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A case developing minimal change disease during the course of IgG4-related disease
Kazunori Yamada1,2, Takeshi Zoshima1, Kiyoaki Ito1
1a Division of Rheumatology, Kanazawa University Graduate School of Medicine , Kanazawa , Ishikawa , Japan.
Abstract:
We describe a 66-year-old male with immunoglobulin G4-related disease (IgG4-RD) presenting with minimal change disease (MCD). Three years prior to this admission, the patient had been diagnosed with IgG4-RD. The development of sudden massive proteinuria (4+; 16.7 g/gCr) with a weight gain of 8 kg within a two-week period was noted, and nephrotic syndrome was suspected. The patient's serum IgG4 level did not increase and hypocomplementemia was not found. A renal biopsy showed no cellular infiltration in the renal interstitium, and no spiking or bubbling was found on periodic acid methenamine silver staining. On electron microscopy, foot process effacement was seen, but no subepithelial electron-dense deposits were found. The patient was diagnosed with MCD. Ten days after starting prednisolone (60 mg/day), proteinuria was negative. Since IgG4-RD and MCD share a T-helper 2-dominant immunoreaction, the development of MCD in IgG4-RD patients may reflect more than a mere coincidence.
Insights
Immunoglobulin G4-related disease (IgG4-RD) can present with minimal change disease (MCD), a rare kidney disorder. This case highlights a potential link, suggesting shared immune pathways may contribute to both conditions.
Area of Science:
- Nephrology
- Immunology
- Pathology
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition.
- Minimal change disease (MCD) is a common cause of nephrotic syndrome in adults.
- The co-occurrence of IgG4-RD and MCD is infrequently reported.
Observation:
- A 66-year-old male with a history of IgG4-RD developed sudden massive proteinuria and weight gain, indicative of nephrotic syndrome.
- Laboratory results showed no elevation in serum IgG4 levels or hypocomplementemia.
- Renal biopsy findings were consistent with Minimal Change Disease, including foot process effacement on electron microscopy.
Findings:
- The patient was diagnosed with Minimal Change Disease (MCD) despite having a history of IgG4-related disease (IgG4-RD).
- Treatment with prednisolone rapidly resolved proteinuria, confirming the diagnosis of MCD.
- The absence of elevated IgG4 or hypocomplementemia in this presentation is noteworthy.
Implications:
- The shared T-helper 2-dominant immunoreaction between IgG4-RD and MCD suggests a potential pathophysiological link.
- This case underscores the importance of considering MCD in IgG4-RD patients presenting with nephrotic syndrome.
- Further research into the immunological overlap may reveal novel therapeutic targets for both conditions.
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