By Rajlakshmi Mukhopadhyay
What does the summer holiday compose of if you’re a medical student? Enjoying the sunshine, catching up with family and friends and perhaps a placement in your home country or abroad. I had the good fortune to be accepted for a placement in the nephrology department at a top London hospital.
I entered the grand entrance of the hospital on my first day with a few butterflies in my stomach. I had no idea of what I should expect. Having only experienced the more compact nature of the hospitals in Riga and Scotland, the entrance itself was daunting. There were people everywhere. I decided to stop and take a breath.
Heading up to the nephrology department, I was greeted by the members of staff who run this very busy unit on a day-to-day basis. After a few introductions, I joined the nurses and doctors who were preparing for a ward round to review the various patients who had been admitted as in-patients.
Among these patients was a 45-year-old Asian man with a rare condition. He had presented to his family doctor with a urinary tract and a persistent respiratory tract infection. The patient’s weakness had prompted the doctor to take a blood sample for analysis and shockingly, the kidney function appeared to be greatly affected. He was referred quickly to the nephrology department.
“Only about 1 case in a million people is diagnosed in white European populations.
Further tests revealed that the patient had a rare condition called Anti-Glomerular Basement Membrane Disease or Goodpasture’s Syndrome. Only about 1 case in a million people is diagnosed in white European populations. The numbers are even rarer in other populations. It is also most common at the ages of 18-30 and 50-65. Both men and women are equally affected by this condition.
“The capillaries that are attacked are based in the glomeruli, and these capillaries have the function of filtering the blood and creating urine.”
What causes this condition?
The cause of Goodpasture’s Syndrome is not fully known but there can be a few triggers to the disease. Lithotripsy, a shock wave given to break and remove a kidney stone, and other forms of kidney trauma could act as triggers. However, our patient had not suffered from any other type of kidney trauma. The disease can also affect the lungs as well as the kidneys, or it can just affect the lungs. It can also be caused by autoantibodies that attack the walls of the blood vessels.
The capillaries that are attacked are based in the glomeruli, which have the function of filtering the blood and creating urine. The capillaries have thin basement membranes and they are attacked by the anti-glomerular basement membrane or anti-GBM antibodies. The antibodies are found in the blood serum and therefore they can travel everywhere in the body.
Anti GBM antibodies
In the kidney, the disease is not usually diagnosed until its sudden acceleration, which can mean that the patient’s kidney can be completely destroyed by the time treatment is initiated. A form of inflammation, anti-GBM glomerulonephritis occurs as a result of the action of the autoantibodies. Blood leaks into the urine and the volume of urine created decreases, causing retention of the waste products within the body.
Symptoms include fever, nausea, vomiting, weight loss, chest pain, anaemia, respiratory and kidney failure.
How is anti-GBM disease diagnosed and treated?
When a patient is brought into the hospital, a number of tests are performed to diagnose this condition. Blood and urine can be analysed to detect changes in the kidney function and anaemia. There is also a specific blood test for Anti-Glomerular basement membrane antibodies. As many as a third of the patients with the disease will also have antineutrophilic cytoplasmic antibodies and these can also be detected using a blood test.
Anti-GBM treatment occurs in three stages:
- The anti-GBM antibody is removed using a process called plasmapheresis. In this procedure, the patient’s blood is removed from the body in small amounts and the antibody is removed from the blood. Then the cleansed blood is returned to the patient.
- Immunosuppression is provided to restrict further antibody production.
- If a specific chemical is felt to have triggered the disease, future exposures are avoided.
Although the kidney function is decreased, it is not possible to provide a transplant for the patient. The reason for this is that the same antibodies will attack the kidneys again and create kidney failure once again.
What is the future for our patient?
After the initial ward round and in the days afterwards, I spent a while every day chatting to the patient about his life and his future ahead. According to the doctors, the initial plasmapheresis treatment appeared to be working but he still had a long time ahead in and out of hospital in the future. The patient had a family with young children and, while he worried for the future, he seemed optimistic about the success of his treatment. He was grateful that the disease had been diagnosed early. As his family came to see him during visiting hours with his young children jumping onto his bed and chatting away about their day, I saw him transform from our patient and into a father, a husband and a son.