Aggressive treatment may be needed for idiopathic membranous nephropathy with focal segmental glomerulosclerosis lesions

SUMMARY OBJECTIVE: The purpose of this study was to analyze the clinical, pathological, prognostic features and treatment response of the coexistence of focal segmental glomerulosclerosis lesions with idiopathic membranous nephropathy. METHODS: This is a two-center retrospective cohort study. Patients of idiopathic membranous nephropathy were enrolled and divided into two groups with or without focal segmental glomerulosclerosis lesions according to the renal biopsy. Laboratory data and pathological manifestation were compared. Renal phospholipase A2 receptor was detected by immunofluorescence. During the follow-up, the effects of different therapies and renal function were estimated. RESULTS: A total of 236 patients were finally enrolled in this study, of which 60 and 176 idiopathic membranous nephropathy patients were enrolled in the FSGS+ and FSGS- groups, respectively. The FSGS+ group showed a higher percentage of hypertension history (38.3 vs. 20.0%, p=0.004), with a significantly higher level of systolic pressure [137 (120, 160) mmHg vs. 130 (120, 140) mmHg, p=0.009]. Main laboratory findings, including serial albumin (20.4±7.8 g/L vs. 24.5±6.7 g/L, p<0.001), 24-h proteinuria [5.61 (3.10, 7.87) g/day vs. 3.82 (2.31, 5.79) g/day, p=0.002], serial creatinine [80.8 (65.8, 97.9) μmol/L vs. 72.0 (58.7, 84.9) μmol/L, p=0.003], and estimated glomerular filtration rate [86 (66, 101) mL/min/1.73 m2 vs. 95 (81, 108) mL/min/1.73 m2, p=0.007] showed significant differences between the two groups. Pathologically, patients with focal segmental glomerulosclerosis lesions appeared with a higher percentage of crescents, a more severe degree of interstitial fibrosis, and a higher level of membranous nephropathy stage. Renal phospholipase A2 receptor showed a relatively lower positive rate of only 75.0% in the FSGS+ group in comparison with the positive rate of 90.3% in the FSGS- group (p=0.031). The prognosis was generally similar between the two groups. Among patients who were given non-immunosuppression treatment, those with focal segmental glomerulosclerosis lesions took a relatively longer period of time to achieve complete remission (29.3±7.0 m vs. 15.4±8.9 m, p=0.025) and experienced a higher rate of renal function deterioration (37.5 vs. 5.4%, p=0.033) compared with the other ones. While among those receiving immunosuppression treatment, both groups received similar remission rates. CONCLUSION: Compared with FSGS- group, idiopathic membranous nephropathy with focal segmental glomerulosclerosis lesions represented more severe nephrotic syndrome and worse renal function. In view of the renal function decline during the follow-up, more aggressive treatment with the use of immunosuppressants should be considered for idiopathic membranous nephropathy patients with focal segmental glomerulosclerosis lesions.

De Novo Focal Segmental Glomerulosclerosis (FSGS) Post Covid 19 Vaccination: Case Series in A Single Centre in Malaysia

@#Introduction: Mass COVID-19 vaccination has been pivotal in the fight against this pandemic. The occurrence of glomerular disease following COVID-19 vaccinations particularly mRNA vaccine has been reported. The reported cases in the region are limited and number of cases reported are low in contrast to the total number of vaccine doses given worldwide, the healthcare providers should be alerted about such issues to provide swift and proper management. Case Series: Here, we report 3 cases of Focal segmental glomerulosclerosis (FSGS) following COVID-19 vaccination and their outcomes. Two of the patients received BNT162b2 vaccination and one received CoronaVac vaccination. The mean age of the patients was 33+/-7 years old. The mean duration onset of FSGS was 23+/-19 days post vaccinations. Two of the patients (BNT162b2 vaccination and CoronaVac vaccination) achieved complete remission after corticosteroid therapy. This is the first reported case of De Novo FSGS following CoronaVac vaccination in the literature. The third patient, who received BNT162b2 vaccination and presented late (42 days post vaccination) was not in remission despite three months of immunosuppressive treatment. Conclusion: The treating physician needs to be aware of the possibility of the development of FSGS associated with COVID-19 vaccination and how to proceed with vaccination schedule in these populations. Overall, the advantage of COVID-19 vaccination far outweighs the possibility of COVID-19 vaccine-associated glomerular disease.

