Clinicopathological Significance and Renal Outcomes of Light Microscopic Patterns in Complement Component 3 Glomerulopathy
Clinicopathological Significance and Renal Outcomes of Light Microscopic Patterns in Complement...
Wani, Asif Sadiq; Zahir, Zafirah; Gupta, Amit; Agrawal, Vinita
2020-05-01 00:00:00
Background: Complement component 3 glomerulopathy (C3G) is a disease diagnosed based on the predominance of C3 immunostaining in glomeruli. The popular electron microscopic subtyping of C3G into dense deposit disease and C3 glomerulonephritis (GN) is not without limitations. We aimed to study the light microscopic (LM) patterns of C3G along with their clinicopathological correlation and treatment outcome. Methods: C3G biopsies were classified into 4 LM patterns (membranoproliferative GN [MPGN], mesangial proliferative GN [MesPGN], diffuse proliferative GN [DPGN], and crescentic GN [CrGN]). These patterns were compared for clinicopathological profile, treatment outcome, and renal survival. Further, predictors of end-stage renal disease (ESRD) were determined using the Cox proportional hazards model. Results: Of 162 biopsies, there were 83 MPGN, 36 DPGN, 22 MesPGN, and 21 CrGN. Majority of the patients were young, with males being more than females. About half (48%) of the patients received immunosuppression, steroids alone (29%) or steroids with other immunosuppressants (19%). The overall remission rate was 32.7% (median follow-up = 14 months). CKD developed in 46 patients and 31 patients progressed to ESRD. Predictors of progression to ESRD were older age (hazard ratio [HR] = 1.04, p < 0.01), advanced renal failure at presentation (HR = 3.73, p < 0.01), glomerulosclerosis (HR = 5.07, p < 0.01), and severity of interstitial fibrosis and tubular atrophy (HR = 8.25, p = 0.01). Conclusions: The LM patterns differed in their clinicopathological profiles, without any significant difference in their renal outcomes. Glomerulosclerosis and interstitial fibrosis portend a poor prognosis. Besides CrGN, MesPGN pattern of C3G presented as a severe form of disease.
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Clinicopathological Significance and Renal Outcomes of Light Microscopic Patterns in Complement Component 3 Glomerulopathy
Background: Complement component 3 glomerulopathy (C3G) is a disease diagnosed based on the predominance of C3 immunostaining in glomeruli. The popular electron microscopic subtyping of C3G into dense deposit disease and C3 glomerulonephritis (GN) is not without limitations. We aimed to study the light microscopic (LM) patterns of C3G along with their clinicopathological correlation and treatment outcome. Methods: C3G biopsies were classified into 4 LM patterns (membranoproliferative GN [MPGN], mesangial proliferative GN [MesPGN], diffuse proliferative GN [DPGN], and crescentic GN [CrGN]). These patterns were compared for clinicopathological profile, treatment outcome, and renal survival. Further, predictors of end-stage renal disease (ESRD) were determined using the Cox proportional hazards model. Results: Of 162 biopsies, there were 83 MPGN, 36 DPGN, 22 MesPGN, and 21 CrGN. Majority of the patients were young, with males being more than females. About half (48%) of the patients received immunosuppression, steroids alone (29%) or steroids with other immunosuppressants (19%). The overall remission rate was 32.7% (median follow-up = 14 months). CKD developed in 46 patients and 31 patients progressed to ESRD. Predictors of progression to ESRD were older age (hazard ratio [HR] = 1.04, p < 0.01), advanced renal failure at presentation (HR = 3.73, p < 0.01), glomerulosclerosis (HR = 5.07, p < 0.01), and severity of interstitial fibrosis and tubular atrophy (HR = 8.25, p = 0.01). Conclusions: The LM patterns differed in their clinicopathological profiles, without any significant difference in their renal outcomes. Glomerulosclerosis and interstitial fibrosis portend a poor prognosis. Besides CrGN, MesPGN pattern of C3G presented as a severe form of disease.
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