Treatment Options In Goodpastures.

Kidney (Photo credit: Joshua Schwimmer)

Overview of Goodpastures Syndrome.

A rapidly progressive form of kidney disease associated with antibodies directed against the structural components of the functional subunits of the kidney and the lung. The disease therefore gives rise to both lung and kidney pathology a situation termed pulmonary renal syndrome.

The exact cause is unknown. It is considered an autoimmune disorder (other autoimmune disorder's that affect the kidney include Lupus Nephritis). The bodies own immune system is responsible for targeting both lung and kidney tissues. The initial immune system activation may occur in response to viral infection or even the inhalation of toxin from the environment such as inhaling gasoline. Smoking increases the risk of the disease it is most likely to occur in men from ages 16 to 61.

It is possible to be predisposed to good pastures syndrome based solely on your genes. It is therefore possible to have family members with the same problem. The disorder is however relatively rare.


Symptoms of Goodpastures.

The predominant symptoms of Goodpastures syndrome includes cough productive of bloody sputum due to bleeding within the lungs from the inflammation caused there. The kidney disease produced usually has symptoms such as swelling fo the feet or around the eyes retention of fluid and elevated blood pressure with or without headaches. There is lethargy and weakness associated with decreased appetite if the condition has been going on long enough. The nonspecific symtpoms of weakness and lethargy loss of appetite are due to the systemic nature of the disease as well as the anemia associated with it. The anemia is frequently multifactorial, due both to iron loss due to the loss of blood in the lungs as well as kidney disease. Because of episodes of bleeding into the lungs there may be episodic shortness of breath which may be due both to retention of fluid from kidney failure as well as blood filling the spaces within the lung for gas exchange.


Treatment Options.

The mainstay of treatment of Goodpastures is early recognition followed by plasmapherisis (a process of removal of the immune system components of blood) and administration of cytotoxic medication, (medication targeted against rapidly dividing cells of the immune system) and steroid therapy.

Goalfinder immune-system-action (Photo credit: gfinder)

On the horizon are some interesting therapeutic options.


A monoclonal antibody known as ox-8 is the latest experimental drug with efficacy in experimental good pastures disease in rats. It is deemed effective in the prevention and treatment of experimental inflammation of the kidney in rats. We will be hearing more about this one in the future.


Because goodpastures is caused by activity of the native immune system against the collagen forming the structural component of the filtration apparatus of the kidney it is possible to treat the disease by turning off the immune reaction against that specific structural component which is collagen. This has been accomplished in rats utilizing nasally administered collagen which induces tolerance. When tolerance occurs the immune system accepts a previous enemy as a friend and does not attack. This would effectively turn off the immune response against the kidney if it can be achieved in humans


Much basic research is needed and continues to be done looking at goodpasture's disease. Although a rare disease the mechanisms of tolerance and autoimmunity governing the pathology of this condition if properly understood and targetable would herald a new day in the treatment of many other kidney diseases of similar etiology.