Goodpasture's Syndrome or Anti-GBM Disease: Causes, Symptoms, Treatment- Plasmapheresis
Goodpasture's syndrome also is known as Anti-GBM disease and antiglomerular basement membrane disease.
This is an uncommon disorder that involves quick worsening of lung disease and kidney failure. However, in some cases either of them may also get affected i.e. either lung or kidney.
This syndrome is a rare autoimmune dysfunction. An autoimmune dysfunction is a condition in which immune system of body starts attacking and destroying its own healthy tissues. Patients suffering with Goodpasture syndrome usually start developing substances which start attacking a protein known as collagen present in glomeruli i.e. the filtering units of kidneys and the small air sacs present in lungs. The defective response of the immune system could also result in inflammation of the kidney's filtering units and bleeding in the air sacs of the lungs.
The GBM or Glomerular Basement Membrane of kidneys helps in filtering extra and waste fluid from blood. The substances that attack this collagen are called Antiglomerular Basement Membrane Antibodies. These antibodies are responsible for damaging the Glomerular Basement Membrane forcing it to cause damage to kidney.
In some cases inspiration of hydrocarbon solvents while breathing and viral infections of respiratory system may also result in Goodpasture syndrome. This occurs when immune system of body mistakenly attacks tissues or organs assuming them to be foreign chemicals and viruses.
Epidemiology of Goodpasture's Syndrome or Anti-GBM Disease
Goodpasture's syndrome is a rare disease. About 1 to 2% of all cases of rapidly progressive glomerulonephritis represent Goodpasture's syndrome. The incidence is about 1 in 2 million. Males are about seven times more prone to Goodpasture syndrome when compared to females. However, Goodpasture syndrome more commonly affects in adolescence. In children, both the sexes equally get affected with Goodpasture syndrome. About 60 to 80 percent of cases are reported to be both renal and pulmonary disease. About 10 percent cases are reported to be of lung problem. About 20 to 40 percent cases are reported to be kidney problem.
Causes and Risk Factors of Goodpasture's Syndrome or Anti-GBM Disease
Goodpasture's syndrome is usually caused by accumulation of pulmonary alveolar hemorrhage and acute glomerulonephritis. Goodpasture's syndrome is an autoimmune disorder caused by type 2 antigen-antibody reactions which often results in glomerulonephritis and diffuse pulmonary hemorrhage.
- Insult to the lungs may lead to both renal and pulmonary disease.
- Genetic predisposition with presence of HLA-DRw2.
- HLA-B7 relates to more severe anti-GBM nephritis and is seen to be more frequent.
- Exposure to hydrocarbons and organic solvents.
- Exposure to metal dusts.
- Renal transplantation in Alport's syndrome.
- Infection such as influenza.
Signs and Symptoms of Goodpasture's Syndrome or Anti-GBM Disease
Symptoms often develop too fast over a few days to weeks, but sometimes may occur slowly over a period some months to years. Listed below are few of the symptoms.
- Appetite loss.
- Coughing blood.
- Burning sensation with urination.
- Dry cough.
- Pale skin.
- Shortness of breath.
- Swelling in any region of body, particularly lower extremities.
- Nausea and vomiting.
Treatment for Goodpasture's Syndrome or Anti-GBM Disease
- The treatment for Goodpasture's syndrome usually concentrates on removing harmful antibodies present in blood. Plasmapheresis is a treatment which is done to remove complete blood from body in order to replace plasma with donated plasma, fluid or protein. Removal of these defected antibodies helps in reducing inflammation in lungs and kidneys.
- Steroids like prednisone and medications that are useful in suppressing immune system can also be given.
- Kidney damage can be delayed by controlling the blood pressure. Angiotensin receptor blockers and ACE inhibitors help in controlling high blood pressure.
- Limiting fluids and salt intake helps in reducing swelling. Low protein diet is advised in some instances. Dialysis may be required in case of renal failure, and in some severe cases kidney transplant may be required if the kidney stops functioning appropriately.
Investigations for Goodpasture's Syndrome or Anti-GBM Disease
A complete subjective and physical examination is performed to diagnose Goodpasture's syndrome. Signs like fluid overload, high blood pressure, abnormal heart and lung sounds may implicate Goodpasture's syndrome.
Abnormal results of urinalysis such as blood and protein in the urine. Abnormal red blood cells may also be found.
Tests For Diagnosing Goodpasture's Syndrome or Anti-GBM Disease May Include:
- Chest x-ray.
- Anti-glomerular basement membrane test.
- Arterial blood gas.
- Lung biopsy.
- Kidney biopsy.
- Serum creatinine.
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