Glomerular parietal epithelial expression of CD44 in minimal change nephrotic syndrome and primary focal segmental glomerulosclerosis: A clinico-pathological study

Introduction: Minimal change nephrotic syndrome (MCNS) and focal segmental glomerulosclerosis (FSGS) are the two common causes of nephrotic syndrome (NS) in both children and adults with overlapping clinical features, but with distinct prognostic and therapeutic implications. The distinction between these relies entirely on histopathology, which can sometimes be difficult. CD44 is expressed by activated parietal epithelial cells, plays a role in matrix deposition and thus in the pathogenesis of FSGS. Aims: To assess the expression of CD44 in MCNS and FSGS and to evaluate its association with the known clinical and histopathological prognostic factors. Materials and Methods: Thirty cases each of MCNS and FSGS were studied. The clinical, laboratory, histopathological, and CD 44 immunohistochemical data were recorded. The findings were analyzed and correlated. A P value of < 0.05 was considered statistically significant. Results: Statistical association was noted between CD44 positivity and serum creatinine (p = 0.031), estimated glomerular filtration rate (p = 0.040), segmental sclerosis (p < 0.001), tubular atrophy (p = 0.027), interstitial fibrosis (p = 0.027), and histological diagnosis (p < 0.001). The sensitivity, specificity, positive predictive, and negative predictive values were 90%, 76.67%, 79.41% and 88.46%, respectively. Conclusions: CD44 immunostain can effectively distinguish MCNS from FSGS. The congruent results of CD44 positivity with known prognostic factors support the possibility of using the CD44 marker as a predictive tool in selecting high-risk patients and offering appropriate therapeutic measures.

Glomerulonefritis por cambios mínimos post vacuna COVID-19 AstraZeneca / Glomerulonephritis due to minimal change disease post COVID-19 vaccine AstraZeneca

RESUMEN Reportamos un caso de una mujer de 28 años, con síndrome nefrótico debido a glomerulonefritis por cambios mínimos 48horas después de la administración de la vacuna contra SARS-CoV2 de AstraZeneca. La paciente tuvo síndrome nefrótico idiopático en la infancia tratado empíricamente con corticoides y ciclosporina, en remisión completa desde los 9 años. Algunos reportes sugieren que determinadas enfermedades glomerulares podrían asociarse infrecuentemente a las vacunas.

ABSTRACT We report a case of a 28-year-old woman with minimal change disease secondary nephrotic syndrome 48 hours after the administration of the AstraZeneca SARS-CoV2 vaccine. The patient had suffered idiopathic nephrotic syndrome in childhood treated empirically with corticosteroids and cyclosporine, in complete remission from the age of 9. Some reports suggest that glomerular disease might appear infrequently associated to some vaccines.

Advances in pathogenesis of minimal change nephrotic syndrome / 国际儿科学杂志

Minimal change nephrotic syndrome (MCNS) is the most common nephrotic syndrome among children.Although the details of pathogenesis remain unknown, it is widely considered that upregulated expression of T lymphocyte cell cytokines contributes to the initiation of MCNS.Moreover, studies had revealed that altered number and function disorder of B lymphocyte cells could change the functions of antigen presentation, participating in the onset of MCNS by affecting the function of T lymphocyte cells.Recently, CD80 has emerged as a popular research topic which exerts its effects via the change of podocytes morphology, thereby affecting the glomerular permeability.However, neither immune disorder nor podocyte dysfunction is poorly demonstrated to be associated with the pathogenesis of MCNS, the hypothesis such as "a 'two hit disorder" and "γδT cells exacerbate podocyte injury via the CD28/B7-1-phosphor-SRC kinase pathway" are raised.In the current review, we summarized the related investigations to help us to understand the mechanisms and pathogenesis of MCNS.

Advances in pathogenesis of minimal change nephrotic syndrome / 国际儿科学杂志

Minimal change nephrotic syndrome (MCNS) is the most common nephrotic syndrome among children.Although the details of pathogenesis remain unknown,it is widely considered that upregulated expression of T lymphocyte cell cytokines contributes to the initiation of MCNS.Moreover,studies had revealed that altered number and function disorder of B lymphocyte cells could change the functions of antigen presentation,participating in the onset of MCNS by affecting the function of T lymphocyte cells.Recently,CD80 has emerged as a popular research topic which exerts its effects via the change of podocytes morphology,thereby affecting the glomerular permeability.However,neither immune disorder nor podocyte dysfunction is poorly demonstrated to be associated with the pathogenesis of MCNS,the hypothesis such as "a two hit disorder" and "γδT cells exacerbate podocyte injury via the CD28/B7-1-phosphor-SRC kinase pathway" are raised.In the current review,we summarized the related investigations to help us to understand the mechanisms and pathogenesis of MCNS.

Interleukin-13 Increases Podocyte Apoptosis in Cultured Human Podocytes

PURPOSE: Podocytes are important architectures that maintain the crucial roles of glomerular filtration barrier functions. Despite this structural importance, however, the mechanisms of the changes in podocytes that can be an important pathogenesis of minimal change nephrotic syndrome (MCNS) are not clear yet. The aim of this study was to investigate whether apoptosis is induced by interleukin (IL)-13 in cultured human podocytes. METHODS: Human podocytes were treated with different IL-13 doses and apoptotic cells were analyzed using terminal deoxynucleotidyl transferase dUTP nick-end labeling (TUNEL assay) and fluorescence-activated cell sorting (FACS). RESULTS: The IL-13 increased the number of TUNEL-positive cells in a dose-dependent manner at 6 and 18 hours (P<0.05 and P<0.05, respectively). The apoptosis rate was appeared to be increased slightly in the IL-13-stimulated podocytes (8.63%, 13.02%, and 14.46%; 3, 10 and 30 ng/mL, respectively) than in the control cells (7.66%) at 12 hours by FACS assay. CONCLUSION: Our study revealed that IL-13 expression may increase podocyte apoptosis. Blocking the IL-13 signal pathway can potentially play an important role in regulating the apoptosis of podocytes.

To assess the reduction in bone mineral density among children who completed steroid therapy for nephrotic syndrome

Introduction: Minimal Change Nephrotic Syndrome is the most common type of nephrotic syndrome accounting for 85% of cases. It is the most common primary or idiopathic type of nephrotic syndrome in children. It occurs between 1 to 12 years of age, but most commonly 2 to 6 years. Even though the majority of cases show remission of nephrotic syndrome, the hypocalcemia due to Glucocorticoids are very severe. It reduces the bone mineralization and reducing the bone mineral content and thereby reducing bone density. Aim of the study: To assess the reduction in bone mineral density among children who have completed the first course of steroid therapy for nephrotic syndrome by measuring biochemical markers of bone. Materials and methods: This study was done to find out the reduction in the Bone mineral density among children who completed steroid therapy for nephrotic syndrome, by using bone biochemical markers. This study also helped to assess the side- effect of Glucocorticoids on bone density and to prevent bone demineralization and pathological fractures in children. Results: The results showed there was a reduction in the serum calcium values among children with MCNS. This implied hypocalcemia among children due to GCs and the P value is significant <0.001. This represented the corrected calcium levels among the children after drug effect. It implied the D. Sampath Kumar, S. Prasanna, P. Sakthi Seethalakshmi. To assess the reduction in bone mineral density among children who completed steroid therapy for nephrotic syndrome. IAIM, 2018; 5(2): 94-104. Page 95 overall the corrected calcium levels at low levels with MEAN=8.34 mg%. The P value was <0.024 Significant. The total proteins were normal among children after completing the glucocorticoid therapy. The P value was <0.001 and was significant. Mean = 5.68. Standard Deviation (SD) = 0.28. The serum phosphorus was almost normal among remission MCNS Children and at higher levels among defaulters, SDNS and SRNS. Conclusion: Glucocorticoids is the drug of choice and standard therapy for Minimal Change Nephrotic Syndrome (MCNS), but the drug-induced hypocalcemia and hypovitaminosis D are assessed by our study. Added to the above, the disease itself characterized by hypocalcemia and hypovitaminosis D. So, all children should undergo this assessment to prevent growth failure and pathological fractures. Nutritional supplements are recommended for the quality of life among children.

Pathogenesis of minimal change nephrotic syndrome: an immunological concept / 소아과

Idiopathic nephrotic syndrome (INS) in children is characterized by massive proteinuria and hypoalbuminemia. Minimal change nephrotic syndrome (MCNS) is the most common form of INS in children. The pathogenesis of MCNS still remains unclear, however, several hypotheses have been recently proposed. For several decades, MCNS has been considered a T-cell disorder, which causes the impairment of the glomerular filtration barrier with the release of different circulating factors. Increased levels of several cytokines are also suggested. Recently, a "two-hit" theory was proposed that included the induction of CD80 (B7-1) and regulatory T-cell (Treg) dysfunction, with or without impaired autoregulatory functions of the podocyte. In contrast to the well-established involvement of T cells, the role of B cells has not been clearly identified. However, B-cell biology has recently gained more attention, because rituximab (a monoclonal antibody directed against CD20-bearing cells) demonstrated a very good therapeutic response in the treatment of childhood and adult MCNS. Here, we discuss recent insights into the pathogenesis of MCNS in children.

Acute Kidney Injury Following Plastic Bronchitis Associated with Influenza B Virus in a Child with Nephrotic Syndrome.

Background: Plastic bronchitis is a rare but life-threatening disorder and is usually associated with congenital heart disease or pulmonary disease. Case characteristics: A 5- year-old boy with minimal change nephrotic syndrome who developed a relapse along with cough, fever and dyspnea. Observation: Chest X-ray showed atelectasis of right upper lobe of lung, and nasal swab was positive for influenza B virus. His respiratory condition worsened, and required ventilation; bronchoscopy revealed bronchial casts. This was followed by acute kidney injury which was successfully managed with hemodialysis. Message: Children with nephrotic syndrome on immunosuppressive agents can develop plastic bronchitis and acute kidney injury, following influenza virus infection.

Alteration of glomerular anionic sites and expression of renal heparanase in respiratory syncytial virus nephropathy in rats / 中华实用儿科临床杂志

Objective To observe the alteration of glomerular anionic sites and renal heparanase (Hpa)expression in respiratory syncytial virus(RSV) nephropathy in rats,and to investigate the role of Hpa in the pathogenesis of proteinuria in RSV nephropathy.Methods Twenty-five Sprague-Dawley (SD) rats were inoculated with 6 x 106plaque forming units(PFU) RSV and were sacrificed on days 4,8,14 and 28 post-inoculation(RSV4,RSV8,RSV14 and RSV28 group).Five normal SD rats served as normal control.The proteinuria and serum parameters were measured.Glomerular anionic sites were measured by histochemical electron microscopy.The expression of Hpa in kidney was determined by immunohistochemical staining.The relationship between the expression level of Hpa and the quantity of 24-hour urine protein was studied.Results After inoculation,the proteinuria increased,especially in RSV14 group.The 24-hour urine protein of RSV14 group,RSV8 group,RSV28 group,RSV4 group and normal group were (30.9860 ± 3.3464)mg,(15.3212 ± 1.2249) mg,(13.9193-2.1409) mg,(11.5857± 1.5705) mg,(3.9780 ± 0.6224) rag,respectively.The serum albumin of RSV14 group decreased.The anionic sites of glomerular basement membrane(GBM) decreased in RSV nephropathy.The number of anionic sites per 1000 nm GBM of RSV14 group,RSV8 group,RSV28 group,RSV4 group and normal control group were 12.0000 ± 1.5811,14.0000 ± 1.0000,14.6000 ± 1.1401,16.8000 ± 0.8366 and 21.2000 ± 1.3038,respectively.Hpa in glomeruli couldn't be detected in normal rats.Glomerular Hpa expression was up-regulated in RSV nephropathy.The expression of glomerular Hpa of RSV14 group,RSV8 group,RSV28 group and RSV4 group were 0.6622 ±0.1145,0.5511 ± 0.0257,0.3524 ± 0.0296 and 0.4521 ± 0.0087,respectively.The expression level of Hpa in RSV8 group and RSV14 group was higher than that in RSV4 group and RSV28 group.There was a linear positive correlation between the expression level of glomerular Hpa and the quantity of 24-hour urine protein (r =0.783,P ＜ 0.05).Conclusions The increased expression of glomerular Hpa in RSV nephropathy of rats leads to loss of the glomerular anionic sites and damage of the electrostatic barrier of GBM,which promote the proteinuria.

