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Crystalglobulin-Induced Nephropathy and Keratopathy
Matthew R D'Costa1, Lauren A Dalvin2, Sandhya Manohar1
1Department of Nephrology and Hypertension, Mayo Clinic, Rochester, MN.
Kidney Medicine
|August 1, 2020
Summary
Crystalglobulinemia is a rare condition where crystallized proteins damage tissues. Early diagnosis and a multidisciplinary approach, including stem cell transplant, improved patient outcomes.
Area of Science:
- Nephrology
- Ophthalmology
- Hematology
Background:
- Crystalglobulinemia, a rare manifestation of monoclonal gammopathy, involves vascular deposition of crystallized monoclonal proteins causing tissue injury.
- Monoclonal gammopathies can present with diverse systemic manifestations, often posing diagnostic challenges.
Observation:
- A 56-year-old man presented with migratory polyarthralgias and blurry vision, later developing malignant hypertension and kidney failure.
- Kidney biopsy initially suggested idiopathic thrombotic microangiopathy, but further evaluation revealed an underlying monoclonal protein disorder and crystalline keratopathy.
Findings:
- Pronase staining of kidney biopsy confirmed crystalglobulin-induced nephropathy.
- Treatment with chemotherapy and autologous stem cell transplantation led to disease remission, improved kidney function, and reduced symptoms.
Implications:
- This case highlights the varied systemic presentations of monoclonal gammopathies.
- A multidisciplinary approach is crucial for diagnosing and managing crystalglobulinemia and related disorders.
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