Research progress of the mechanism of rituximab in the treatment of focal segmental glomerulosclerosis / 上海交通大学学报（医学版）

Rituximab (RTX), as a monoclonal antibody of CD20 acting on B cell epitopes, has been applied to the field of kidney since 2005, and has become a research hotspot in the clinical treatment of glomerulonephritis. At present, in addition to its clinical safety and efficacy, some researchers are still committed to explore the mechanism of RTX in the treatment of renal diseases, trying to find out whether there is a specific target in renal tissue. In this paper, the mechanism of RTX in the treatment of focal segmental glomerulosclerosis is reviewed.

A Successfully Treated Case of Recurrent Focal Segmental Glomerulosclerosis (FSGS) with Plasmapheresis and High dose Methylprednisolone Pulse Therapy

Focal segmental glomerulosclerosis (FSGS) in children, which is a kind of nephrotic syndrome showing steroid resistance, usually progresses to a substantial number of end stage renal disease (ESRD). Although the pathogenesis of primary FSGS is unclear, several recent studies have reported that FSGS is associated with circulating immune factors such as soluble urokinase-type plasminogen activator receptor (suPAR) or anti-CD40 autoantibody. We report a successfully treated case of a 19-year-old female patient who experienced a recurrence of primary FSGS. After the diagnosis of FSGS, the patient progressed to ESRD and received a kidney transplantation (KT). Three days later, recurrence was suspected through proteinuria and hypoalbuminemia. She has been performed plasmapheresis and high dose methylprednisolone pulse therapy and shown remission status without increasing proteinuria for four years after KT. In conclusion, strong immunosuppressive therapy may be helpful for a good prognosis of recurrent FSGS, suppressing several immunologic circulating factors related disease pathogenesis.

A Case of Collapsing Glomerulopathy in Renal Allograft / 대한신장학회지

Collapsing glomeruopathy (CG) is a clinicopathologic variant of focal segmental glomerulosclerosis (FSGS) and is characterized by severe nephrotic syndrome, rapid progression to end stage renal disease, and features of visceral epithelial cell injury and glomerular capillary collapse. Such characteristics closely resemble those of HIV associated nephropathy. The frequency of CG has increased over the last decade. The cause of CG is unknown. The lesion has rarely been described in renal allografts with features similar to CG in native kidney. We recently identified allograft CG in a 44 year-old male patient who underwent biopsy for graft dysfunction after autodermic graft. The biopsy showed typical characteristics of CG. Serologically, the patient had no evidence of HIV infection. The renal function was not restored to normal in spite of methylprednisolone pulsing therapy. Now he is on conservative treatment with a functioning graft.

Kidney Transplantation Due to Familial Focal Segmental Glomerulosclerosis in 4 Children with Identical HLA-A24 from 2 Families / 대한이식학회지

Focal segmental glomerulosclerosis (FSGS) is a relatively common glomerular disease which is known to be the final pathway of glomerular injuries caused by variable etiologies. There are some renal diseases that are known to have a tendency of familial inheritance such as adult polycystic kidney disease, thin glomerular basement membrane disease, and Alport's syndrome, nephrotic syndrome with many other diseases. Fanconi et al. described the familial occurrence of the nephrotic syndrome first. Since then, a number of other reports have described the cases of nephrotic syndrome within families, though only a handful of families were confirmed as FSGS with histologic evidence. Recently, reports of familial occurrence of FSGS are increasing in number. These patients have been found to be steroid-resistant and unresponsive to immunosuppressive drugs, and most of them progressed to the end stage renal disease. The specific factors leading to glomerular change are not clearly known, but a genetic predisposition has been postulated. A number of reports pointed out the importance of HLA type as a genetic factor related to the pathogenesis of FSGS but the genetic and immunological linkages in FSGS have not been clearly defined yet. We report cases with 4 patients in two unrelated families with HLA-A24 recovered from FSGS after kidney transplantation.