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1.
J Med Case Rep ; 18(1): 192, 2024 Apr 20.
Artigo em Inglês | MEDLINE | ID: mdl-38641649

RESUMO

BACKGROUND: Immunoglobulin G4-related disease is an inflammatory disease affecting multiple organs including the kidney. Immunoglobulin G4-related kidney disease most commonly manifests as a tubulointerstitial nephritis and is associated with glomerular disease in a proportion of cases. Membranous nephropathy is the most frequent glomerular lesion. Herein, we report the first documented case of immunoglobulin G4-related disease presenting with nephrotic syndrome owing to minimal change disease. CASE PRESENTATION: A 67-year-old South Asian male presented to our service with systemic upset and leg swelling. He had heavy proteinuria (urine protein:creatinine ratio 1042 mg/mmol) and was hypoalbuminemic (17 g/L) and hypercholersterolemic (9.3 mmol/L), consistent with the nephrotic syndrome. His serum creatinine was 140 µmol/L, and he was hypocomplementemic (C3 0.59 g/L, C4 < 0.02 g/L) with raised immunoglobulin G4 subclass levels (5.29 g/L). Kidney biopsy demonstrated minimal change disease alongside a plasma-cell-rich tubulointerstitial nephritis with strong positive staining for immunoglobulin G4. A diagnosis of minimal change disease in the setting of immunoglobulin G4-related disease was made. He was commenced on oral prednisolone at 60 mg daily but suffered infectious complications, including necrotizing fasciitis within 3 weeks of starting treatment, ultimately resulting in his death 52 days after initial presentation. CONCLUSION: This case highlights the potential for immunoglobulin G4-related disease to be associated with a spectrum of glomerular pathologies including minimal change disease. It adds to the differential diagnosis of secondary causes of minimal change disease, and moreover, aids as an important reminder of the potential complications of high-dose steroids used in its treatment.


Assuntos
Doença Relacionada a Imunoglobulina G4 , Nefrite Intersticial , Nefrose Lipoide , Síndrome Nefrótica , Humanos , Masculino , Idoso , Doença Relacionada a Imunoglobulina G4/complicações , Síndrome Nefrótica/complicações , Síndrome Nefrótica/diagnóstico , Síndrome Nefrótica/tratamento farmacológico , Nefrose Lipoide/complicações , Nefrose Lipoide/diagnóstico , Nefrose Lipoide/tratamento farmacológico , Nefrite Intersticial/diagnóstico , Nefrite Intersticial/tratamento farmacológico , Imunoglobulina G
2.
BMC Nephrol ; 25(1): 53, 2024 Feb 09.
Artigo em Inglês | MEDLINE | ID: mdl-38336671

RESUMO

BACKGROUND: Minimal change nephrotic syndrome (MCNS) can be complicated by thymoma; however, no standard therapy for thymoma-associated MCNS has yet been established. We herein describe a case of steroid-resistant MCNS associated with thymoma, treated effectively with rituximab. CASE PRESENTATION: A 71-year-old Japanese man was referred to our department with severe proteinuria (20 g/gCr). Renal biopsy showed minimal change disease and computed tomography revealed an anterior mediastinal mass. Based on these findings, he was diagnosed with thymoma-associated MCNS. He was treated with oral prednisolone (50 mg/day) and cyclosporine, and underwent thymectomy and plasma exchange. However, no improvement in proteinuria was observed. He therefore received intravenous rituximab 500 mg, resulting in a marked decrease in proteinuria from 5328 to 336 mg/day after 1 week. CONCLUSIONS: This case suggests that rituximab might be an effective therapy in patients with steroid-resistant MCNS associated with thymoma.


