diagnosis

The diagnosis is secured when a nephritic sediment is present and the patient in the laboratory anti- glomerular basement membrane antibody ( anti -GBM - AK ) has ( immunoelectrophoresis ).

Therapy

Currently, this disease can not be cured. However, (cyclophosphamide and high-dose prednisone) the formation of new anti -GBM Ab inhibited by immunosuppressive drugs. By means of plasmapheresis (blood exchange), the antibodies are removed. When immunoadsorption they are filtered out and the blood plasma to the patient then reinfused back, creating larger amounts of blood can be cleaned. Thus, the general condition is improving. It is important to note that there - at least temporarily - with a loss of acquired immune defense (eg immunization ) is to be expected.

The therapy lasts about 8-12 months. Recurrences are rare. The mortality of the disease could be reduced as from 90% to 20% currently. However, this can cause in the patients an increased antibody formation, which is called non-return ( rebound ).

Forecast

Without treatment of Goodpasture's syndrome is hopeless. The disease is rapidly progressive, so early diagnosis is important.