Maternal and fetal outcomes in pregnancy complicated by pre-existing lupus nephritis: Insights from a developing country Pakistan.

BACKGROUND: Pregnancy in women with systemic lupus erythematosus (SLE) has remained a great challenge for clinicians in terms of maternal and fetal outcomes. The outcomes in women with pre-existing lupus nephritis (LN) are variable. The impact of different classes of LN on maternal and fetal outcomes during pregnancy is not well defined, as data is very scarce, especially from the developing countries. METHODS: A retrospective analysis was conducted on 52 women with 89 pregnancies. All had biopsy-proven LN. Those women who conceived at least 6 months after the diagnosis were included. The analysis was conducted between July 1998 and June 2018 at Sindh Institute of Urology and Transplantation (SIUT), evaluating the outcomes for both the mother and the fetus with a minimum follow-up of 12 months after child birth. RESULTS: The mean maternal age at SLE diagnosis was 21.45 ± 6 years and at first pregnancy was 26.49 ± 5.63 years. The mean disease duration was 14.02 ± 19.8 months. At conception, 47 (52.8%) women were hypertensive, 9 (10%) had active disease while 38 (42.7%) and 42 (47.2%) were in complete and partial remission, respectively. A total of 17 (19.1%) were on mycophenolate mofetil (MMF), which was switched to azathioprine (AZA). Out of 89 pregnancies, 56 (62.9%) were successful, while 33 (37.07%) had fetal complications like spontaneous abortion, stillbirth, perinatal death, and intrauterine growth retardation (IUGR). There were more vaginal deliveries (33 [58.92%]) than caesarean sections (23 [41.07%]). Renal flare was observed in 33 (37.1%) women while 15 (16.9%) developed pre-eclampsia. Proliferative LN was found in 56 (62.9%) cases, but no significant differences were found in maternal and fetal outcomes in relation to LN classes (p = .58). However, disease outcomes at 12 months were significantly poor in those with active disease at the time of conception (p < .05). There was only one maternal death. A total of 10 (11.2%) women showed deterioration in renal function and 5 (5.6%) were dialysis-dependent at 12 months. CONCLUSION: The maternal and fetal outcomes in pre-existing LN depend on the disease activity at the time of conception. No correlation was found between International Society of Nephrology/Renal Pathology Society (ISN/RPS) classes of LN and adverse disease and pregnancy outcomes.

The aetiology, risk factors and outcome of Acute Kidney Injury in patients requiring Kidney Replacement Therapy: A prospective single center experience.

Objective: To find out the causes, associated leading factors and impact of kidney replacement therapy on patients hospitalised for acute kidney injury in a tertiary care setting. METHODS: The prospective, cross-sectional study was conducted at the Sindh Institute of Urology and Transplantation, Karachi, from January 1 to March 31, 2022, and comprised patients of either gender aged 18 or more years presenting with acute kidney injury as per the Kidney Disease Improving Global Outcomes criteria and required kidney replacement therapy. Complete, partial or no recovery was the main outcome parameter noted at the end of 90 days. Possible aetiologies were identified and categorised as pre-renal, renal and post-renal aetiologies. Risk factors for acute kidney injury were recorded, including age, gender and co-morbidities. Data was analysed using SPSS 22. RESULTS: Of the 210 patients with mean age 46.1±14.24 years, 109(52%) were males and 101(48%) were females (p>0.05). Hypertension was the most common comorbidity 98(46.8%), followed by diabetes mellitus 75(35.7%) and underlying chronic kidney disease 55(26.2%). Multiple therapeutic interventions were required, including vasopressors in 101(48.1%) patients and mechanical ventilation in 31(14.8%). Renal failure due to intrinsic renal aetiology was the most common 98(46.7%), followed by post-renal aetiology 61(29%). There was no significant association between outcomes and aetiologies (p>0.05). There was increase in chronic kidney disease cases from 55(26.2%) patients at baseline to 107(50.9%) at the end of 90 days. Complete recovery was noted in 71(33.8%) patients, partial in 73(34.76%), no recovery in 34(16.1%) patients who required maintenance kidney replacement therapy, and 32(15.2%) patients died. Conclusion: Complete recovery after 90 days of kidney replacement therapy was observed in one-third of the patients. Intrinsic renal aetiology was the most prevalent, and hypertension was the most common comorbidity.

