Crystalglobulin-Induced Nephropathy and Keratopathy

Matthew R. D'Costa, Lauren A. Dalvin, Sandhya Manohar, Leo J. Maguire, Joseph Peter Grande, Wilson Gonsalves, Samih H. Nasr, Marie C Hogan

Research output: Contribution to journalArticle

Abstract

Crystalglobulinemia, a rare manifestation of monoclonal gammopathy, results from vascular deposition of crystallized monoclonal proteins leading to tissue injury. A 56-year-old man initially presented several years earlier with migratory polyarthralgias and blurry vision with no unifying diagnosis. Following an acute episode of malignant hypertension and rapidly progressive kidney failure, kidney biopsy was performed and was interpreted as idiopathic thrombotic microangiopathy. Further evaluation revealed an underlying monoclonal protein disorder. Slit-lamp biomicroscopy evaluation showed crystalline keratopathy. Re-evaluation of the kidney biopsy material with pronase staining confirmed crystalglobulin-induced nephropathy. The patient was initially treated with cyclophosphamide, bortezomib, and dexamethasone with partial response, followed by autologous stem cell transplantation with normalization of monoclonal protein studies, improvement in kidney function and joint symptoms, and decreased corneal deposits. His disease recurred but did not require additional treatment 1 year later. This case exemplifies the unique systemic presentation of diseases in the monoclonal gammopathy spectrum and emphasizes the need for a multidisciplinary approach when caring for these patients.

Original languageEnglish (US)
Pages (from-to)71-74
Number of pages4
JournalKidney Medicine
Volume1
Issue number2
DOIs
StatePublished - Mar 1 2019

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Paraproteinemias
Kidney
Thrombotic Microangiopathies
Malignant Hypertension
Biopsy
Pronase
Proteins
Arthralgia
Stem Cell Transplantation
Cyclophosphamide
Dexamethasone
Renal Insufficiency
Blood Vessels
Joints
Staining and Labeling
Wounds and Injuries
Therapeutics
Slit Lamp
Bortezomib

Keywords

  • Crystalglobulin nephropathy
  • crystalglobulinemia
  • crystalline keratopathy

ASJC Scopus subject areas

  • Nephrology
  • Internal Medicine

Cite this

Crystalglobulin-Induced Nephropathy and Keratopathy. / D'Costa, Matthew R.; Dalvin, Lauren A.; Manohar, Sandhya; Maguire, Leo J.; Grande, Joseph Peter; Gonsalves, Wilson; Nasr, Samih H.; Hogan, Marie C.

In: Kidney Medicine, Vol. 1, No. 2, 01.03.2019, p. 71-74.

Research output: Contribution to journalArticle

D'Costa, Matthew R. ; Dalvin, Lauren A. ; Manohar, Sandhya ; Maguire, Leo J. ; Grande, Joseph Peter ; Gonsalves, Wilson ; Nasr, Samih H. ; Hogan, Marie C. / Crystalglobulin-Induced Nephropathy and Keratopathy. In: Kidney Medicine. 2019 ; Vol. 1, No. 2. pp. 71-74.
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