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Anti-glomerular basement (anti-GBM) disease is an uncommon disorder with a bimodal age of presentation. Patients presenting with dialysis-dependent renal failure have poor renal outcomes. There is limited data regarding the clinical presentation and outcomes of anti-GBM disease from India. We conducted this prospective study to analyze the clinical presentation and outcomes of anti-GBM disease at a large tertiary care hospital in North India over 1 1/2 years. Subjects with a biopsy proven anti-GBM disease (light microscopic examination showing crescents and immunofluorescence examination showing linear deposition of IgG) with or without positive anti-GBM antibodies in serum were included in the study and followed-up for at least 12 months. All the patients were treated with steroids, cyclophosphamide, and plasma exchange. A total of 17 patients (nine males) were included. The mean age at presentation was 39.11 ± 16.58 (range 11-72) years. Twelve patients (70%) presented with rapidly progressive glomerulonephritis (RPGN), 4 (23.5%) presented with Goodpasture syndrome, while 1 (5.8%) had nephritic syndrome, 7 (41%) were hypertensive, and 14 (82.3%) required dialysis at the time of presentation. Four patients (23.5%) had associated anti-neutrophil cytoplasmic antibody positivity (anti-myeloperoxidase antibodies in all). Fourteen (87.5%) patients had crescentic glomerulonephritis, while 5 (31.25%) showed necrotizing (n = 4) or granulomatous (n = 1) in the vasculitis. Of 16 patients who received treatment, four (23.25%) achieved complete remission. In this single-center study, the majority of anti-GBM disease patients presented with RPGN and had crescentic glomerulonephritis on biopsy with poor treatment outcome. [ABSTRACT FROM AUTHOR]

The aim was to evaluate the role of α-smooth muscle actin (SMA) and collagen type IV as markers of chronicity in renal biopsies of IgA nephropathy patients and to correlate the degree of their interstitial expression with renal function as judged by serum creatinine. Renal biopsies from 29 clinically, histologically and immunologically confirmed cases of IgA nephropathy were reviewed to assess activity and chronicity indices. Immunohistochemical stains for α-SMA and collagen type IV was performed on 23 patients with adequate tissue available in the block. The interstitial expression of α-SMA and collagen type IV was then correlated with chronicity and activity indices, serum creatinine and 24 hours urinary protein. Pearson's coefficient of correlation, unpaired-t test were used for statistical analysis. α-SMA and collagen type IV were shown to be expressed in the interstitium in all 22 cases showing interstitital fibrosis. Both showed a similar distribution pattern with predominant periglomerular and peritubular positivity. The cases were divided into two groups (low and high grade) depending on the percentage of interstitial area showing positivity for these two antibodies. On statistical analysis, the expression of both α-smooth muscle actin and collagen type IV showed a striking correlation with the histological chronicity index (P < 0.01). A positive correlation was also noted with the serum creatinine at the time of diagnosis. It is seen that an immunohistochemical approach to grading interstitial fibrosis as in this study is far simpler than the histological grading systems prevalent and is an important baseline prognostic indicator. [ABSTRACT FROM AUTHOR]

The morphological spectrum of light chain deposition disease (LCDD) may range from normal glomerular morphology to mesangio-proliferative to mesangio-capillary to nodular sclerosing patterns. Due to the inconsistencies regarding treatment and the universally poor graft outcome of post-transplant LCDD, it is imperative to maintain a high index of suspicion and perform relevant investigations for clinching this diagnosis. A 40-year-old lady was diagnosed as a case of membrano-proliferative glomerulonephritis 3 years back, for which she underwent a live unrelated renal allograft transplant. Postoperative period was complicated by an acute rise in serum creatinine on the 21st postoperative day. Biopsy showed patchy acute cortical necrosis, which responded to conservative management. The present admission was for renal failure and subnephrotic proteinuria. A kidney biopsy was performed, and all the 14 glomeruli examined showed a mesangiocapillary pattern of glomerular injury with cellular nodule formation in some. The nodules were PAS and Congo red negative. Immunofluorescence showed glomerular and tubular basement staining for Kappa light chains only. Electron microscopy showed the characteristic granular deposits in subendothelial location in the glomerulus, and in tubular basement membranes, thus confirming the diagnosis of LCDD. Membranoproliferative pattern of glomerular injury in the pre- and posttransplant setting has a wide range of differential diagnoses; LCDD being one of them. [ABSTRACT FROM AUTHOR]

The aim was to evaluate the role of a-smooth muscle actin (SMA) and collagen type IV as markers of chronicity in renal biopsies of IgA nephropathy patients and to correlate the degree of their interstitial expression with renal function as judged by serum creatinine. Renal biopsies from 29 clinically, histologically and immunologically confirmed cases of IgA nephropathy were reviewed to assess activity and chronicity indices. Immunohistochemical stains for alpha-SMA and collagen type IV was performed on 23 patients with adequate tissue available in the block. The interstitial expression of alpha-SMA and collagen type IV was then correlated with chronicity and activity indices, serum creatinine and 24 hours urinary protein. Pearson's coefficient of correlation, unpaired-t test were used for statistical analysis. alpha-SMA and collagen type IV were shown to be expressed in the interstitium in all 22 cases showing interstitital fibrosis. Both showed a similar distribution pattern with predominant periglomerular and peritubular positivity. The cases were divided into two groups (low and high grade) depending on the percentage of interstitial area showing positivity for these two antibodies. On statistical analysis, the expression of both a-smooth muscle actin and collagen type IV showed a striking correlation with the histological chronicity index (P<0.01). A positive correlation was also noted with the serum creatinine at the time of diagnosis. It is seen that an immunohistochemical approach to grading interstitial fibrosis as in this study is far simpler than the histological grading systems prevalent and is an important baseline prognostic indicator.