Dialysis-related amyloidosis
Dialysis-related Amyloidosis Dialysis-related amyloidosis (DRA) is a condition that occurs in patients who have been on long-term dialysis, typically for more than 5 years. It is characterized by the accumulation of amyloid proteins, particularly beta-2 microglobulin, in various tissues of the body. This can lead to a range of symptoms and complications, primarily affecting the musculoskeletal system.
Pathophysiology[edit | edit source]
The primary protein involved in dialysis-related amyloidosis is beta-2 microglobulin. This protein is normally filtered by the kidneys, but in patients with renal failure, it accumulates in the blood. During dialysis, especially with older dialysis membranes, beta-2 microglobulin is not adequately removed, leading to its deposition in tissues as amyloid fibrils.
Clinical Manifestations[edit | edit source]
Patients with dialysis-related amyloidosis may experience a variety of symptoms, including:
- Joint Pain and Stiffness: Commonly affects the shoulders, wrists, and knees.
- Carpal Tunnel Syndrome: Due to amyloid deposition in the carpal tunnel, leading to compression of the median nerve.
- Bone Cysts and Fractures: Amyloid deposits can weaken bones, making them more susceptible to fractures.
- Visceral Involvement: In some cases, amyloid deposits can affect organs such as the heart and gastrointestinal tract.
Diagnosis[edit | edit source]
Diagnosis of dialysis-related amyloidosis is based on clinical symptoms, imaging studies, and biopsy. Imaging techniques such as MRI can reveal amyloid deposits in joints and bones. A definitive diagnosis is made by biopsy, which shows amyloid deposits that stain positively with Congo red dye.
Treatment[edit | edit source]
The primary treatment for dialysis-related amyloidosis is to improve dialysis efficiency to reduce beta-2 microglobulin levels. This can be achieved by:
- High-flux Dialysis: Using dialysis membranes that more effectively remove beta-2 microglobulin.
- Hemodiafiltration: A dialysis technique that combines diffusion and convection to enhance solute removal.
- Kidney Transplantation: The most effective treatment, as it restores kidney function and reduces beta-2 microglobulin levels.
Prognosis[edit | edit source]
The prognosis for patients with dialysis-related amyloidosis varies. While improving dialysis techniques can slow the progression of the disease, the condition can significantly impact quality of life due to chronic pain and disability. Kidney transplantation offers the best chance for improvement.
See Also[edit | edit source]
External Links[edit | edit source]
- [National Institute of Diabetes and Digestive and Kidney Diseases](https://www.niddk.nih.gov/)
- National Organization for Rare Disorders
NIH genetic and rare disease info[edit source]
Dialysis-related amyloidosis is a rare disease.
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