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.

Characterization of a Trpc6 Transgenic Mouse Associated with Early Onset FSGS.

Rationale: Mutations in Transient Receptor Potential Channel 6 (TRPC6) gene are associated with autosomal dominant focal and segmental glomerulosclerosis (FSGS). The majority of the identified mutations affect the ion channel function. Since calcium channels are promising candidate drug targets, there is an an urgent need for a mouse model to assess new therapeutic drugs and to help delineate the pathogenic process leading to FSGS. We have previously reported the generation of three independent transgenic mouse lines carrying different Trpc6 mutations that display a glomerular disease comparable to the phenotype presented by individuals with FSGS. However, the utility of these models for drug testing is dampened by the late-onset of the presentation and the mild phenotypic manifestations. Methodology: In order to obtain a time-effective mouse model for Trpc6-associated FSGS we generated a new transgenic mutant Trpc6 mouse model emulating the amino acid change carried by the first pediatric patient of FSGS associated with a TRPC6 mutation: M132T. Results: Mice carrying the orthologous Trpc6 M131T transgene showed early onset proteinuria and early signs of FSGS. When exploring molecular consequences of the overexpression of this mutated form of Trpc6 in podocytes, differences in expression levels of Axin2 and β-catenin were found in glomeruli from transgenic Trpc6 M131T mice. These data supports the proposed molecular mechanisms related to the activation of calcineurin-NFAT/Wnt signaling, as outcome of the increased calcium influx caused by the mutated form of Trpc6. Conclusion: Given that the Trpc6 M131T mouse develops an early onset of FSGS-like phenotypes it represents a promising model for studying the pathogenesis of FSGS caused by TRPC6, facilitating the assessment of new drugs as treatments and allowing further studies to understand underlying molecular pathways involved in the development of the TRPC6 mediated disease.

Study of the morphologic variants of focal segmental glomerulosclerosis: a Brazilian report / Estudo brasileiro das variantes morfológicas da glomerulosclerose segmentar e focal

INTRODUCTION: Focal segmental glomerulosclerosis (FSGS) is the most frequent primary glomerulopathy in Brazil and its incidence is increasing worldwide. Pathogenesis is related to podocyte injury, which may be due to several factors including viruses, drugs, genetics and immunological factors. In 2004, the Columbia classification of FSGS identified five histological variants of the disease: collapsing (COL), usual (NOS), tip lesion (TIP), perihilar (PHI) and cellular variant (CEL). The objective of this study was to classify the FSGS biopsies in these morphological variants. METHODS: One hundred thirty-one cases of renal biopsies with primary FSGS diagnosis, which had been performed at a Brazilian reference center from 1996 to 2006, were classified according to the Columbia criteria. RESULTS: FSGS cases were distributed as follows: 38.2% NOS variant, 36.6% COL, 14.5% TIP, 6.9% PHI and 3.8% CEL. CONCLUSION: COL variant of FSGS seems to be more prevalent in Brazil in comparison with other centers worldwide, which may be related to environmental and socioeconomic factors.

INTRODUÇÃO: A glomerulosclerose segmentar e focal (GESF) é a glomerulopatia primária mais frequente no Brasil e sua incidência está aumentando em todo o mundo. Sua patogênese está relacionada com a lesão de podócitos, que pode ser devida a vários fatores, incluindo vírus, drogas, fatores genéticos e imunológicos. Em 2004, a classificação de Columbia GESF definiu cinco variantes histológicas da doença: colapsante (COL), usual (NOS), lesão apical (TIP), Peri-hilar (PHI) e variante celular (CEL). O objetivo deste estudo foi classificar as biópsias com diagnóstico de GESF nessas variantes morfológicas. MÉTODOS: Cento e trinta e um casos de biópsias renais com diagnóstico de GESF primária em um centro brasileiro de referência em nefrologia, no período de 1996 a 2006, foram classificados de acordo com os critérios de Columbia. RESULTADOS: Os casos se distribuíram da seguinte forma: 38,2% da variante de NOS; 36,6% de COL; 14,5% de TIP; 6,9% de PHI; 3,8% de CEL. CONCLUSÃO: A variante COL de GESF parece ser mais prevalente no Brasil do que em outros centros internacionais e isso pode ser reflexo de fatores socioeconômicos e ambientais.