The prevalence and risk factors of kidney disease in first degree relatives of patients with ESRD treatment - single center study.

OBJECTIVE: To determine the prevalence and risk factors of kidney disease in first degree relatives of patients undergoing treatment for end-stage renal disease. METHODS: The prospective, cross-sectional study was conducted at the Sindh Institute of Urology and Transplantation, Karachi, from May 1 to July 31, 2021, and comprised patients undergoing treatment for end-stage renal disease at the pre-transplant out-patients clinic, and their first degree relatives. Risk factors of chronic kidney disease, including age, gender, body mass index, hypertension, diabetes mellitus, and the causes of index cases were investigated alongside proteinuria, haematuria and estimated glomerular filtration rate. Diagnosis was made according to the criteria of the National Kidney Foundation-Kidney Disease Outcomes Quality Initiative. The participants were divided chronic kidney disease group 1, and healthy group 2. The risk factors were compared between the groups. Data was analysed using SPSS 22. RESULTS: Of the 1,406 subjects assessed, 266(19%) were ESRD patients; 175(65.8%) males and 91(34.2%) females with mean age 34.04±11.19 years. 1,140(81%) first degree relatives of these 266 ESRD patient were assessed; 595(52.2%) males and 545(47.8%) females with mean age 36.78±13.76 years. Among the relatives, 146 (12.8%) had chronic kidney disease out of which 54 (4.7%) were already aware of their underlying disease. Older age, hypertension and diabetes mellitus were among the risk factors for chronic kidney disease (p<0.05), while gender was not significantly different between groups 1 and 2 (p>0.05). The relatives of index cases with underlying stone disease were at higher risk of haematuria 39(22.4%), whereas the relatives of index cases with chronic glomerulonephritis were at higher risk of proteinuria 67(28.03%) compared to index cases of other kinds (p<0.05). CONCLUSIONS: Screening in the high-risk population might help to identify early chronic kidney disease patients for making suitable interventions to prevent disease progression.

Clinicopathological characteristics and renal outcomes of adult patients with pauci-immune necrotizing glomerulonephritis according to ANCA status.

BACKGROUND: Pauci-immune necrotizing glomerulonephritis (PING) is commonly associated with the presence of antineutrophilic cytoplasmic antibodies (ANCAs) but a significant number of patients do not have these antibodies. The significance of ANCA-negativity in the context of Berden's classification of PING is not known. METHODS: A retrospective analysis was conducted on all patients with histopathological diagnosis of idiopathic PING irrespective of ANCA status diagnosed between January 1998 to December 2018 and followed up at renal clinic for > 12 months. All biopsies were reclassified by Berden's classification. Clinicopathological characteristics and renal outcomes of ANCA-positive and ANCA-negative patients were compared. RESULTS: Out of 134 patients, 66 (49.5%) were ANCA-negative. The mean age was 34.76 ± 13.3 years. Compared with the ANCA-positive patients, ANCA-negative patients had significantly greater prevalence of nephrotic-range proteinuria (74.23% Vs 57.9%, P = 0.036) with less extra-renal manifestations (P < 0.05)). On histology, focal and crescentic classes dominated with less number of globally sclerosed glomeruli (2.7% Vs 5.07%, P = 0.02) and more mesangial proliferation (22.7% Vs 4.41%, P = 0.002) in the ANCA-negative group, whereas sclerotic was predominant in the ANCA-positive group (P = 0.05). More patients achieved complete and partial recovery in ANCA-negative patients (42.4% Vs 20.5%, P < 0.05) with better renal survival (27.27% Vs 16.17%, log-rank test: P = 0.03) and less patient mortality (13.63% vs 30.8%, log-rank test: P = 0.04) at 2 years. CONCLUSION: Our study confirms high prevalence of ANCA negativity among our cohort and this group presents with isolated renal involvement with better renal and patient survival. The ANCA-positive group showed significantly more patients in the sclerotic class, lower 2-year renal survival, and higher 2-year mortality as compared to the ANCA-negative group. However, the complete and partial responses to treatment were significantly better in the ANCA-negative group. Key Points • This study shows a high prevalence of ANCA negativity in cases of PING in Pakistani population, as almost half of patients in this study did not have these antibodies. • This negativity is more prevalent in the Asian populations but its significance in the context of Berden's classification of PING is unknown. • ANCA-negative group exhibited less severe phenotype and better outcomes compared with ANCA-positive group.

