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Background: Collapsing focal segmental glomerulosclerosis (cFSGS) is an aggressive glomerular disease presenting as a nephrotic syndrome that has lower rates of remission with conventional immunosuppressive therapy and rapidly progresses to end-stage-renal-disease (ESRD). We report eight cases of HIV-negative cFSGS treated with rituximab., Methods: The current report is a retrospective case series of cFSGS treated with rituximab from January 2011 to March 2020, at varying phases of the disease., Results: Eight out of the 70 cFSGS patients received rituximab. The median age of patients was 30 years (IQR 24.25-37.5); five patients were males. The median serum creatinine, mean serum albumin and median 24 hours urinary protein at presentation was 0.9 (IQR 0.66-1.27) mg/dL, 2.95 ± 1.15 g/dL, 4.87 (IQR 1.64-5.75) g/day, respectively. Two patients were steroid-resistant, one steroid and tacrolimus dependent, one steroid and cyclosporine dependent, two steroids and tacrolimus resistant, one steroid, tacrolimus, cyclophosphamide, mycophenolate mofetil resistant and one steroid-resistant and tacrolimus dependent before rituximab therapy. Rituximab was given either as targeted therapy (after an initial dose of 375 mg/m 2 ; patients having CD-19 levels >5/μL or >1% at 1 month received additional low-dose [100 mg] of rituximab), or weekly regimen. Five patients received CD-19 targeted rituximab; three received weekly doses of 375 mg/m 2 , cumulative doses being 820 ± 228.03 mg, and 1800 ± 721.11 mg, respectively. At the end of median follow-up of 15 months, five (62.5%) patients were in remission (three partial, two complete remissions), two (25%) were resistant to therapy; one (12.5%) progressed to ESRD., Conclusion: Rituximab is reasonably safe and achieves/maintains remission in 60% of cFSGS cases., (© 2020 Asian Pacific Society of Nephrology.)

Primary forms of minimal change disease and focal segmental glomerulosclerosis are rare podocytopathies and clinically characterized by nephrotic syndrome. Glucocorticoids are the cornerstone of the initial immunosuppressive treatment in these two entities. Especially among adults with minimal change disease or focal segmental glomerulosclerosis, relapses, steroid dependence or resistance are common and necessitate re-initiation of steroids and other immunosuppressants. Effective steroid-sparing therapies and introduction of less toxic immunosuppressive agents are urgently needed to reduce undesirable side effects, in particular for patients whose disease course is complex. Rituximab, a B cell depleting monoclonal antibody, is increasingly used off-label in these circumstances, despite a low level of evidence for adult patients. Hence, critical questions concerning drug-safety, long-term efficacy and the optimal regimen for rituximab-treatment remain unanswered. Evidence in the form of large, multicenter studies and randomized controlled trials are urgently needed to overcome these limitations., Competing Interests: Declaration of Competing Interest VA reports grants from ADDMEDICA, outside the submitted work; AB reports consulting fees from Chemocentryx, from Merck/MSD and from Astra Zeneca, outside the submitted work; MB reports lecture fees from Boehringer Ingelheim, from Bristol-Myers Squibb, from Novartis, travel support from Hexal, lecture fees and travel support from Vifor Fresenius and lecture fee from Servier, outside the submitted work; CKC reports grants from GlaxoSmithKline, grants and consultancy fees from Retrophin, outside the submitted work; SF reports lecture fees from VIFOR Pharma, outside the submitted work; CK reports being funded by National Institute for Health Research (NIHR) as academic clinical fellow (ACF), during the conduct of the study. All the other authors declared no competing interests., (Copyright © 2020 The Author(s). Published by Elsevier B.V. All rights reserved.)

Rituximab is currently used after the conventional agents have failed in the management of steroid-dependent (SD)/ steroid-resistant (SR) podocytopathies and have a safer toxicity profile. We report 53 adults with podocytopathies who were managed effectively with CD19-targeted rituximab therapy., Methods: This was a prospective study carried out at a tertiary care centre in India between January 2014 and June 2019. Adults between 16 and 60 years with SD, frequently relapsing (FR), and SR nephrotic syndrome (NS) due to podocytopathy received rituximab in a CD19-targeted approach., Primary Outcome: Percentage of patients who were in remission at 6 and 12 months. Secondary outcome: Percentage of patients in remission at the last follow-up, rituximab dose and adverse events of rituximab therapy., Results: Fifty-three adults with SD/FR/SR NS received CD19-targeted rituximab. The median age at the time of first rituximab injection was 30.09 ± 13.21 (16.53) years. At the time of first rituximab infusion, all patients were in remission with steroids and/or calcineurin inhibitors (CNIs). Fifty (94.33%) patients were in remission at the end of 6 and 12 months and the last follow-up (median: 36 months). The mean total dose of rituximab at 1 year was 788.7 ± 128.1 (6 001 100) mg. At last follow-up (median 36 months), 42 (79%) patients did not require any additional CNI or steroids therapy. No serious adverse events to rituximab were noted., Conclusion: CD19-targeted rituximab therapy is safe and efficacious in the management of SD/SR adult podocytopathy. Also, rituximab is effective in maintaining remission in treatment naïve adult SD or FR podocytopathy., (© 2020 Asian Pacific Society of Nephrology.)

Aim: A significant proportion of patients with minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are either steroid dependent or steroid resistant, requiring long-term calcineurin inhibitors (CNI) use. Rituximab has more favourable safety profile. The present study was undertaken to evaluate the efficacy and safety of rituximab in CNI-dependent patients., Methods: This was a prospective observational study conducted from July 2014 to February 2018. Steroid-dependent nephrotic syndrome or steroid-resistant nephrotic syndrome (biopsy proven MCD/FSGS), who were CNI dependent were enrolled. Mean age at enrolment was 22.77 ± 7.45 years. All patients received rituximab at a dose of 375 mg/m 2 at entry in the study. CD-19 levels were monitored monthly and patients having CD-19 levels >5/μL and/or > 1% received additional low-dose (100 mg) of rituximab., Results: A total of 24 patients were followed up for 12 months. At the end of 6 and 12 months, 87.5% and 79.16% of the patients achieved remission, respectively. Eight (33.33%) patients developed relapse. The mean dose of rituximab in the first year was 791 mg. The average cost of rituximab in the first year was 487.17$. Rituximab was well-tolerated, with mild infusion reactions, respiratory tract infection and oral candidiasis in 5 (20.83%), 5 (20.83%) and 1 (4.17%) patient, respectively., Conclusions: CD-19 targeted rituximab is a safe and cost-effective agent in remission maintenance in adults with CNI dependent. Over three-fourths of the patients with CNI-dependent podocytopathy maintain clinical remission with CD-19 targeted rituximab therapy., (© 2018 Asian Pacific Society of Nephrology.)

Aim: Recurrence of focal segmental glomerulosclerosis (FSGS) in the post-transplant setting is variable with high rates of graft loss. Risk factors of recurrence include young age and Caucasian race. Data on outcome of recurrent FSGS from South Asia is scanty. We describe our experience of managing adults with recurrent FSGS with different therapies. Settings and Design: The study was conducted at the Department of Nephrology and Renal transplant surgery, Post Graduate Institute of Medical education and Research Institute, Chandigarh, India, and this was an observational study. Methods: We analyzed outcomes of patients with biopsy-proven recurrent FSGS over the last 5 years (2012–2017). Recurrence was defined as significant proteinuria (albuminuria ≥ 3+) and a demonstrable FSGS lesion on light microscopy and/or electron microscopy suggestive of diffuse foot process effacement. Results: Thirteen patients with 14 recurrences of FSGS post-renal transplant were identified. Mean age of the patients was 33.15 (±8.32) years. Median time to recurrence of FSGS was 45 days. Plasma exchange (PLEX) alone was used in two patients with 50% in remission. Combined PLEX and rituximab were used in six recurrences with remission in 83.3% of them. Five recurrences were treated with only angiotensin-converting enzyme inhibitor/angiotensin receptor blockade (ACEi/ARB) due to financial constraints. Of them, 4 (80%) achieved remission in proteinuria. One patient did not receive any therapy and expired in the 1st month of follow-up. Conclusion: The present series is one of the largest reports of recurrent FSGS from South Asia. Furthermore, the current report strengthens the use of ACEi/ARB in patients with recurrent FSGS. [ABSTRACT FROM AUTHOR]

The article presents a case study of 19-year-old-male with swelling of the lower limbs and periorbital puffiness of 2 months' duration. The diagnosis of minimal-change disease (MCD) was made and the patient was started on oral prednisolone. The patient treated with rituximab and results show a favourable effect of rituximab in an adult with CG.