Assuntos
Nefrose Lipoide , Síndrome Nefrótica , Timoma , Neoplasias do Timo , Masculino , Humanos , Idoso , Timoma/complicações , Timoma/diagnóstico por imagem , Timoma/tratamento farmacológico , Ciclosporina/uso terapêutico , Nefrose Lipoide/complicações , Nefrose Lipoide/tratamento farmacológico , Rituximab/uso terapêutico , Timectomia/efeitos adversos , Neoplasias do Timo/complicações , Neoplasias do Timo/cirurgia , Síndrome Nefrótica/complicações , Prednisolona , Proteinúria/etiologia
3.
Am J Nephrol ; 55(1): 25-36, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-37963441

RESUMO

INTRODUCTION: Rituximab has been proven effective and safe in pediatric patients with frequently relapsing or steroid-dependent nephrotic syndrome (FR/SDNS). We aimed to analyze the efficacy and safety of rituximab in adult FR/SDNS patients with minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS). METHODS: A retrospective cohort study at three nephrology centers in China included adult FR/SDNS patients with biopsy-proven MCD or FSGS. Primary outcomes were relapse frequency and first relapse-free survival time. Adverse events were well recorded, and logistic regression analyses were used to investigate the risk factors of relapse. RESULTS: Eighty-one patients (age, 25.0 years; interquartile range, 20.0-40.5; 67% males; 82.7% MCD) received an average rituximab dose of 1,393.8 ± 618.7 mg/2 years during the 2-year follow-up period. The relapse frequency, calculated as the ratio of relapse times to follow-up years, significantly decreased after rituximab treatment (0.04 [0.00, 0.08] vs. 1.71 [1.00, 2.45], p < 0.001). The first relapse-free survival time was 16.7 ± 8.0 months. Fifty-seven patients (70.4%) achieved cessation of corticosteroids and immunosuppressants within 3 months after the first rituximab infusion. Adverse events were mostly mild, and no severe treatment-related adverse events were observed. Low serum albumin level before rituximab and high CD56+CD16+ natural killer cell count after rituximab were independent risk factors of relapse within 2 years after rituximab treatment. CONCLUSION: Rituximab was proven an effective and safe treatment option for adult FR/SDNS patients with MCD or FSGS in maintaining disease remission and minimizing corticosteroid exposure.


Assuntos
Glomerulosclerose Segmentar e Focal , Nefrose Lipoide , Síndrome Nefrótica , Masculino , Adulto , Humanos , Criança , Feminino , Rituximab/efeitos adversos , Glomerulosclerose Segmentar e Focal/complicações , Glomerulosclerose Segmentar e Focal/tratamento farmacológico , Estudos Retrospectivos , Síndrome Nefrótica/tratamento farmacológico , Síndrome Nefrótica/induzido quimicamente , Nefrose Lipoide/tratamento farmacológico , Nefrose Lipoide/induzido quimicamente , Imunossupressores/efeitos adversos , Recidiva , Doença Crônica , Resultado do Tratamento
4.
CEN Case Rep ; 13(1): 53-58, 2024 02.
Artigo em Inglês | MEDLINE | ID: mdl-37244881

RESUMO

Mass vaccination is the most important strategy to terminate the coronavirus disease 2019 (COVID-19) pandemic. Reports suggest the potential risk of the development of new-onset or relapse of minimal change disease (MCD) following COVID-19 vaccination; however, details on vaccine-associated MCD remain unclear. A 43-year-old man with MCD, who had been in remission for 29 years, developed nephrotic syndrome 4 days after receiving the third dose of the Pfizer-BioNTech vaccine. His kidney biopsy revealed relapsing MCD. Intravenous methylprednisolone pulse therapy followed by oral prednisolone therapy was administered, and his proteinuria resolved within 3 weeks. This report highlights the importance of careful monitoring of proteinuria after COVID-19 vaccination in patients with MCD, even if the disease is stable and no adverse events occurred during previous vaccinations. Our case report and literature review of COVID-19 vaccine-associated MCD indicated that MCD relapse tends to occur later after vaccination and slightly more often following the second and subsequent vaccine doses than new-onset MCD.


Assuntos
COVID-19 , Nefrose Lipoide , Masculino , Humanos , Adulto , Vacinas contra COVID-19/efeitos adversos , COVID-19/prevenção & controle , Nefrose Lipoide/diagnóstico , Nefrose Lipoide/tratamento farmacológico , Nefrose Lipoide/etiologia , Vacinação/efeitos adversos , Doença Crônica , Proteinúria , RNA Mensageiro
5.
Clin Exp Nephrol ; 28(3): 217-224, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37924431

RESUMO

BACKGROUND: Although rituximab (RTX) is recommended by kidney disease improving global outcomes as one of the standard therapies for primary membranous nephropathy (pMN), given the constraint of insurance coverage, it is not clear how the drug is used in Japan. METHODS: This cross-sectional study was conducted via a web-based survey between November and December 2021. The participants were certified nephrologists and recruited through convenience sampling. Experience with RTX for pMN was compared to experience with RTX for minimal change nephrotic syndrome (MCNS). Reasons for withholding RTX for pMN, even when it is indicated, were also investigated. Furthermore, the proportion difference in RTX experience was analyzed. RESULTS: Responses from 380 nephrologists across 278 facilities were analyzed. RTX was used for pMN by 83 (21.8%), which was less than the 181 (47.6%) who had used RTX for MCNS (ratio of proportions: 0.46). RTX use for pMN was more frequent in facilities performing 41-80 and 81 or more kidney biopsies annually (vs. none) and by physicians with experience in anti-PLA2R antibody measurement. RTX administration for pMN was covered by insurance for 56 (67.5%), was facility-paid for 10 (12.0%), and was copaid by patients for 6 (7.2%). The most common reason for withholding RTX for pMN was difficulty in ensuring financing (146, 79.3%). CONCLUSIONS: RTX use for pMN is less common than for MCNS but not infrequent. Treatment with RTX was more frequent in biopsy-intensive facilities, and it was fully paid by the facility or patient in one-fifth of cases.


Assuntos
Glomerulonefrite Membranosa , Nefrose Lipoide , Humanos , Rituximab/uso terapêutico , Glomerulonefrite Membranosa/patologia , Nefrologistas , Japão , Estudos Transversais , Nefrose Lipoide/tratamento farmacológico , Internet
6.
Clin Exp Med ; 23(8): 5367-5376, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37796359

RESUMO

Mild mesangial proliferative IgA nephropathy with minimal change disease (MCD-IgAN) and mild mesangial proliferative IgA nephropathy without minimal change disease (Non-MCD-IgAN) have similar characteristics on light microscopy. Nevertheless, their discrepancies in clinicopathological features and prognosis remain unknown. A total of 589 patients with biopsy-proven mild mesangial proliferative IgA nephropathy (M-IgAN) combined with light microscopy and immunofluorescence were enrolled. Firstly, the diagnoses of the patients by electron microscopy were recorded and used as the gold standard. We calculated the sensitivity and specificity using nephrotic syndrome (NS) as the diagnostic criteria to identify MCD-IgAN. Then, excluding patients with a 24-h urinary total protein less than 0.5 g/day, incomplete clinical data, or less than the six-month follow-up, we included 184 cases of non-MCD-IgAN and 98 cases of MCD-IgAN. The patients' clinicopathological and outcome data were collected and compared. Among the 589 patients, according to electron microscopy, 381 were diagnosed with non-MCD-IgAN, 167 with MCD-IgAN, and 41 with M-IgAN complicated by other glomerular diseases. Using NS as the diagnostic criteria to distinguish non-MCD-IgAN and MCD-IgAN, the sensitivity and specificity were 83.8% and 99.5%, respectively. The patients in the MCD-IgAN group tended to be younger, hypotensive, with lower urinary erythrocytes, and more likely to achieve complete remission, and fewer patients progressed to the endpoint than those in the non-MCD-IgAN group (all P < 0 .05). NS appears to be an objective indicator for differentiating MCD-IgAN from non-MCD-IgAN. Non-MCD-IgAN varies greatly from MCD-IgAN in clinicopathology and treatment response, with a poorer prognosis.


Assuntos
Glomerulonefrite por IGA , Nefrose Lipoide , Síndrome Nefrótica , Humanos , Glomerulonefrite por IGA/diagnóstico , Glomerulonefrite por IGA/complicações , Nefrose Lipoide/diagnóstico , Nefrose Lipoide/complicações , Nefrose Lipoide/tratamento farmacológico , Síndrome Nefrótica/etiologia , Prognóstico , Estudos Retrospectivos
7.
Medicine (Baltimore) ; 102(41): e35470, 2023 Oct 13.
Artigo em Inglês | MEDLINE | ID: mdl-37832087

RESUMO

RATIONALE: A predominant Th2 immune response is suggested in the pathogenesis of both minimal change disease (MCD) and membranous nephropathy (MN); however, consecutive development of the 2 diseases in a patient is extremely rare. PATIENT CONCERN: A Japanese man, who developed nephrotic syndrome in his 50s and was diagnosed with MCD by renal biopsy, experienced a relapse of proteinuria approximately 3 years later during long-term steroid treatment. Since the proteinuria was resistant to increase in steroid dosage, repeat renal biopsy was performed, which revealed a small amount of glomerular subepithelial immune deposits containing immunoglobulin (Ig)G (dominantly IgG4). Immunostaining for thrombospondin-type-1-domain-containing-7A (THSD7A) was positive on the glomerular capillary walls, whereas that for other causative antigens of MN, such as phospholipase A2 receptor or neural epidermal growth factor-like 1 protein, was negative. Detailed examination found no associated condition, including malignancies and allergic diseases. DIAGNOSIS: The diagnosis of THSD7A-associated idiopathic MN was made. INTERVENTIONS AND OUTCOMES: He received further increased dose of steroids. Thereafter he maintained clinical improvement because his urinary protein level was decreased. LESSONS: The present case suggested that histological transition from MCD to MN is possible and repeat biopsy would be crucial for accurate diagnosis.


Assuntos
Glomerulonefrite Membranosa , Nefrose Lipoide , Masculino , Humanos , Nefrose Lipoide/induzido quimicamente , Nefrose Lipoide/tratamento farmacológico , Nefrose Lipoide/patologia , Glomérulos Renais/patologia , Proteinúria/patologia , Imunoglobulina G , Esteroides , Autoanticorpos , Receptores da Fosfolipase A2
8.
BMJ Case Rep ; 16(9)2023 Sep 15.
Artigo em Inglês | MEDLINE | ID: mdl-37714559

RESUMO

We report the case of nephrotic syndrome after COVID-19 vaccination. The patient was a man in his 30s with no comorbidities other than atopic dermatitis. Over the course of 2 weeks after the first COVID-19 vaccination, systemic oedema gradually appeared. He was referred to the nephrology department for investigation of the systemic oedema. On admission, he presented with pitting oedema in his lower extremities. Initial examinations revealed massive urinary protein and decreased serum albumin, at 13.9 g/g Cr and 1.5 g/dL, respectively. Renal biopsy was performed, and minimal change disease was diagnosed. Prednisolone 60 mg/day was promptly started on the 5th day of hospitalisation, and complete remission was achieved on the 12th day. Prednisolone was once tapered off in 1.5 years successfully though minimal change disease was relapsed in 1 month after the steroid withdrawal.


Assuntos
COVID-19 , Nefrose Lipoide , Masculino , Humanos , Vacina de mRNA-1273 contra 2019-nCoV , COVID-19/prevenção & controle , Vacinas contra COVID-19/efeitos adversos , Nefrose Lipoide/tratamento farmacológico , Nefrose Lipoide/etiologia , Prednisolona/uso terapêutico
9.
PLoS One ; 18(8): e0289870, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37590275

RESUMO

Minimal change disease (MCD) is characterized by edema and nephrotic range proteinuria (NS). However, the fate of MCD without nephrotic proteinuria requires elucidation. We retrospectively reviewed 79 adults diagnosed with primary MCD at their initial renal biopsy at a tertiary hospital between May 2003 and June 2017. Clinicopathologic features were compared between patients with and without NS. The frequency of flaring to nephrotic proteinuria and renal outcomes were assessed during follow-up. There were 20 and 59 patients in the Non-NS and NS groups, respectively. The Non-NS group had a lower frequency of acute kidney injury (AKI) during the follow-up period [5.0% vs. 59.3%, p <0.001]. The response rate to steroid treatment was 100% in the Non-NS group and 92.3% in the NS group (p = 1.000). Except for one patient, the Non-NS group was treated with steroids when their proteinuria increased to a nephrotic level. There were no differences in the frequency of the first relapse or the number of relapses among patients with initial remission from nephrotic range proteinuria. At the final visit, the complete remission rate was 73.4%. The estimated glomerular filtration rate during follow-up was significantly better in the NS group than the Non-NS group, given the higher rates of AKI at renal biopsy. The rates of renal events, end-stage renal disease, and mortality did not differ between the groups. Adult MCD patients with nephrotic and non-nephrotic range proteinuria showed similar outcomes. Accordingly, this population must be carefully managed, regardless of the amount of proteinuria at renal biopsy.


Assuntos
Injúria Renal Aguda , Nefrose Lipoide , Adulto , Humanos , Nefrose Lipoide/complicações , Nefrose Lipoide/tratamento farmacológico , Estudos Retrospectivos , Rim , Proteinúria
10.
Medicine (Baltimore) ; 102(22): e33892, 2023 Jun 02.
Artigo em Inglês | MEDLINE | ID: mdl-37266627

RESUMO

RATIONALE: Only 1 case of IgA nephropathy (IgAN) with minimal change disease (MCD) associated with primary Sjögren's syndrome (SS) has been reported. We additionally describe IgAN with MCD associated with primary SS. PATIENT CONCERNS: A 80-year-old woman visited our hospital complaining of generalized edema that had started 4 weeks prior. She reported a sense of thirst and dry eye for the last 5 years. DIAGNOSES: Her initial laboratory findings were compatible with nephrotic syndrome; both the antinuclear antibody (1:80) and anti-SS-A (Ro) antibody (200 U/mL) tests were positive. A salivary gland scan revealed markedly decreased uptake for both the parotid and submandibular glands. The Schirmer test was positive. The random urine protein/creatinine ratio was 10 mg/mg. Renal biopsy was compatible with IgAN with superimposed MCD. INTERVENTIONS: Furosemide was intravenously administered with intermittent albumin infusion for her edema control. She was started on prednisone 40mg daily for 6 weeks, which was tapered to 5 mg for another 6 months after starting prednisolone. OUTCOMES: Over the next 6 months, her edema improved and the proteinuria decreased significantly. LESSONS: Physician should suspect IgA with MCD when patient with SS clinically showed nephrotic syndrome, and perform renal biopsy for pathologically diagnosis and appropriate treatment.


Assuntos
Glomerulonefrite por IGA , Nefrose Lipoide , Síndrome Nefrótica , Síndrome de Sjogren , Humanos , Feminino , Idoso de 80 Anos ou mais , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/tratamento farmacológico , Síndrome Nefrótica/complicações , Nefrose Lipoide/complicações , Nefrose Lipoide/diagnóstico , Nefrose Lipoide/tratamento farmacológico , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/diagnóstico , Glomerulonefrite por IGA/tratamento farmacológico , Edema/diagnóstico
11.
Clin Exp Nephrol ; 27(9): 767-775, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-37310570

RESUMO

BACKGROUND: With the publication of the "Evidence-Based Clinical Practice Guideline for Nephrotic Syndrome 2020," we examined nephrologists' adherence to the recommendations of four of its clinical questions (CQs). METHODS: This was a cross-sectional web-based survey conducted between November and December 2021. The target population comprised nephrologists certified by the Japanese Society of Nephrology who were recruited using convenience sampling. The participants answered six items regarding the four CQs about adult patients with nephrotic syndrome and their characteristics. RESULTS: In total, 434 respondents worked in at least 306 facilities, of whom 386 (88.9%) provided outpatient care for primary nephrotic syndrome. Of these patients, 179 (41.2%) answered that they would not measure anti- phospholipase A2 receptor antibody levels in cases of suspected primary membranous nephropathy (MN) in which kidney biopsy was not possible (CQ1). Regarding immunosuppressants as maintenance therapy after relapse of minimal change nephrotic syndrome (CQ2), cyclosporine was the most common choice (290 [72.5%] and 300 [75.0%] of 400 respondents after the first and second relapses, respectively). The most common treatment for steroid-resistant cases of primary focal segmental glomerulosclerosis (CQ3) was cyclosporine (323 of 387, 83.5%). For the initial treatment of primary MN with nephrotic-range proteinuria (CQ4), corticosteroid monotherapy was the most common choice (240 of 403, 59.6%), followed by corticosteroid and cyclosporine (114, 28.3%). CONCLUSION: Gaps in recommendations and practices regarding serodiagnosis and treatment of MN (i.e., CQ1 and 4) are observed, suggesting the need to address the barriers to their insurance reimbursement and the lack of evidence behind them.


