Goodpasture Syndrome

Classification according to ICD-10
M31.0	Hypersensitivity angiitis Goodpasture syndrome
ICD-10 online (WHO version 2019)

The 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 a combination of glomerulonephritis (a certain type of kidney inflammation) and pulmonary bleeding in 1919.

Diffuse pulmonary hemorrhage in Goodpasture syndrome in the pathological specimen.

According to current understanding, the disease is caused by autoantibodies formed against the basement membrane of the blood vessels, especially in the area of ​​the kidney glomeruli and the alveoli . According to the classical system of allergies according to the pathomechanism , an autoimmune disease of type II is present. Involvement in the lungs causes bleeding with bloody sputum ( haemoptosis ), which leads to pulmonary siderosis (iron deposition).

Glomerulonephritis is a rapidly progressing glomerulonephritis that belongs to the group of diffuse-extracapillary glomerulonephritis - loop necrosis and strong crescent formation occur. The antibodies are directed against the non-collagenous domain of the alpha-3 chain of collagen type IV of the glomerular and alveolar basement membrane. They can be detected in the blood serum.

Occurrence

Very rare ( incidence : 0.5–1 case per million population and year). The syndrome affects men twice as often as women. The disease usually manifests itself between the ages of 20 and 40. Inhaling toxic substances (such as gasoline fumes and dusty bird droppings) can be B. be a trigger.

diagnosis

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

therapy

At the moment this disease cannot be cured. However, immunosuppressive drugs ( cyclophosphamide and high-dose prednisone ) can inhibit the formation of new anti-GBM AKs. The antibodies are removed with a plasmapheresis (plasma exchange, plasma separation ); this is done in a similar way to plasma donation with dialysis machines. Such a plasma separation usually takes place in the intensive care unit in the acute stage. However, this treatment must be repeated at least twice a week, as the plasma replicates quickly. The plasma is then discarded and not infused back into the patient. Any vaccination protection is lost. During immunoadsorption , the antibodies are filtered out and the blood plasma is then reinfused back into the patient, whereby larger amounts of blood can be purified. This improves the general condition. It should be noted that - at least temporarily - a loss of the acquired immune system (e.g. vaccination protection ) is to be expected.

Therapy takes about 8 to 12 months. Recurrences are rather rare. The mortality of the disease could be reduced from 90% to the current 20%. However, this may cause increased formation of antibodies in the patients, which as a non-return ( rebound is called).

forecast

Without therapy, the prognosis for Goodpasture syndrome is poor . The disease is rapidly progressive , so early diagnosis is important.

Individual evidence

1. ^ AD Salama, JB Levy, L. Lightstone, CD Pusey: Goodpasture's disease . In: Lancet . tape 358 , no. 9285 , September 2001, p. 917-920 , doi : 10.1016 / S0140-6736 (01) 06077-9 , PMID 11567730 .

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !