Rituximab treatment in resistant lupus nephritis: A single-center prospective study.

BACKGROUND: Lupus nephritis (LN) is a serious manifestation of systemic lupus erythematosus (SLE) and failure to respond to traditional immunosuppression increases morbidity and mortality. Rituximab has been considered a novel therapeutic option for the management of SLE. MATERIALS AND METHODS: We conducted a single-center, prospective, observational study from July 2018 to June 2019 to evaluate the effectiveness of rituximab in patients with resistant LN. Resistant LN was defined as the failure to respond to conventional immunosuppressive therapy including both cyclophosphamide and mycophenolate mofetil. All adult patients (> 18 years) with biopsy-proven class III/IV LN were included in the study. Four doses of intravenous rituximab (375 mg/m2) on 0, 1, 2, 3 weeks were administered. Patients were followed for 6 months, and the rates of complete renal response (CRR), partial renal response (PRR), or no renal response (NRR) were measured. The change in baseline 24-hour urine protein, mean serum creatinine levels, and mean serum CD-19 levels at 24 weeks were also measured. RESULTS: Six months after rituximab therapy, total sustained renal response (CRR+PRR) was observed in 52% cases of resistant LN (CRR was achieved in 24% of patients and PRR in 28%, respectively). Rituximab was associated with a significant decline in the 24-hour urine protein, even in non-responders. However, the improvement in eGFR and serum creatinine was not statistically significant. The mean absolute CD-19 count was significantly low in responders compared to the non-responder group. CONCLUSION: Rituximab is a safe and effective therapeutic strategy for patients with resistant LN.

Idiopathic Nonlupus Full-House Nephropathy: Clinicopathological Presentation and Comparison with Lupus Full-House Nephropathy.

Full-house pattern on immunofluorescence (IF) on kidney biopsy in a patient without systemic lupus erythematosus is termed as nonlupus full-house nephropathy (FHN). In this study, we retrospectively compiled patients with nonlupus FHN and compared them with lupus FHN for clinicopathological presentation. We included patients with full-house IF patterns in renal biopsies collected from March 2007 to August 2018, clinical and histopathological data at the time of presentation were studied retrospectively. Treatment received and outcome at the end of follow-up was studied. Patients with nonlupus FHN who did not show any systemic disease (idiopathic group) were compared with a group of lupus nephritis patients. Of 178 patients, 34 had nonlupus FHN with 21 having idiopathic nonlupus FHN and 13 patients having secondary nonlupus FHN (membranous nephropathy, IgA nephropathy, postinfection glomerulonephritis). Males were more often in idiopathic nonlupus FHN patients than lupus FHN patients (P = 0.005). Kidney biopsies more often showed a mesangial (P = 0.0006) and less proliferative pattern of injury (P = 0.0002) and less intense C1q staining (P = 0.0001) in idiopathic nonlupus than lupus FHN. Clinically, idiopathic nonlupus FHN presented with more proteinuria (P = 0.0059) and less complement consumption (P = 0.001) than lupus FHN patients. Compared to lupus FHN, nonlupus has mainly nephrotic syndrome as clinical presentation. There was no difference in the clinical outcome between lupus FHN and idiopathic nonlupus FHN. Nonlupus FHN is not a very common condition and has less female involvement than in lupus FHN. Idiopathic nonlupus FHN has certain histopathological features with less C1q staining by IF, less frequent proliferative lesions and higher mesangial or membranous lesions by light microscopy compared to lupus FHN. Regarding outcomes, there is no significant difference between lupus FHN and idiopathic nonlupus FHN.

Characteristics, maternal and neonatal outcomes of acute kidney injury in preeclampsia: A prospective, single-center study.

