Journal of Diagnostics Concepts & Practice >
Analysis of pathological features related to clinical prognosis in C3 glomerulopathy
Received date: 2024-06-27
Online published: 2024-12-25
Objective To analyze the pathological features associated with the clinical prognosis of C3 glomerulopathy (C3G). Methods A total of consecutive 7 cases, all hospitalized in the Department of Nephrology, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine between 2012 and 2022, and histologically diagnosed with C3G by renal biopsy, were collected. Their clinical, pathological, and follow-up data were retrospectively analyzed to observe pathological characteristics related to prognosis. Results The cohort consisted of 7 C3G patients (male/female: 3/4). The median age at onset was 51 years, and the median age at diagnosis was 54 years. The median follow-up time was 84 months (range: 48-144 months). Two patients (Case 1, Case 7) were lost to follow-up. Two patients (Case 5, Case 6) had a poor prognosis, while three patients (Case 2-4) had a favorable prognosis. Clinically, 2 patients (Case 3, Case 7) presented with nephrotic syndrome, 3 patients (Case 1, Case 3, Case 6) had renal insufficiency, 6 patients (Case 1-6) had hematuria, and all 7 patients exhibited hypocomplementemia and hypertension, without significant extrarenal manifestations.Among the 5 patients with long-term follow-up, at the 2-year mark: 1 patient (Case 3) achieved complete remission of proteinuria and serum creatinine; 3 patients (Case 2, Case 4, Case 5) had stable proteinuria and renal function; and 1 patient (Case 6) showed increased proteinuria and declining renal function. At the 6-year follow-up: 2 patients (Case 2, Case 4) maintained a good prognosis; 1 patient (Case 3) was no longer followed up after achieving complete remission of proteinuria and creatinine; 1 patient (Case 6), who already had a poor prognosis at 2 years, exhibited a further increase in proteinuria and a significant decline in renal function; and 1 patient (Case 5) newly progressed to heavy proteinuria.At the 6-year follow-up, a review of renal pathology revealed that the 3 cases with a favorable prognosis (Case 2-4) primarily showed diffuse capillary endothelial cell hyperplasia with neutrophil infiltration on light microscopy, while global glomerulosclerosis and glomerular basement membrane pseudo-double contour formation were not prominent. Electron microscopy demonstrated relatively few electron-dense deposits in the mesangial region. In contrast, the 2 cases with a poor prognosis (Case 5, Case 6) were characterized by an increased proportion of global glomerulosclerosis[ greater than (age-20)/2 × 100%] ,extensive glomerular basement membrane pseudo-double contours on light microscopy, and abundant mesangial electron-dense deposits on electron microscopy, with minimal capillary endothelial cell hyperplasia and neutrophil infiltration. Conclusions C3G patients whose histopathology is predominantly characterized by acute lesions (such as glomerular capillary endothelial cell hyperplasia and neutrophil infiltration) have a more favorable prognosis and can be treated aggressively with corticosteroids and immunosuppressants. Conversely, C3G patients whose histopathology is predominantly characterized by chronic lesions (such as global glomerulosclerosis, glomerular basement membrane pseudo-double contour formation, and a high quantity of mesangial electron-dense deposits) have a poor prognosis, and conservative management is recommended to avoid overtreatment.
Key words: C3 glomerulopathy; Clinical manifestation; Renal pathology; Prognosis
ZHANG Junhua , LI Yilin , XIE Jingyuan , ZHANG Chunli , XU Jing . Analysis of pathological features related to clinical prognosis in C3 glomerulopathy[J]. Journal of Diagnostics Concepts & Practice, 2024 , 23(06) : 587 -593 . DOI: 10.16150/j.1671-2870.2024.06.005
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