Background and Aims:
Full-house immunofluorescence in a kidney biopsy is a common observation in lupus nephritis (LN) and was previously used synonymously with the diagnosis of LN. Though a minority of the patients will develop features suggestive of SLE during follow-up, a majority of the patients will continue without any clinical or serological evidence of systemic lupus erythematosus (SLE) over time. Our aim to conduct this study was to work up the clinicopathological spectrum of these “full-house” nephropathies (FHN) which were not due to lupus nephritis.
Methods:
A total of 6244 renal biopsies were evaluated at SGPGIMS Lucknow from January 2007 to December 2017 for full-house immunofluorescence. All those patients who had no clinical or serological evidence of SLE at the time of renal biopsy or at any time during follow up were included.
Results:
Among 498 patients with full house immunofluorescence, 81 patients had no clinical or serological evidence of SLE at the time of renal biopsy or at any time during follow up. The prevalence of non-lupus FHN in this study was 19.4%, and the major diagnoses were membranous nephropathy (25.9%), IgAN (22.2%), MPGN (14.8%), DPGN (12.3%), Crescentic GN (12.3%), Amyloidosis (8.6%), C1q nephropathy (3.7%).
Conclusions:
Full-house nephropathy (FHN), not otherwise suggestive of lupus nephritis, can also be found in a number of other conditions. Non-lupus full house nephropathy is an umbrella term for such cases which do not satisfy the standard criteria of SLE. This will prevent misclassifying these patients into SLE and further prevent them from unnecessary immunosuppression protocols.
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.
Background:
Glomerular diseases vary with age, and it is important to investigate the spectrum of glomerular diseases in pediatric patients to help in a more precise clinical diagnosis and optimize the management of patients. We aimed to study the clinicopathologic pattern of pediatric glomerular diseases in North India.
Methods:
This is a 5-year retrospective, single-center cohort study. The database was searched to identify all pediatric patients with glomerular diseases in their native kidney biopsies.
Results:
About 2890 native renal biopsies were studied, of which 409 were pediatric glomerular diseases. The median age was 15 years with a male preponderance. Nephrotic syndrome was the most common presentation (60.8%), followed by non-nephrotic proteinuria with hematuria (18.5%), rapidly proliferative glomerulonephritis (7%), isolated hematuria (5.3%), acute nephritic syndrome (3.4%), non-nephrotic proteinuria (1.9%), and advanced renal failure (0.7%). Minimal change disease (MCD) was the most common histological diagnosis, followed by focal segmental glomerulosclerosis (17.4%), IgA nephropathy (IgAN; 10%), membranous nephropathy (6.6%), lupus nephritis (5.9%), crescentic glomerulonephritis (2.9%), and C3 glomerulopathy (2.9%). Diffuse proliferative glomerulonephritis (DPGN) was the most common histological diagnosis in patients with hematuria and non-nephrotic as well as nephrotic range proteinuria. The most common histological diagnoses for isolated hematuria and acute nephritic syndrome were IgAN and postinfectious glomerulonephritis (PIGN), respectively.
Conclusions:
MCD and lupus nephritis are the most common pediatric primary and secondary histopathologic diagnoses, respectively. The adolescent-onset glomerular diseases have a higher frequency of IgAN, membranous nephropathy, and DPGN. PIGN is still an important differential in our pediatric patients presenting with acute nephritic syndrome.
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