BACKGROUND: Preeclampsia is an important cause of pregnancy-related acute kidney injury (AKI). The objective of our study was to determine the incidence, characteristics, and maternal and neonatal outcomes of AKI in pregnant women with preeclampsia. MATERIALS AND METHODS: A prospective, observational, single-center study from January 2019 to January 2020. Patients admitted with preeclampsia were included. Patients with obstetric complications were excluded. AKI was defined according to Kidney Disease Improving Global Outcomes (KDIGO) 2012 criteria. RESULTS: Total number of patients with preeclampsia was 104, out of which 25% developed AKI. Among those with AKI, nulliparity (61.5%) was the most common risk factor for preeclampsia followed by prior history of preeclampsia (15.4%), pregestational hypertension (11.5%), pregestational diabetes mellitus (3.8%), and chronic kidney disease (3.8%). There was no significant difference in maternal mortality between those with AKI (15.4%) and without AKI (7.7%). Intermittent hemodialysis was needed in 15.4%. At the end of 90 days follow-up, complete recovery of renal function occurred in 53.8%, partial recovery in 23.1% and end-stage kidney disease (ESKD) in 7.7%. Perinatal death occurred in 26.9%, preterm birth in 23.1% and stillbirth in 7.7% of those with AKI and was not significantly different from those without AKI. The mean of birth weight in newborns delivered by patients with AKI (2.53 ± 0.73 kg) was significantly lower compared to those without AKI (2.82 ± 0.58 kg). CONCLUSION: AKI was associated with a lower mean birth weight of newborns. Complete recovery of renal function was seen in 53.8% of patients with AKI and preeclampsia.

Histological patterns of renal diseases in children: A 12-year experience from a single Tertiary Care Center in North-East India.

This study was conducted to retrospectively investigate the indications for renal biopsy in the native kidneys of children and to analyze the pathological findings in a single tertiary care hospital in North-East India for the past 12 years. All children (≤18 years) who underwent renal biopsy at our hospital from March 2007 to April 2018 were included. Renal tissue specimens were studied under light and immunofluorescence microscopy. The study group included 254 patients (female 57%). The median age was 15 years (range 6-18 years). The most frequent indications for renal biopsy were nephrotic syndrome (NS) (53.9%), urinary abnormality in systemic disease (22.1%), nephritic syndrome (15.4%), asymptomatic hematuria (4.7%), significant proteinuria (3.1%), and unexplained renal failure (0.8%). On histopathological examination, primary glomerular diseases were the most frequent (68.9%) followed by secondary glomerular diseases (30.3%) and tubulointerstitial diseases (0.8%). The most common primary glomerular diseases were minimal change disease (26.8%), focal segmental glomerular sclerosis (12.2%), diffuse proliferative glomerulonephritis (9.1%), membranous nephropathy (8.7%), IgA nephropathy (8.3%), membranoproliferative glomerulonephritis (2%), and mesangioproliferative glomerulonephritis (2%). Lupus nephritis (LN) (29.5%) was the most common secondary glomerular disease. NS was the most common indication of renal biopsy, and LN was the most common histopathological diagnosis in children ≤18 years.

A Comparable Study on the Outcomes of Urgent Initiated Peritoneal Dialysis Versus Conventional Start, A Single Centre Study from North-East India.

INTRODUCTION: To avoid temporary hemodialysis, urgent initiated PD (UIPD) has been designed. In these patients, PD is initiated within 3 days after PD catheter placement. In this study, we evaluated the outcomes of UIPD in end-stage renal disease patients compared with the conventional start of PD. METHODS: This is a single-center observational study, comparing outcomes of UIPD to conventional initiation of PD. All patients diagnosed with ESRD from March 2013 to February 2019 and were willing for CAPD were recruited. In UIPD group treatment was initiated at day 2 of catheter insertion with a dialysate volume of 1000 mL per dwell for 2 hours gradually increased to 2000 mL per dwell volume by 8 to 10 days. RESULTS: During the study period, 98 patients were started on peritoneal dialysis in our hospital: 35 UIPD, 63 conventional PD. The mean age was 60.81 ± 13.04 years. 67% of patients were males with diabetes mellitus (32%) being the most common cause of CKD. Among the patients in UIPD, the mean age was 58.49 ± 16.1 years, while as in conventional group mean age was 62.10 ± 10.9 years. The Median follow-up time was 381 days. Technique survival was seen in 95 patients (96.9%). There was no difference in technique failure between UIPD vs conventional group. Total complications in our study occurred in 16 patients out of 98 patients during this period. There was no significant difference in the complication rates between the UIPD group and the conventional group. CONCLUSION: Our study showed that catheter patency, technique survival, and catheter-related complications were comparable between UIPD and conventional start peritoneal dialysis.

Prevalence of Nondiabetic Renal Disease in Patients with Type 2 Diabetes Mellitus with Clinicopathological Correlation: A Study from a Tertiary Care Center of Assam, India.

Diabetes mellitus is the most common cause of chronic kidney disease worldwide. The prevalence of nondiabetic renal disease (NDRD) among patients with type 2 diabetes mellitus (T2DM) varies widely. This study aimed to evaluate the renal biopsies performed on type 2 diabetic patients for suspicion of NDRD and to correlate clinicopathological findings. All T2DM patients aged > 18 years were included in this study, who had renal biopsy performed for the following reasons: recent-onset nephrotic syndrome, unexplained rapid deterioration of renal function, proteinuria not accompanied by retinopathy, and unexplained hematuria. Renal biopsy was analyzed by light microscopy and immunofluorescence. Based on biopsy findings, the patients were grouped into three: (i) isolated NDRD, (ii) NDRD ± diabetic nephropathy (DN), and (iii) isolated DN. A total of 140 patients were enrolled in this study. Recent-onset nephrotic syndrome was the most common indication for biopsy, followed by the presence of active urine sediment. Forty-two percent of the patients had isolated DN, while NDRD was seen in 34% and DN ± NDRD in 24%. Focal segmental glomerulosclerosis (FSGS) and IgA nephropathy were the most common causes of isolated NDRD, while chronic tubulointerstitial nephritis (CTIN) was common in NDRD plus DN. Short duration of diabetes, absence of diabetic retinopathy, and lower glycated hemoglobin were predictive of NDRD. NDRD was seen in 58% of the patients with atypical presentations. FSGS and CTIN were common in NDRD diseases. Judicious use of biopsy in diabetic patients with atypical presentation may help in the diagnosis of NDRD.

Clinical and histopathological profile of lupus nephritis and response to treatment with cyclophosphamide: A single center study.

Lupus nephritis (LN) is an immune-complex glomerulonephritis that is usually manifested by proteinuria, active urinary sediment, hypertension, and renal failure. The objective of this study is to study the clinical and histopathological profile of LN and the response to treatment with cyclophosphamide. This was a retrospective study conducted in a tertiary care center in Assam, India, where 176 LN patients who underwent renal biopsy were included. The presenting features, laboratory parameters such as proteinuria, hematuria, and the histopathological class of the patients were studied. Among the 176 patients, 89.8% were female and 10.2% were male and maximum patients (61.3%) were in the age group of 20-40 years. Pedal edema was present in 100% of the patients, decreased urine output in 43.7%, malar rash in 38%, joint pain in 42%, hair loss in 63%, hypertension in 41.4%, oral ulcers in 31.8%, seizures in 17%, psychosis in 13%, hematuriain 78.4%, anemia in 72.1%, thrombocytopenia in 51.1%, and leukopenia in 31.7% of patients. The anti-nuclear antibody was positive in all patients and anti-dsDNA was positive in 70.5% of the patients. The most common histopathological type was class IV (50%), followed by class III (17.6%). One hundred and two patients received intravenous cyclophosphamide as initial treatment of whom, 40 received the Eurolupus regimen and 62 received the NIH regimen. The number of patients who underwent remission in both the regimen was compared. The response rate of initial treatment with cyclophosphamide in the Eurolupus group was 62.5% and in the NIH group was 64.5% (P >0.05). Majority of the patients had proliferative LN in this study, of which class IV was the most common. Proliferative LN, if not detected and treated early, leads to poor outcome in terms of patient and renal survival. Hence, patients with systemic lupus erythematosus should be evaluated for kidney involvement and treated accordingly for better outcome.

Focal and segmental glomerulosclerosis in renal allograft recipients: a clinico-pathologic study of 37 cases.

Focal and segmental glomerulosclerosis (FSGS) in renal allografts may occur as a recurrence of primary FSGS, as a de novo phenomenon or as a complication of calcineurin inhibitor toxicity. There are very few studies in the literature describing the clinico-pathologic characteristics of FSGS in renal allografts. To the best of our knowledge, no such study exists from the Indian subcontinent. Thirty-seven cases showing FSGS, of 426 transplant biopsies performed over a 4-year period (2006-2009), were included in this study. The pre- and post-transplant clinical data were noted. FSGS was classified as per the Columbia scheme. Appropriate statistical tests were applied. The age of the study patients ranged from 13 to 54 years, with a male preponderance. Thirty-five patients (94.6%) were diagnosed as FSGS more than 12 months after transplantation. All the patients presented with renal dysfunction (median serum creatinine 2.8 mg/dL) and detectable proteinuria at the time of diagnosis. Histologically, FSGS-NOS (70.3%) was observed as the most common subtype, followed by collapsing and perihilar varieties (13.5% each). Most of the biopsies (83.7%) showed grade-2 to -3 interstitial fibrosis and tubular atrophy. Follow-up data were available in 27 patients (73%), of whom 12 (44.4%) had graft loss with dialysis-dependent state at last follow-up. FSGS is one of the important causes of graft dysfunction, especially late in the post-transplantation period in cases of de novo FSGS. The long-term outcome of renal allografts developing this glomerular pathology is quite dismal, with a significant proportion of patients suffering graft loss.

Focal segmental glomerulosclerosis in idiopathic membranous glomerulonephritis: a clinico-pathological and stereological study.

BACKGROUND: The phenomenon of focal segmental glomerulosclerosis (FSGS) in idiopathic membranous glomerulonephritis (IMGN) has not been adequately studied. There is also a paucity of detailed glomerular morphometric and stereologic analyses data on renal biopsy in this association. METHODS: Twenty-three (23) patients with IMGN and superimposed FSGS were compared to 35 patients with IMGN alone with respect to the clinical and laboratory features, light microscopic findings and stereologic parameters (glomerular cross-sectional area and estimated glomerular volume). RESULTS: In the clinical parameters, patients with IMGN-FSGS had a significantly higher incidence of hypertension, raised serum creatinine and microscopic haematuria. The mean 24-h urinary protein excretion was higher in the group with IMGN-FSGS (7.4 +/- 1.36 g) as compared to IMGN alone (3.85 +/- 0.7 g, P < 0.001, Mann-Whitney test). On light microscopy, biopsies with IMGN-FSGS frequently had mesangial hypercellularity and more extensive tubulo-interstitial disease than those with IMGN alone. Stereological analysis showed that the non-sclerosed glomeruli in biopsies with IMGN-FSGS had a higher mean cross-sectional area (185466.7 +/- 32493.3 micro(2)) and higher estimated volume (855200 +/- 152640 micro(3)) as compared to glomeruli in cases with IMGN alone (76000 +/- 14719.2 micro(2) and 576666.7 +/- 131233.3 micro(3), respectively). CONCLUSION: The present study is probably the first systematic analysis of stereologic parameters in renal biopsies of IMGN with FSGS. Our results objectively demonstrate the glomerular enlargement in the non-sclerosed glomeruli in cases of IMGN with FSGS. This detection of enlarged glomeruli may serve to alert the renal pathologist to the possibility of coexisting FSGS, which is a poor prognostic factor in IMGN.