Amyloidosis is a rare but well-known complication of most of the long term illness. It is the deposition of abnormally folded proteins in the body compartments. However, we see a type of amyloidosis in patients with Chronic Kidney Diseases (CKD) and chronic hemodialysis. This is termed as dialysis related amyloidosis (Beta 2 Amyloidosis). Additionally This type of amyloidosis has found in patient with severe renal impairment, but has not undergone any dialysis.
Mechanism of Dialysis Related Amyloidosis
Our kidney is the most vital organ for the excretory functions of the body. Usually, it removes all the excess chemicals from the body. Beta 2 macroglobulin is also filtered out from the kidney. However, When someone has chronic kidney disease, they have to undergo regular hemodialysis to maintain regular kidney functions. But the artificial dialysis membrane fails to filter out the type of macroglobulin called beta 2 macroglobulin (a type of small protein) from the body.
The failure to remove the beta-amyloid associated macroglobulin can cause the accumulation of them in the body compartments. Some of the sites are the joints and synovium, heart, tongue, and some other organs.
Complications of Dialysis Associated Amyloidosis
Depending on the site of the deposition of the Beta 2 globulin, it cause different clinical signs and symptoms. Most often these patients presenting with chronic joint pains. However in addition to joint pains dialysis related amyloidosis causes lot of different clinical features.
- Chronic joint pain
- Carpal tunnel syndrome
- Cardiomegaly
- Heart failure
- Macroglossia (large tongue)
- Bone fractures
How to Prevent Dialysis Related Amyloidosis
All the patients who undergo chronic dialysis are prone to get dialysis-associated amyloidosis. It is estimated to have 50% of patients getting dialysis related amyloidosis with in 10years of dialysis. Additionally most of the treatment methods for dialysis associated beta 2 amyloidosis fail to completely cure the complications like chronic joint pains, bone destructions and etc.
Therefore it is essential to take adequate precautions to prevent this conditions in all patients undergoing hemodialysis.
Young patients are more prone to get dialysis associated amyloidosis as they are getting more dialysis. Therefore it is better to transplant them early as possible. Longer the duration of dialysis , higher the risk of DAA.
Early kidney transplant is the best method to prevent dialysis related amyloidosis.
However some of the patient with End stage kidney failure may not fit enough to undergo kidney transplant. In such situations we have to take some other methods to prevent this condition.
Most importantly maintaining the residual kidney function is necessary. This will patient own kidneys to function with limitations. As a result, kidneys are able to remove some of the beta 2 macro globulins from the body.
The percentage of beta 2 amyloidosis increase when we use low flux dialyzers. Therefore it is advisable to use high flux dialyzers. When we use high flux dialyzers, a larger pore size allows the dialyzer to remove globulins from the blood.
In addition to the dialyzer, there is important role of dialysate solutions. Therefore always use high quality dialysis solutions in each dialysis.
Some centers also use beta 2 macroglobulin filters to remove beta 2 globulins from the blood. This also reduce the plasma level of beta 2 globulins. As a result there is less chance of accumulation of amyloids in the body tissues.
Related articles
- Bardin T, Zingraff J, Kuntz D, Dru¨ eke T. Dialysis related amyloidosis. Nephrol Dial Transplant 1986; 1: 151–154
- Zingraff J, Noe¨l LH, Bardin T, et al. Beta-2-microglobulin amyloidosis in chronic renal failure (letter). N Engl J Med 1990;323: 1070–1071
- Mohamed OMH, Taher MM, Elfakey WEM. Beta 2-microglobulin amyloidosis causing carpal tunnel syndrome, mimic steal syndrome. MOJ Anat & Physiol. 2018;5(2):127-129. DOI: 10.15406/mojap.2018.05.00176