A Case of Adult Minimal Change Nephrotic Syndrome Associated with Thin Basement Membrane Nephropathy / 대한신장학회지

Thin basement membrane nephropathy (TBMN) is characterized by persistent hematuria, mild proteinuria, normal renal function and family history of hematuria. Many studies report that TBMN commonly occurs together with other glomerular diseases such as minimal change nephrotic syndrome, membranous nephropathy, IgA nephropathy and focal segmental glomerulosclerosis. Especially, the case of TBMN with minimal change nephrotic syndrome has been rare. We report a case of adult minimal change nephrotic syndrome with TBMN in a 44-year-old female with general edema and microscopic hematuria. On renal biopsy, electron microscopic examination demonstrated diffuse thinning of glomerular basement membrane with the thickness less than 250nm and diffuse foot process effacement. Treatment with corticosteroid resulted in complete remission of proteinuria.

A Case of Minimal Change Nephrotic Syndrome Presented as Portal Vein Thrombosis and Acute Renal Failure / 대한신장학회지

Patients with the nephrotic syndrome are at risk of developing thromboembolic complications. Much evidence suggests that a hypercoagulable state exists in the setting of the nephrotic syndrome, but the exact mechanisms are poorly understood. The nephrotic syndrome associated with portal vein thrombosis is relatively uncommon. We experienced a case of minimal change nephrotic syndrome presented as portal vein thrombosis and acute renal failure. On renal biopsy, electron microscopic examination reveals diffuse foot process effacement. Treatment with corticosteroid and anticoagulation resulted in complete remission of proteinuria.

Clinicopathologic Study on Adults Idiopathic Nephrotic Syndrome in Korea / 대한신장학회잡지

PURPOSE: We examined the clinical characteristics and incidence of adults idiopathic nephrotic syndrome (NS) according to pathologic diagnosis, age, sex. METHODS: We retrospectively reviewed the clinical and pathological characteristics of primary glomerular lesions in adults idiopathic NS taken a renal biopsy from 1978 to 2005 at the Dongsan Medical Center. We compared the prevalence of adults idiopathic NS according to the pathologic diagnosis between two time intervals 1978 to 1990 and 1991 to 2005. RESULTS: The patients had mean age of 36.7+/-16.3 years and male to female ratio was 1.7:1 with male predominance. The frequency of histopathologic diagnoses were minimal change nephrotic syndrome (MCNS) 51.6%, membranous glomerulonephritis (MGN) 21.3%, focal segmental glomerulosclerosis (FSGS) 12.1%, IgA nephropathy 9.1%, membranoproliferative glomerulonephritis (MPGN) 4.2% in decreasing order of frequency. The mean age was youngest in MCNS (32.9+/-15.1) and oldest in MGN (46.2+/-16.6). Between 1978 to 1990 period and 1991 to 2005 period, the prevalence of MGN was significantly increased, whereas the prevalence of MPGN was decreased significantly. The prevalence of MCNS had a tendency to decrease and that of IgA nephropathy had a tendency to increase, however, both didn't reach statistical significance. The incidence of FSGS didn't show a significant change during the both study periods. CONCLUSION: MCNS was the most common disease among adults idiopathic NS. MGN was the most frequent etiology in patients older than 45 years. The incidence of MGN was increased over the 28-year period, and that of MPGN decreased significantly. There was no change in the frequency of FSGS.