Combination Treatment of Rituximab and Plasmapheresis in Acute Cellular Rejection with Focal Segmental Glomerular Sclerosis after Renal Transplantation / 대한이식학회지

Focal segmental glomerular sclerosis (FSGS) accounts for recurrence in 20% to 40% of the renal allografts after transplantation, and it causes graft loss in 13% to 20% of the cases. We report here on successfully treating acute cellular rejection (ACR) combined with FSGS after a kidney transplantation with a combination treatment of plasmapheresis, rituximab and steroid pulse therapy. A 53-year-old female patient whose primary kidney disease was unknown developed massive proteinuria after living donor kidney transplantation. A urine protein/creatinine ratio of 13.42 and an elevated serum creatinine level was detected on postoperative days (POD) 10 and a renal biopsy showed acute cellular rejection (Banff IIb) combined with FSGS. We started steroid pulse therapy on POD 11. She underwent 5 plasmapheresis sessions in the first 3 week after transplantation and she received one dose of rituximab (375 mg/m2) on POD 12. The proteinuria decreased below the nephrotic range at POD 20 and the serum creatinine level was normalized. Three months later, the proteinuria was at 35 mg/day with stable graft function. Rituximab and plasmapheresis is a possible option to treat FSGS combined with a relapse of proteinuria after renal transplantation.

Maintenance of Recurred Focal Segmental Glomerulosclerosis of Transplanted Kidney for More Than 9 Months by Performing Plasmapheresis / 대한수혈학회지

Focal segmental glomerulosclerosis (FSGS) is thought to be caused by FSGS permeability factor (FSGF) in the plasma, and this plasma FSGF might be responsible for FSGS recurrence after transplantation. Therefore, to reduce the FSGF in plasma, plasmapheresis (PE) is usually used to treat recurred FSGS. We report here on the PE treatment of 33-year-old women who had recurrence of FSGS after transplantation. After recurrence, she was treated by intensive PE for 1 month and she achieved complete remission. But because the proteinuria was increased when we stopped PE, we regularly continued PE for 28 month with the patient in a state of partial remission. To the best of our knowledge, this is the second report on treating recurred FSGS with intensive PE and this is the first report for long term maintenance with performing regular PE.

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.

Second Trial of Cyclosporin A-Induced Remission in Other Immunosuppressant Therapy-Resistant FSGS Patient

Focal segmental glomerulosclerosis(FSGS) has been detected in approximately 10% of cases of idiopathic nephrotic syndrome in children, and exhibits a poor response to initial steroid therapy, as well as a higher rate of progression to chronic renal failure and relapse after kidney transplantation. We describe a case of an eleven year-old boy with steroid-resistant FSGS who exhibited a response to a second trial of cyclosporin A(CsA) therapy. At the age of 26 months, this patient was diagnosed with steroid-resistant FSGS. For 9 years, he had undergone a gauntlet of therapies to induce remission; oral steroids, cyclophosphamide, methylprednisolone(mehylPd) pulse therapy, CsA, and ibuprofen therapy. Although these therapies failed to induce remission, the patient's renal function remained in the normal range during the nine years of treatment. At the age of ten years, the patient's proteinuria decreased, and complete remission was attained with a second administration of CsA, coupled with a low dose of oral steroids. This patient continues to receive CsA without relapse. Therefore, our major concern involves the possibility of relapse after the discontinuation of CsA therapy. Our findings in this case suggest that, in cases of refractory FSGS, if renal insufficiency does not emerge, aggressive therapy for the amelioration of proteinuria should be continuously pursued.

Two Cases of FSGS Maintaining Renal Function by Long Term Cyclosporine Treatment / 대한신장학회잡지

Cyclosporine can cause remission of 60% in steroid resistant FSGS, but its responses are variable. Now we report two cases of steroid resistant FSGS who are maintaining remission using cyclosporine continuous therapy. The first patient had been failed several times of steroid therapy, had edema, azotemia and severe proteinuria. We used steroid pulse therapy then maintenance dose of oral cyclosporine to reduce proteinuria for more than 6 years. He has been received cyclosporine therapy up to now and maintaining normal renal function. The second patient had severe azotemia who needed hemodialysis but after cyclosporine therapy, he recovered his renal funciton. The findings of renal biopsies in one patient after 6 years of cyclosporine therapy revealed that there was no improvement of sclerosing glomeruli, then we guess that maintenance therapy of cyclosporine might need for lifelong period.

Long-term Prognostic Factors in Pediatric Focal Segmental Glomerulosclerosis

PURPOSE: Efforts to predict long-term outcome of focal segmental glomerulosclerosis(FSGS) have been made but have yielded conflicting results. Reports are rare especially in pediatric patients. In this study, we reviewed the predictable prognostic factors in patients of FSGS. METHOD: Fifty children who diagnosed as biopsy-proven FSGS at depart- ment of pediatrics at Yonsei university were studied retrospectively. Based on medical records, response to treatment and pathologic slides, we compared normal renal function group and decreased renal function group, assessed the factors affecting renal survival and progression to renal failure. RESULTS: The mean age at onset was 8 1/12 years, sex ratio was 2.3 : 1, and the mean duration of follow-up was 7 1/12 years. The overall renal survival rate was 34% at 5 years, 8% at 10 years. Five-year survival rate was 74% in normal renal function group and 27% in decreased renal function group. Between the two groups, there were no significant differences in age at onset, sex ratio, amount of proteinuria, incidence of hematuria and hypertension, mesangial hypercellularity. Decreased renal function group showed higher serum creatinine level, poor response to treatment, higher percent of glomeruli with sclerosis, moderate to severe tubulointerstitial change and vascular change(p<0.05). The prognostic factors of renal survival rate were same as above and incidence of hypertension also affected renal survival(p<0.05). The progression rate to renal failure did not show statistically significant factor. CONCLUSION: We reviewed the factors affecting long-term outcome of FSGS. Serum creatinine level, steroid responsiveness, and the degree of glomerulosclerosis were significant prognostic factors.

Study on Circulating Factor(s) in Patient with Recurrent Focal Segmental Glomerulosclerosis / 대한신장학회잡지

BACKGROUND: Although a significant number of studies were done on focal segmental glomerulosclerosis(FSGS), its pathogenesis has not been sufficiently established yet. Recent studies suggested certain types of circulating factor(s) played an important role in development and recurrence after renal transplantation of FSGS by modifying the glomerular permeability of albumin. The purpose of this study performed on animals and through molecular-biological experiments is to certify the role of circulating factor (s), which cause proteinuria, by manipulating plasma of a FSGS patient who showed massive of proteinuria and wide effacement of glomerular epithelial foot processes in histologic examination after renal transplantation. also, whose massive proteinuria decreased significantly after plasma exchange. METHODS: The patient's plasma prior to(plasma A) or post to(plasma B) plasma exchange were injected into tail veins of two groups of male Sprague-Dawley rats, six in each. The ratio of 24 hour urine protein and urine creatinine(Uprt/Ucr) was calculated for each case. The 2D gel electrophoresis was performed in plasma A and plasma B. The pattern of 2D gel electrophoresis of plasma A was compared to those of plasma B and healthy human serum. RESULTS: Compared to control group, there was no significant differences in 24-hour Uprt/Ucr afer injecting 1, 2, 3, 5 mL of plasma A(p>0.05). There was no significant difference in 24-hour Uprt/Ucr between the injecting groups of plasma A and plasma B(p>0.05). We were not able to observe any new protein which did not appear in plasma B or healthy human serum in 2D gel electrophoresis. CONCLUSION: These results suggest that the proteinuria developed in a few hours after renal transplantation and is related to wide effacement of glomerular epithelial foot processes, and that it may be induced by a certain factor which is eliminated by the plasma exchange or restrained by the immunosuppressive agents. However, we were not able to find certain circulating factor(s) which rapidly changes albumin permeability in the patient's plasma with FSGS.

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.

Glomerular Hypertrophy and Sclerosis in Rats with Chronic Puromycin Aminonucleoside Nephropathy / 대한신장학회잡지

PURPOSE: This experimental study was conducted to determine serial morphological changes of rat's kidney with chronic puromycin aminonucleoside (PAN) nephropathy. Special emphasis was given to the occurrence of glomerular hypertrophy and its relationship to the subsequent development of focal segmental glomerulosclerosis(FSGS). METHODS: Sprague-Dawley rats weighing 200-230g were used and divided into control(n=9) and experimental group(n=15). Rats were given subcutaneous injections of PAN at a dose of of 2mg/100g body weight, or an equivalent volume of normal saline and six injection were given over a period of 9 weeks, at weeks 0, 1, 3, 5, 7 and 9. At weeks 4, 8 and 11, rats were sacrificed and kidney weight, kidney weight/body weight(%) and various laboratory tests including serum protein and albumin were determined. Renal tissues were prepared with Histochoice(R) fixative and paraffin embedding for morphologic study. RESULTS: Kidney weight and kidney weight/body weight(%) were increased significantly in experimental group compared to controls at 4, 8 and 11 weeks. Heavy proteinuria along with lowering of serum protein and albumin and elevation of serum cholesterol was seen in experimental group at week 4 and this change became more marked on weeks 8 and 11. The frequency of FSGS in experimental animal, at week 4, 8 and 11 were 0.6%, 10.6% and 26.2% respectively(p<0.05) and the development of FSGS was more marked in juxtamedullary glomeruli compared to cortical glomeruli. Glomerular surface area showed significant increase in experimental animals compared to controls(p<0.01), the percentage of increase being 12.0, 14.7 and 12.3% at week 4, 8 and 11. And the surface areas of juxtamedullary glomeruli were larger than those of cortical glomeruli throughout the study period. CONCLUSION: In summary, present study indicates that glomerular hypertrophy occurs and precedes the development of FSGS in rats with chronic PAN nephropathy and juxtamedullary glomeruli are more susceptible to developing FSGS compared to cortical glomeuli.

A Case of Nephrotic Syndrome Associated with Total Hydatidiform Mole / 대한신장학회잡지

A case study and review of nephrotic syndrome associated with a total Hydatidiform mole in 54- year-old female is presented. She has generalized edema, nephrotic range proteinuria(8.05gm/day), hypoalbuminemia(2.5g/dl) and high serum level of beta- hCG(200,000IU/L). Radiological investigations showed a 16-cm sized heterogenous enhanced mass in the uterus. A renal biopsy performed before evacuation of H-mole showed a focal segmental glomerulosclerosis. The complete remission of symptoms and signs of the nephrotic syndrom after evacuation of a molar tissue was achieved. A review of the literature revealed that this patient appears to be the first case of the FSGS with nephrotic syndrome associated with a total mole that remitted completely after the mole evacuation.

Three-Dimensional Morphometric Analysis of Segmental Glomerulosclerosis in the Rat Remnant Kidney Model / 대한신장학회잡지

In the previous study, serial section analysis in the Adriamycin animal model of progressive glomerulosclerosis, diffuse, rather than focal glomerulosclerosis were reported. However, the study in patients with nephrotic syndrome and idiopathic focal segmental glomerulosclerosis by three-dimensional analysis demonstrated a focal pattern of sclerosis. The distribution of lesions, whether focal or diffuse, has important implications, not only for pathogenesis and potential therapeutic response, but also for relevance of animal studies to human disease. We therefore evaluated the distribution of segmental glomerulosclerosis in the rat remnant kidney model. We used male M nich-Wistar rats weighing 250 grams at the start of the experiments. Ten rats had 1-1/2 nephrectomy. At the 24th week after the surgical procedure, GFR and arterial BP were measured. Renal tissues were then perfused with 1% glutaraldehyde at physiologic pressures. Sixty serial sections(4 micrometer thick) per a rat were stained with PAS. An average of 19.2+2.4 glomeruli was examined. Sclerosis assessed on a single section involved 32.6+11.4%. After the serial section analysis, the percent of glomeruli involved by sclerosis increased to 57.6+15.0%. Our results show that single section examination of kidney tissue underestimates glomerulosclerosis in the renal ablation model. However, much more intact glomeruli were present in the renal ablation model compared to Adriamycin rat model after serial section analysis. This finding may reflect the different pathogenic mechanism between the two animal models.

Expression of Transforming Growth Factor-beta and Morphologic Changes of Glomerulosclerosis in FGS/NgaKist Mouse

Focal segmental glomerulosclerosis (FSGS) is presented as not only one of the primary glomerular diseases but also as a secondary phenomenon for chronic irreversible renal diseases. The main pathological feature of FSGS is the accumulation of extracellular matrix in the glomeruli, for which overexpression of transforming growth factor-beta (TGF-beta) may be responsible for the accumulation of pathological matrix. A new animal model (FGS/NgaKist mouse) of renal failure by spontaneously generating glomerulosclerosis was developed. To elucidate the role of TGF-beta for FSGS, authors observed glomeruli of FGS/NgaKist mouse periodically. FGS/NgaKist mouse strain showed progression of proteinuria and focal glomerular sclerosis with the aging. The glomeruli showed anti IgG, IgA, IgM and complement complex deposits and extracellular matrix accumulation in the mesangium. TGF-beta mRNA and beta2antibody expressions were increased with the advance of glomerular sclerosis. The results suggest the following; FSGS of FGS/NgaKist strain is immune mediated disease and this stimuli on mesangial or endothelial cells may activate TGF-beta gene in their nuclei. This activation, in turn, can cause sclerosis by increasing TGF-beta mRNA transcription followed by secretion of TGF-beta and its action as cytokine for making collagen fibrils.

