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Focal segmental glomerulosclerosis associated with cutaneous and systemic plasmacytosis
Shinsuke Isobe1, Naro Ohashi2, Naoko Katahashi2
1Internal Medicine 1, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi-ku, Hamamatsu, 431-3192, Japan. isobe58@hama-med.ac.jp.
CEN Case Reports
|September 24, 2017
Summary
This study reports the first case of focal segmental glomerulosclerosis (FSGS) in a patient with cutaneous and systemic plasmacytosis (CSP). Treatment with prednisolone and cyclosporine successfully managed both skin lesions and proteinuria.
Area of Science:
- Nephrology
- Dermatology
- Hematology
Background:
- Cutaneous and systemic plasmacytosis (CSP) is a rare lymphoproliferative disorder.
- Renal involvement, particularly glomerulonephritis, is uncommon in CSP, though it is considered a variant of Castleman disease.
Purpose of the Study:
- To report the first case of nephrotic syndrome associated with focal segmental glomerulosclerosis (FSGS) in a patient with CSP.
- To explore potential pathogenetic mechanisms linking CSP and FSGS.
Main Methods:
- Case presentation of a 41-year-old Japanese man with nephrotic syndrome and CSP.
- Renal biopsy with light microscopy, electron microscopy, and lymph node biopsy.
- Clinical assessment of treatment response to prednisolone and cyclosporine.
Main Results:
- Renal biopsy revealed focal segmental glomerulosclerosis (FSGS) with diffuse foot process effacement, without interstitial plasma cell infiltration.
- Lymph node biopsy excluded Castleman disease.
- The patient showed successful treatment of skin lesions and proteinuria with prednisolone and cyclosporine.
Conclusions:
- This is the first documented case of FSGS associated with CSP.
- While the causal link remains unclear, elevated IL-6 and VEGF levels, along with decreased podocyte VEGF expression, may play a role in the renal pathology of CSP.
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