Methylprednisolone Pulse Therapy in Adult-Onset Minimal Change Nephrotic Syndrome / 대한신장학회지

PURPOSE: The incidence of complete remission is lower and the relapse is more frequent in adult-onset minimal change nephrotic syndrome (MCNS) are observed especially when compared with those in children. This study was designed to examine the effect of methylprednisolone pulse therapy in adultonset MCNS comparing to oral steroid as an initial therapeutic modality. METHODS: We have retrospectively reviewed the clinical data of 25 adult-onset MCNS patients. Twelve patients were treated with three intravenous pulses of methylprednisolone (1 g daily) followed by oral prednisolone 1 mg/kg daily for 4-8 weeks and also by low doses of oral prednisolone for 4-6 months (MP group) Thirteen patients were initially treated with oral prednisolone 1 mg/kg daily for 4-8 weeks and then with low doses of oral prednisolone (PD group). RESULTS: The response to therapy was similar between MP and PD group, with a complete remission obtained in 83.3% and 84.6%, respectively. No statistically significant difference between the two groups was observed in the rate of response at 8 weeks (58.3% versus 69.2%). The mean time to response was not different between MP group (37.9+/-28.0 days) and PD group (45.5+/-40.2 days). No difference was recognized between the two groups with respect to relapse rate. CONCLUSION: These data suggest that a short course of methylprednisolone pulse therapy followed by oral prednisolone is not superior to oral prednisolone therapy as an initial therapeutic modality in adult-onset MCNS.

Minimal Change Nephrotic Syndrome After Apitoxin Therapy: A Case Report / 대한신장학회지

Bee stings have previously been implicated in the development of nephrotic syndrome, but the reported cases in the literature are rare. Furthermore, there has been no case of nephrotic syndrome after bee venom (apitoxin) therapy. We experienced a 28-year-old female who developed generalized edema 6 days after an intramuscular injection of apitoxin. The physical examination and laboratory findings were relevant with nephrotic syndrome and the renal biopsy revealed minimal change nephrotic syndrome. The corticosteroid treatment induced prompt remission with resolution of edema and normalization of the laboratory findings. There was no relapse of the disease during the 6-month follow-up. We report this case together with brief review of literatures.

The Levels of IgG Subclasses and Vaccine-induced Viral Antibodies in Childhood Minimal Change Nephrotic Syndrome

PURPOSE: Hypogammaglobulinemia has been observed in nephrotic syndrome, but its pathophysiology remains unknown. We evaluated serum immunoglobulins, IgG subclasses, and vaccine-induced viral antibodies(anti-hepatitis B surface IgG and anti-measles IgG) in children with minimal change nephrotic syndrome(MCNS). METHODS: Using the stored sera, the levels of immunoglobulin(IgG, IgM, IgA, and IgE) and IgG subclasses(IgG 1, 2, 3, and 4), anti-HBs Ab and anti-measles IgG of 21 children with MCNS were analyzed and compared to those of 25 age-matched healthy children. RESULTS: The mean values of IgG and IgG1 were 390+/-187 mg/dL and 287+/-120 mg/dL in nephrotic children, and 1,025+/-284 mg/dL and 785+/-19 mg/dL in control children, respectively. The values of the total IgG and the 4 IgG subclasses in nephrotic children were all significantly depressed(P<0.001), but the IgM(251+/-183 mg/dL vs. 153+/-55 mg/dL, P=0.02) and IgE values(P=0.01) were elevated, and the IgA values were not changed. The seropositivity of anti-HBs IgG was 42.9%(9 of 21 cases) in the MCNS group and 52%(13/25) in the control group, and that of anti-measles IgG was 76%(16/21) and 92%(23/25), respectively, but there was no statistical difference between the two groups. CONCLUSION: IgG and IgG subclass levels in MCNS children are all depressed without significant seronegativity of the vaccine-induced viral antibodies. Further studies are needed to resolve the cause of hypogammaglobulinemia in MCNS.

