A case of proliferative glomerulonephritis with monoclonal IgG3κ deposits accompanied by glomerular capillary microaneurysms.

Glomerular capillary aneurysms are distinctly rare and specific glomerular lesions characterized by aneurysmal dilatation of the glomerular capillaries. This formation is associated with glomerular capillary injuries with focal mesangiolysis. Here, we report a case of proliferative glomerulonephritis with monoclonal immunoglobulin G deposits (PGNMID) presenting with multiple glomerular capillary microaneurysms. A 53-year-old woman presented with persistent proteinuria and microhematuria. She had no underlying diseases, such as hematopoietic or lymphoproliferative disorders. A renal biopsy showed diffuse membranoproliferative lesions with foam cell infiltration and multiple microaneurysms of the glomerular capillary on light microscopy. Immunofluorescence analysis showed granular deposits of monoclonal immunoglobulin G3 kappa (IgG3κ), C1q, C3, and C4 in the glomeruli. Electron microscopy revealed different sizes of non-organized electron-dense deposits in the mesangial, subendothelial, and subepithelial areas. In addition, glomerular endothelial cells showed swelling and loss of fenestra or diffuse formation of fenestrated diaphragms, accompanied by irregular thinning of the glomerular basement membrane. Furthermore, immunostaining for CD31 (a marker for endothelial cell) and low-vacuum scanning electron microscopy study identified loss of endothelial cells in microaneurysm, suggesting severe glomerular endothelial cell injury. After a renal biopsy, only the medication for dyslipidemia was continued because there were no physical symptoms, such as edema, and urinary abnormalities continued with stable renal function. Further studies are needed to elucidate the pathogenesis of glomerular capillary injury in PGNMID and clarify the clinical and pathological characteristics of PGNMID with glomerular capillary microaneurysms.

Anemia and atrial fibrillation as independent risk factors for new-onset chronic kidney disease: the TAMA-MED Project-CKD and AF.

BACKGROUND: Various risk factors have been identified for the new onset or rapid deterioration of chronic kidney disease (CKD). However, it is thought that many risk factors that have not yet been clarified remain. METHODS: Based on the results of specific annual health checkups at Tama City (n = 18 383) in 2017 and 2018, we analyzed the factors that cause new-onset CKD and the risk factors that rapidly worsen renal function. For new-onset CKD, proteinuria and estimated glomerular filtration rate (eGFR) <60 mL/min/1.73 m2 were examined separately. Rapid deterioration of renal function was defined as an eGFR ≥25% less than the previous year. RESULTS: Multivariate analysis showed that in addition to age and impaired glucose tolerance, anemia and atrial fibrillation (AF) were risk factors for the new appearance of proteinuria. Risk factors for a decrease in eGFR to <60 mL/min/1.73 m2 were age and hyperuricemia. Age, systolic hypertension, urinary protein and urinary occult blood, high triglycerides and anemia were significant risk factors for the rapid deterioration of renal function in patients with CKD Stage ≥3. CONCLUSIONS: From the results of specific annual health checkups at Tama City, AF, anemia and hyperuricemia were identified as risk factors for new-onset CKD over a short period of 1 year. Anemia was also a factor for the rapid deterioration of kidney function in subjects with renal dysfunction.

Effective improvement of minimal change nephrotic syndrome with uncontrollable high low-density lipoprotein cholesterol level using evolocumab accompanied by the development of acute pancreatitis.

Nephrotic syndrome is sometimes refractory; however, it is rarely accompanied by acute pancreatitis. A 47-year-old Japanese woman complaining of limb edema was diagnosed with nephrotic syndrome. Blood and urine examinations suggested minimal change nephrotic syndrome (MCNS), and pulse intravenous methylprednisolone was administered, followed by oral prednisolone. Although proteinuria improved, the patient's condition remained unchanged, and diuresis was insufficient. As in patients with other nephrotic syndromes, this patient showed significant dyslipidemia. Atorvastatin was started for remarkable dyslipidemia since her admission, but her low-density lipoprotein cholesterol (LDL-C) level did not improve significantly. During the clinical course, she developed acute pancreatitis, and large-volume fluid replacement was performed. Although diuretic levels were increased in response to the increased fluid volume, diuresis was not enough, and lung edema developed. Extracorporeal ultrafiltration was started to ameliorate the lung edema. With the onset of pancreatitis, oral intake, including atorvastatin, was discontinued, and prednisolone was administered intravenously. To treat the high-LDL cholesterolemia, 140 mg of evolocumab was injected subcutaneously. Nausea slightly decreased on the following day, and the administration of 150 mg cyclosporine was initiated. LDL-C levels, proteinuria, and renal function promptly ameliorated. The results of a renal biopsy suggested MCNS. On the 44th day of hospitalization, she had complete remission. Evolocumab is potentially effective for severe nephrotic syndrome with uncontrollable dyslipidemia.