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Case Reports
. 2019 Feb 25;1(2):71-74.
doi: 10.1016/j.xkme.2019.01.006. eCollection 2019 Mar-Apr.

Crystalglobulin-Induced Nephropathy and Keratopathy

Matthew R D'Costa  1 , Lauren A Dalvin  2 , Sandhya Manohar  1 , Leo J Maguire  2 , Joseph P Grande  3 , Wilson I Gonsalves  4 , Samih H Nasr  3 , Marie C Hogan  1
Affiliations
Case Reports

Crystalglobulin-Induced Nephropathy and Keratopathy

Matthew R D'Costa et al. Kidney Med. .

Erratum in

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.

Keywords: Crystalglobulin nephropathy; crystalglobulinemia; crystalline keratopathy.

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Figures

Figure 1
Figure 1
Slit-lamp examination of the right eye in a patient with crystalglobulin-induced nephropathy shows diffuse subepithelial and stromal corneal crystalline deposits (gold arrows) visible on both (A) direct and (B) retroillumination. (C) Confocal microscopy examination shows the presence of subepithelial and stromal hyperreflective deposits. (D) Slit-lamp examination of the right eye following treatment with cyclophosphamide, bortezomib, and dexamethasone reveals fewer crystalline deposits.
Figure 2
Figure 2
Kidney biopsy (light microscopy; silver methenamine stain) from a patient with crystalglobulin-induced nephropathy showed (A) intravascular (arrow) crystalline structures. Immunofluorescence (IF) was originally negative (not shown), but (B, C) pronase IF was negative for κ and positive for λ light chains. Electron microscopy is not shown because the sampled material did not contain crystals.
See this image and copyright information in PMC

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