The Mathematical Model for Predicting the Probability of Minimal Change Nephrotic Syndrome Using Clinical Parameters / 대한신장학회잡지

PURPOSE: We retrospectively investigated to find out the equation of calculating the probability of minimal change nephrotic syndrome (MCNS) using clinical parameters. We prospectively investigated to determine the usefulness of the mathematical model. METHODS: We retrospectively examined 56 patients with nephrotic syndrome (NS) (30 MCNS and 26 non-MCNS) diagnosed by kidney biopsy. A mathematical model for calculating the probability of MCNS was obtained through multiple logistic analysis in SAS statistics package. In addition, we prospectively studied 28 patients with NS. Clinical MCNS and non-MCNS were classified according to the probability of 85% in the mathematical model. Kidney biopsy was performed, and serum albumin and urinalysis were measured after 2 weeks of steroid treatment. RESULTS: In the retrospective study, the mathematical model was P=ea/(1+ea), a=17.2507 - 5.5777xON - 4.2256xALB-0.000579x24PROT - 1.2569xUBL+2.1703xUAL. The mode of onset (ON), 24 hours urine protein (24PROT), serum albumin concentration (ALB), the grade of hematuria (UBL) and proteinuria (UAL) were included as clinical parameters. At the probability of 85%, the sensitivity and specificity for predicting MCNS was 73.3% and 100% respectively. In the prospective study, the result of kidney biopsy was consistent with clinical MCNS and non-MCNS according to a mathematical model. All clinical MCNS showed negative proteinuria on urinalysis and a significant increase in serum albumin after 2 weeks treatment (1.85+/-0.30 g/dL to 2.88+/-0.26 g/dL, p<0.05). CONCLUSION: We conclude that the mathematical model for predicting the probability of MCNS may be useful in diagnosis of the MCNS.

Adult Minimal Change Nephrotic Syndrome: Treatment Response and Relapse / 대한신장학회잡지

BACKGROUND: Minimal change nephrotic syndrome (MCNS) is the most common cause of idiopathic nephrotic syndrome in Korea, not only in children but also in adults. METHODS: We reviewed the medical records of patients older than 16 years who were diagnosed MCNS by percutaneous renal biopsy between 1979 and 2002 and followed more than 6 months there after. RESULTS: Of total 94 patients enrolled, there were 58 men and 36 women (male to female 1.6: 1), the mean age of onset was 30.5 (16-73) years, and the mean follow-up period was 66 (6-297) months. Of 81 patients who were initially given corticosteroid, complete remission (CR) was observed in 68 (84%), partial remission in 7 (8.6%), and failure to remission in 6 (7.4%). In comparison of the 47 patients who showed CR by the 4th week with the group who showed CR after 4 weeks plus who did not show CR after all, male (p=0.04) and renal insufficiency (p=0.01) were more dominant in the latter group. All of 10 patients who were initially given the combination of cyclophosphamide and corticosteroid showed CR. The mean number of relapse per patient per year was 0.37 in 79 patients who achieved CR with initial treatment, 0.44 in 61 patients younger than 40 years, and 0.15 in 18 patients older than 40 years (p=0.02). Remission was maintained longer in patients older than 40 years (p=0.005), and in those with proteinuria less than 10 grams per day (p=0.04). CONCLUSION: Among patients with MCNS, those who presented with initial renal insufficiency show a less favorable response to corticosteroid. Patients older than 40 years show less frequent relapse and longer duration of remission.