Morphologic Comparisons of Focal Segmental Glomerulo-sclerosis between Human and 5/6 Nephrectomy Rat Model

This study was conducted to set up a common mechanism for varying phases of focal segmental glomerulosclerosis(FSGS) by comparing the morphological differences between human FSGS and changes in 5/6 renal ablation animal model, which has been accepted as experimental prototype for hyperfiltration theory as pathogenesis of FSGS. Both the human and the experimental rats showed very similar changes such as segmental glomerulosclerosis, vacuole formations or inclusion of small granules of podocytes, appearance of foamy cells in the capillary lumina, eosinophilic deposits along the mesangial area, and focal atrophy of tubules with associated interstitial fibrosis. The halo, frequently seen in human FSGS, is due to detachment of visceral epithelium from basement membrane, however, did not appear in the experimental rat specimen. On the other hand, the foamy cells and hyalinization were more frequently noted in the rat series and even involved the arterioles. The mesangial proliferation never appeared in the rat series occasionally found in human FSGS. In conclusion, the pathogenesis of FSGS cannot depend solely on the hyperfiltration theory of hemodynamic derangement, but has complex impairment of visceral epithelium and cells forming the constituents of basement membrane.

Morphologic Comparisons of Focal Segmental Glomerulo-sclerosis between Human and 5/6 Nephrectomy Rat Model

This study was conducted to set up a common mechanism for varying phases of focal segmental glomerulosclerosis(FSGS) by comparing the morphological differences between human FSGS and changes in 5/6 renal ablation animal model, which has been accepted as experimental prototype for hyperfiltration theory as pathogenesis of FSGS. Both the human and the experimental rats showed very similar changes such as segmental glomerulosclerosis, vacuole formations or inclusion of small granules of podocytes, appearance of foamy cells in the capillary lumina, eosinophilic deposits along the mesangial area, and focal atrophy of tubules with associated interstitial fibrosis. The halo, frequently seen in human FSGS, is due to detachment of visceral epithelium from basement membrane, however, did not appear in the experimental rat specimen. On the other hand, the foamy cells and hyalinization were more frequently noted in the rat series and even involved the arterioles. The mesangial proliferation never appeared in the rat series occasionally found in human FSGS. In conclusion, the pathogenesis of FSGS cannot depend solely on the hyperfiltration theory of hemodynamic derangement, but has complex impairment of visceral epithelium and cells forming the constituents of basement membrane.

Plasmapheresis in Recurrent Focal Segmental Glomerulosclerosis after Renal Transplantation / 대한신장학회잡지

Focal segmental glomerulosclerosis (FSGS) in pediatric age tends to progress to end stage renal disease and to recur after renal transplantation. And recurrence of FSGS after kidney transplantation results in the graft loss in above half of cases. An unknown circulating factor in serum and immunologic dysfunction may be responsible for the recurrence. So, plasmapheresis to remove the uncertain serum factor and high dose cyclosporin A to control the immunologic system have been tried as the therapeutic regimen. We experienced 5 patients with recurrent FSGS after transplantation and tried plasmapheresis and methyl prednisolone pulse therapy with high dose cyclosporin A for them. The patients were 2 girls and 3 boys, aged between 8 and 14 years. In all cases, the kidney was donated by living related donors. Recurrence of FSGS was detected by postop. 3 days. Plasmapheresis started within 1 week after recurrence in 4 cases, and 2 months in 1 case. Early plasmapheresis brought rapid and sustained remission in 2 cases without evidence of acute rejection and short-term partial response in remaining 3 cases. In conclusion, plasmapheresis with high dose cyclosporin A resulted in a good outcome in recurrent FSGS. And starting plasmapheresis as early, prior to irreversible glomerular scarring, as possible is important for immediate and long-term prognosis in recurrent FSGS after renal transplantation.