Clinicopathological characteristics and outcomes of adult patients with idiopathic membranoproliferative glomerulonephritis according to an immunofluorescence-based classification.

BACKGROUND: The classification of membranoproliferative glomerulonephritis (MPGN) into immune complex-mediated MPGN and complement-mediated MPGN on immunofluorescence has provided insights into two distinct disease processes. There are limited data available on renal outcomes of MPGN from developing countries. METHODS: A retrospective analysis was conducted on biopsy-proven MPGN cases diagnosed between 1998 and 2018 at the Sindh Institute of Urology and Transplantation (SIUT). Secondary causes were excluded. Patients were reclassified as immune complex-mediated-MPGN and complement-mediated-MPGN based on immunofluorescence results. The clinicopathological findings and outcomes of the two groups were compared. RESULTS: In total, 213 patients with idiopathic MPGN were identified. Among these, 163 (76.5%) were reclassified as immune complex-mediated-MPGN and 50 (23.4%) as complement-mediated-MPGN. No significant differences were found between the two groups regarding age, gender, clinical characteristics, biopsy indications, biopsy findings, and renal function at presentation. Overall, 63 subjects (38.7%) with immune complex-mediated-MPGN and 27 (54%) with complement-mediated-MPGN received immunosuppressive agents (p = 0.08). Complete and partial remission rates were higher in immune complex-mediated-MPGN than in complement-mediated-MPGN (76% vs 58%, p < 0.05). At two years, median estimated glomerualr filtration rate (eGFR) tended to be higher in patients with immune complex-mediated-MPGN 91.2 (45.4-113.7) vs 83.45(34.6-102.50) ml/min/1.73 m2, p = 0.22) with significantly better renal survival (76% vs 58%, p = 0.03). Comparatively, more patients progressed to end-stage kidney disease (ESKD) in the complement-mediated-MPGN group (32% vs 19.6%, p = 0.06), with increased overall mortality (5 (10%) vs 7 (4.3%), p = 0.12). CONCLUSION: The clinicopathological features at presentation of complement-mediated-MPGN are similar to those of immune complex-mediated-MPGN. However, it is less frequent and overall prognosis is less favorable.

Prevalence of metabolic syndrome in renal transplant recipients--a single centre experience.

OBJECTIVE: To determine the prevalence of metabolic syndrome (MS) in renal transplant recipients (RTR) using the modified Asian National Cholesterol Education Programme-Adult Treatment Panel III (NCEP-ATP III) criteria. METHODS: A cross-sectional study was conducted on 200 RTRs between January 2008-August 2008. All were more than six months post transplant and above 18 years of age. Subjects with pre-transplant diabetes or New Onset Diabetes Mellitus after renal transplantation, with overt infections, dyslipidaemia or on lipid lowering medication and taking immunosuppressive drugs of the target organ inhibitor group as rapamycin, were excluded. The prevalence of MS was determined using the (NCEP-ATP III) criteria modified for Asians which includes waist circumference, triglycerides, HDL cholesterol, blood pressure and fasting blood glucose. RESULTS: Of the 200 recipients studied, 87 (43.5%) had MS. There were 58 (39.4%) males and 29 (54.7%) females which shows female predominance. The mean age of the MS group was more then that of the non MS group (p < 0.0001). Hypertension and New Onset Diabetes Mellitus were prevalent more in MS group (p < 0.001 and p < 0.0001 respectively). Mean serum creatinine was higher in MS group but there was no significant difference. The prevalence of MS was 4.5% in the first twelve months, with a rise in this figure to 41.3% between one to five years after transplantation. CONCLUSION: There is a high prevalence of MS in Renal Transplant Recipients specifically after one year of transplantation.