Effect of Tumor Necrosis Factor-Alpha on Glomerular Epithelial Cells in Glomerular Permeability

PURPOSE: Minimal Change Disease (MCD) is the most common primary nephrotic syndrome in children. Some suggested that tumor necrosis factor-alpha (TNF-alpha) are involved in the pathogenesis of MCD. METHODS: This study was done to see the changes of plasma and urinary TNF-alpha, and its effect on the determination of permeability of the glomerular basement membrane (BM) contributed by heparan sulfate proteoglycan (HSPG). Study patients consisted of 19 biopsy-proven MCD children aged 2-15 years old. Both plasma and urinary TNF-alpha were measured. Employing the Millicell system, TNF-alpha was screened for the permeability factors. We examined whether TNF-alpha regulated BM HSPG gene expression and HS synthesis in the glomerular epithelial cells (GECs). RESULTS: Urinary TNF-alpha during relapse was significantly increased when compared with that of during remission or controls (364.4+/-51.2 vs 155.3+/-20.8, 36.0+/-4.5 ng/mg cr) (P< 0.05). However, negative results were obtained in the permeability assay using the Millicell system. No difference was seen in the BM HSPG gene expression and HS synthesis in the GECs. CONCLUSION: It seems that TNF-alpha may not play a disease-specific role in the pathogenesis of MCD.

A Case of Adult-Onset Minimal Change Nephrotic Syndrome Relapsed after 15-year of Complete Remission / 대한신장학회잡지

We report an unusual case of adult minimal change nephrotic syndrome relapsed after 15-year of complete remission. In this case, the disease had occurred to the patient for the first time when he was 52 years old; relatively high age, and had been remitted with steroid therapy. After 15 years of complete remission, he visited our hospital with the symptoms of the generalized edema and the pitting edema of both lower extremities that occurred 15 days ago. Massive proteinuria(15, 865 mg/day) and hypoalbuminemia(1.7 g/dL) were detected. The pathologic evaluation of the biopsied specimen of the kidney showed the global sclerosis in 19% of glomeruli in light microscopic finding and the fusion of epithelial foot processes in electron microscopic finding. He was treated with pulse steroid therapy (methylprednisolone 500 mg/day iv for 3 days) and then, with oral prednisolone (60 mg/day). Generalized edema and proteinuria disappeared after 14 days of treatment, and there has been no relapse ever since. Adult-onset minimal change nephrotic syndrome relapses within 4 years after complete remission in 90 % of relapsed patients. The relapse after more than 5 years of complete remission, like this case, is very rare, especially in the case of late-onset disease. However, the possibility of relapse of the minimal change nephrotic syndrome after several years of its remission should be considered constantly and the long-term follow-up more than 10 years may be needed.

A case of minimal change nephrotic syndrome associated with systemic lupus erythematosus / 대한내과학회지

Systemic Lupus Erythematosus (SLE) is an autoimmune disease characterized by the overprocuctionofautoantibodiesandthedepositionofimmune complexes in various organs. Unusual case of systemic lupus erythematosus (SLE) associated with minimal change nephrotic syndrome(MCNS) is described. A 30-year-old woman who has been diagnosed as SLE and treated with prednisolone presented symptoms of nephrotic syndrome. Renal biopsy revealed minor glomerular abnormalities without deposition of immune complexes. The initial heavy proteinuria promptly decreased after the prednisolone dosage was increased and disappeared 10 weeks later. She developed proteinuria again 3 years after the initial episode. Repeated renal biopsy revealed membranous nephropathy. T-cell dysfunction, which is present both in SLE and MCNS, might have triggered MCNS during the course of SLE.