| 초록 |
Case Study: Background IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder characterized by lymphoplasmacytic infiltration rich in IgG4-positive plasma cells and involvement of multiple organs. Renal involvement most commonly presents as tubulointerstitial nephritis; however, glomerular lesions such as membranous nephropathy have also been reported. In some patients, renal manifestations may precede the diagnosis of systemic IgG4-RD by several years. Case presentation A 52-year-old man underwent kidney biopsy in 2008 for nephrotic-range proteinuria. Histopathologic examination revealed membranous glomerulonephritis, stage II. Anti-phospholipase A2 receptor (PLA2R) antibody was negative. He was also diagnosed with type 2 diabetes mellitus in 2008. The patient was treated with cyclosporine and angiotensin receptor blocker therapy and remained under long-term follow-up. Blood glucose was controlled conservatively using metformin, sulfonylureas, and a DPP-4 inhibitor. In 2026, abdominal computed tomography revealed multiple enlarged lymph nodes in the greater omentum and retroperitoneal region. Excisional biopsy of a greater omental lymph node demonstrated follicular hyperplasia with dense infiltration of IgG4-positive plasma cells, findings suggestive of IgG4-related disease. Based on these results, the previously diagnosed membranous glomerulonephritis was considered a possible early renal manifestation of IgG4-RD. Although steroid treatment was recommended, the patient refused pharmacological intervention, and conservative management is currently being pursued. In patients with PLA2R-negative membranous nephropathy, clinicians should consider secondary causes including IgG4-related disease and maintain long-term surveillance for systemic manifestations. Conclusion This case illustrates that PLA2R-negative membranous glomerulonephritis may be related with systemic IgG4-related disease by many years. |