Dysproteinemia and the Kidney: Core Curriculum 2019

Area of Science:

• Nephrology
• Hematology
• Immunology

Background:

• Dysproteinemic kidney diseases involve monoclonal immunoglobulin or light chain production by B-cell or plasma cell clones, leading to kidney damage.
• Presentations range from mild proteinuria to severe acute kidney injury, with diverse histologic patterns including cast nephropathy, deposition diseases, and glomerulonephritis.
• These conditions encompass overt multiple myeloma and smaller clonal populations classified as monoclonal gammopathies of renal significance.

Purpose of the Study:

• To review the pathogenesis, diagnosis, and treatment of dysproteinemic kidney diseases.
• To highlight the spectrum of kidney injury caused by monoclonal proteins.
• To emphasize the importance of treating the underlying clonal disorder for renal recovery.

Main Methods:

• Review of existing literature on dysproteinemic kidney diseases.
• Analysis of clinical presentations and histologic findings.
• Discussion of treatment strategies targeting the underlying clonal disorder.

Main Results:

• Monoclonal immunoglobulins cause various kidney injuries, including cast nephropathy, amyloidosis, and glomerulonephritis.
• Both overt and subtle clonal disorders can lead to significant kidney damage.
• Hematologic response, achieved by eradicating the clone, is crucial for organ-specific recovery.

Conclusions:

• Dysproteinemic kidney diseases require prompt diagnosis and treatment of the underlying B-cell or plasma cell clone.
• Effective treatment leads to hematologic response, which is essential for improving kidney function and preventing progression to end-stage kidney disease.
• Understanding the pathogenesis and diverse presentations is key to managing these complex renal disorders.