Assuntos
Glomerulonefrite Membranosa , Glomerulosclerose Segmentar e Focal , Fidelidade a Diretrizes , Nefrose Lipoide , Síndrome Nefrótica , Adulto , Humanos , Corticosteroides/uso terapêutico , Estudos Transversais , Ciclosporina , População do Leste Asiático , Glomerulonefrite Membranosa/diagnóstico , Glomerulonefrite Membranosa/tratamento farmacológico , Glomerulosclerose Segmentar e Focal/tratamento farmacológico , Internet , Nefrologistas , Nefrose Lipoide/diagnóstico , Nefrose Lipoide/tratamento farmacológico , Síndrome Nefrótica/diagnóstico , Síndrome Nefrótica/tratamento farmacológico , Padrões de Prática Médica , Inquéritos e Questionários
12.
CEN Case Rep ; 12(4): 368-373, 2023 11.
Artigo em Inglês | MEDLINE | ID: mdl-36809634

RESUMO

Kimura disease (eosinophilic granuloma of the soft tissue) is a benign granulomatous disease complicated by nephrotic syndrome. Herein, we report a case of recurrent minimal change nephrotic syndrome (MCNS) complicated by Kimura disease that was successfully treated with rituximab. A 57-year-old man presented to our hospital with relapsed nephrotic syndrome with worsening swelling of the right anterior ear and elevated serum IgE. MCNS was diagnosed on renal biopsy. Treatment with 50 mg of prednisolone rapidly placed the patient in remission. Therefore, RTX 375 mg/m2 was added to the treatment regimen, and steroid therapy was tapered. Early steroid tapering was successful, and the patient is currently in remission. In this case, the nephrotic syndrome flare-up was accompanied by worsening Kimura disease. Rituximab reduced the worsening of symptoms related to Kimura disease, including head and neck lymphadenopathy and elevated IgE levels. Kimura disease and MCNS may share a common IgE-mediated type I allergic condition. Rituximab effectively treats these conditions. In addition, rituximab suppresses Kimura disease activity in patients with MCNS, enables early tapering of steroids, and reduces the total dose of steroids.


Assuntos
Doença de Kimura , Nefrose Lipoide , Síndrome Nefrótica , Humanos , Masculino , Pessoa de Meia-Idade , Imunoglobulina E , Doença de Kimura/complicações , Nefrose Lipoide/complicações , Nefrose Lipoide/diagnóstico , Nefrose Lipoide/tratamento farmacológico , Síndrome Nefrótica/complicações , Síndrome Nefrótica/diagnóstico , Síndrome Nefrótica/tratamento farmacológico , Rituximab/uso terapêutico , Esteroides/uso terapêutico
13.
Saudi J Kidney Dis Transpl ; 34(3): 242-249, 2023 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-38231719

