J Korean Med Sci.
2018 Apr;33(18):e136. 10.3346/jkms.2018.33.e136.
Primary Glomerulonephritis with Unique C4d Deposition and Concurrent Non-infectious Intermediate Uveitis: a Case Report and Literature Review
- Affiliations
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- 1Department of Internal Medicine, BioMedical Research Institute, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea.
- 2Department of Ophthalmology, BioMedical Research Institute, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea.
- 3Department of Pathology, BioMedical Research Institute, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea.
- 4Department of Pathology, Medical Research Institute, Pusan National University Yangsan Hospital, Pusan National University School of Medicine, Yangsan, Korea. mysol@pusan.ac.kr
- KMID: 2412845
- DOI: http://doi.org/10.3346/jkms.2018.33.e136
Abstract
- C4 glomerulopathy is a recently introduced entity that presents with bright C4d staining and minimal or absent immunoglobulin and C3 staining. We report a case of a 62-year-old man with C4 glomerulonephritis (GN) and uveitis. He presented to the nephrology department with proteinuria and hematuria. The patient also had intermediate uveitis along with proteinuria and hematuria. A kidney biopsy that was performed in light of continuing proteinuria and hematuria showed a focal proliferative, focal sclerotic glomerulopathy pattern on light microscopy, absent staining for immunoglobulin or C3 by immunofluorescence microscopy, with bright staining for C4d on immunohistochemistry, and electron-dense deposits on electron microscopy. Consequently, C4 GN was suggested as the pathologic diagnosis. Although laser microdissection and mass spectrometry for glomerular deposit and pathologic evaluation of the retinal tissue were not performed, this is the first report of C4 GN in Korea and the first case of coexisting C4 GN and uveitis in the English literature.
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