Monoclonal gammopathies (MG) represent a varied group of diseases that have in common the clonal production of an immunoglobulin or fragment thereof. The monoclonal immunoglobulin (MIg) is produced by a clone of B cells, plasma cells, or lymphoplasmacytic cells and can be present in its intact form or as fragments (light, heavy, or a light and heavy chain). Systemic amyloidoses are also a rare and heterogenous group of diseases that result from the ability of certain proteins to misfold and polymerize into insoluble amyloid fibrils. These fibrils are deposited in the extracellular space of tissues and organs causing tissue damage and significant morbidity and even mortality. Amyloidosis is named by their native protein and is often classified by whether it is wild type (acquired) or the result of a germline pathogenic variant (mutant).
Both MG and amyloidosis affect the kidney. In the absence of hematological malignancy, patients with a MIg in the serum and/or urine in combination with kidney involvement, are considered to have a “monoclonal gammopathy of renal significance” (MGRS). This categorization addresses a therapeutic gap as patients not meeting criteria for overt multiple myeloma were traditionally not treated, (except for AL amyloidosis and monoclonal immunoglobulin deposition disease (MIDD)). Beside AL amyloidosis the most common amyloidogenic proteins that can affect the kidneys include transthyretin (ATTR), beta-2 microglobulin (Aβ2M), serum amyloid A (AA) amyloid and leukocyte cell derived chemotaxin 2 amyloidosis (ALECT2).
At the end of this course participants should be able to:
Define the different types of MGRS and of amyloidosis affecting the kidney and estimate their prevalence
Describe the pathogenesis of MGRS and of amyloidosis-related kidney disease
Discuss the diagnostic evaluation of patients with MGRS and patients with different types of amyloidosis
Illustrate the current principles of management of MGRS and the different types of amyloidosis affecting the kidney and the role of kidney transplantation
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Introduction to MGRS: pathogenesis, diagnosis, and classification
Presentation: Introduction to MGRS: pathogenesis, diagnosis, and classification
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Quiz: Introduction to MGRS: pathogenesis, diagnosis, and classification
3 Questions | 1 attempt | 0/3 points to pass
3 Questions | 1 attempt | 0/3 points to pass
Pathology of MGRS-Related Kidney Diseases
Presentation: Pathology of MGRS-Related Kidney Diseases
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Quiz: Pathology of MGRS-Related Kidney Diseases
3 Questions | 1 attempt | 0/3 points to pass
3 Questions | 1 attempt | 0/3 points to pass
Presentation: MIDD
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Quiz: MIDD
3 Questions | 1 attempt | 0/5 points to pass
3 Questions | 1 attempt | 0/5 points to pass
Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits (PGNMID)
Presentation: Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits (PGNMID)
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Quiz: Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits (PGNMID)
3 Questions | 1 attempt | 0/5 points to pass
3 Questions | 1 attempt | 0/5 points to pass
Presentation: MIg-associated C3G
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Quiz: MIg-associated C3G
3 Questions | 1 attempt | 0/3 points to pass
3 Questions | 1 attempt | 0/3 points to pass
Light Chain Proximal Tubulopathy
Presentation: Light Chain Proximal Tubulopathy
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Quiz: Light Chain Proximal Tubulopathy
3 Questions | 1 attempt | 0/3 points to pass
3 Questions | 1 attempt | 0/3 points to pass
Presentation: AL Amyloidosis
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Quiz: AL Amyloidosis
3 Questions | 1 attempt | 0/3 points to pass
3 Questions | 1 attempt | 0/3 points to pass
Presentation: AA Amyloidosis
Open to view video.
Quiz: AA Amyloidosis
2 Questions | 1 attempt | 0/2 points to pass
2 Questions | 1 attempt | 0/2 points to pass
Kidney Involvement in Hereditary Transthyretin Amyloidosis (ATTRv)
Presentation: Kidney Involvement in Hereditary Transthyretin Amyloidosis (ATTRv)
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Quiz: Kidney Involvement in Hereditary Transthyretin Amyloidosis (ATTRv)
3 Questions | 1 attempt | 0/6 points to pass
3 Questions | 1 attempt | 0/6 points to pass
Presentation: Pathology of Amyloidosis
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Quiz: Pathology of Amyloidosis
3 Questions | 1 attempt | 0/3 points to pass
3 Questions | 1 attempt | 0/3 points to pass
ALECT-2 and Rare Hereditary subtypesof Renal Amyloidosis
Presentation: ALECT-2 and Rare Hereditary subtypesof Renal Amyloidosis
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Quiz: ALECT-2 and Rare Hereditary subtypesof Renal Amyloidosis
3 Questions | 1 attempt | 0/3 points to pass
3 Questions | 1 attempt | 0/3 points to pass
Accreditation Assessment Survey
15 Questions
CME Certificate
2.00 CME credits | Certificate available
2.00 CME credits | Certificate available
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