UNIVERSITY OF MINNESOTA | Amyloidosis Program
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AMYLOIDOSIS & THE KIDNEYS
AMYLOIDOSIS & THE KIDNEYS
Renal amyloidosis is a serious condition where abnormal amounts of a fibrous protein substance (amyloid) collect - for unknown reasons - and build up in the kidneys, commonly causing disruption of normal, healthy, viable kidney tissue and malfunctioning subsequently with proteinuria, nephrotic syndrome (swelling), and, if untreated, end-stage renal failure. It is diagnosed via kidney biopsy. There are over a dozen different types of amyloidosis that can affect the kidneys, with the most common being AL (light chain), AA (secondary), hereditary TTR amyloidosis and ALECT2 amyloidosis. Treatment varies by type.
Symptoms
Symptoms
Noticeable, persistent foaming in urine, significant swelling in legs/ankles, unexplained fatigue, and sometimes shortness of breath if heart involvement exists.
Diagnosis
Diagnosis
Requires a tissue biopsy, usually of the kidney, but sometimes another organ that is also affected if unable to access the kidney.
Types & Causes
Types & Causes
AL Amyloidosis: it is related to abnormal bone marrow plasma cells (often treated with chemotherapy).
AA amyloidosis: misfolded serum amyloid A, triggered by chronic inflammation (e.g., rheumatoid arthritis, IBD) or infection (tuberculosis), deposits in organs like the kidneys, liver, and spleen.
Hereditary TTR amyloidosis: it is a condition that commonly affects the heart, the nerves and the kidneys.
ALECT2 amyloidosis: it happens in specific ethnic populations (Mexicans, Egyptians), often affecting the interstitium and causing less proteinuria than other forms.
Management
Management
AL Amyloidosis: Targeted chemotherapy, bortezomib, or stem cell transplant.
AA amyloidosis: Controlling the underlying inflammation with agents like IL-1 or TNF- inhibitors.
Hereditary TTR amyloidosis: Focuses on stabilizing the TTR protein to prevent misfolding or silencing its production in the liver.
ALECT2 amyloidosis: Treatment is mostly supportive, there is no specific curative treatment available.
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