RESUMO

Minimal change disease (MCD) is the most common cause of nephrotic syndrome (NS) in children, and in adults, it contributes to 10%-25% of NS. MCD in adults follows a slightly different course associated with increased incidence of steroid resistance, hematuria, and HTN. This is a prospective-record analysis study aimed to analyze the profile of MCD in adults, response to treatment, and relapse rates. A retrospective observational study was carried out and data were collected retrospectively from all biopsy-proven MCD patients between 2012 and 2018. A total of 86 adults were diagnosed to have biopsy-proven MCD. Of these, 32 were excluded due to insufficient data/lost for follow-up. The IBM SPSS Statistics version 22.0 was used for the statistical analysis. Descriptive analysis includes expression of all the explanatory and outcome variables in terms of frequency and proportions for categorical variables whereas in terms of mean ± standard deviation for continuous variables. Chi-square test was used to compare the age, gender, remission, renal failure and response of different drugs, treatment durations, comorbidity conditions, relapse episodes, and different types of infections based on the degree of proteinuria among study patients. A total of 54 biopsy-proven adult MCD patients were analyzed. The mean age of the patients studied was 36.67 years, with the oldest patient being 76 years. In the study group, 37 (68.5%) patients were male and 14 (31.5%) were female. In the study population, 20 (37%) were hypertensive, 3 (5.6%) were diabetic, and 10 (18.5%) had renal failure at presentation. On treatment, 52 out of 54 patients received steroids, of which 41 (75.9%) were steroid responsive, 6 (11.1%) steroid dependent, and 7 (13%) steroid resistant. The mean time for remission in steroidsensitive patients was 8.8 weeks. Among the steroid-dependent and steroid-resistant patients, 11 patients received calcineurin inhibitors (CNIs), of which 3 were CNI resistant. In the study Group 1 patient received cyclophosphamide and two received rituximab. In the study population, two patients failed to achieve remission and one patient was initiated on hemodialysis and later lost for follow-up. Minimal change NS is a type of NS which is highly responsive to steroids with good prognosis in children. Adult MCD patients require a higher and prolonged course of steroid when compared to children. CNIs and rituximab form a promising second-line drug in patients who are steroid resistant/dependent. However, CNI dependency or relapse after stopping steroids is a concern.


Assuntos
Nefrose Lipoide , Síndrome Nefrótica , Insuficiência Renal , Criança , Adulto , Humanos , Masculino , Feminino , Rituximab/uso terapêutico , Nefrose Lipoide/diagnóstico , Nefrose Lipoide/tratamento farmacológico , Nefrose Lipoide/epidemiologia , Estudos Retrospectivos , Estudos Prospectivos , Centros de Atenção Terciária , Síndrome Nefrótica/diagnóstico , Síndrome Nefrótica/tratamento farmacológico , Síndrome Nefrótica/epidemiologia , Inibidores de Calcineurina/efeitos adversos , Biópsia , Insuficiência Renal/complicações , Esteroides/uso terapêutico , Recidiva
14.
Nephrology (Carlton) ; 27(12): 953-961, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36209374

RESUMO

BACKGROUND: Patients with idiopathic minimal change nephrotic syndrome (MCNS) undergoing immunosuppressive therapy are susceptible to infectious complications. Study specifically focusing on adult population's infectious complications is lacking. METHODS: We retrospectively collected 101 adult patients with biopsy-proven idiopathic MCNS and analysed for the infectious complications. Published literatures were also reviewed aiming to evaluate the feasibility of prophylactic antibiotic treatment. RESULTS: Infectious complications developed in 17 of 101 (16.8%) patients, with pneumonia (n = 4), cellulitis/fasciitis (n = 4) and urinary tract infection (UTI) (n = 4) being the dominant diseases, and Gram-negative bacilli the main cause. AKI stage ≥2 (Hazard ratio = 6.1; 95% CI: 1.2-31.9, p = 0.031) and non-remission by treatment (Hazard ratio = 4.4; 95% CI: 1.2-15.6, p = .023) were the two independent risk factors relevant to developing infectious complications. Review of 16 published literatures and our data showed that even no prophylactic antibiotic therapy, only one case of Pneumocystis jirovecii pneumonia developed among the 1787 accumulative cases of MCNS. In contrast, 16 (44%) of acute flare cases were reported among the 36 patients with positive hepatitis B surface antigen that did not receive antiviral prophylactic therapy. CONCLUSIONS: Advanced acute kidney injury and non-remission by treatment are the risk factors toward developing infectious complications in adult MCNS undergoing immunosuppressive therapy. It appears unnecessary to use prophylactic antibiotic for Pneumocystis jirovecii pneumonia or other bacterial infections, while screening and prophylactic therapy for hepatitis B and latent tuberculosis are critical for patients in prevalent area.


Assuntos
Injúria Renal Aguda , Nefrose Lipoide , Síndrome Nefrótica , Pneumonia por Pneumocystis , Adulto , Humanos , Nefrose Lipoide/complicações , Nefrose Lipoide/tratamento farmacológico , Nefrose Lipoide/diagnóstico , Estudos Retrospectivos , Pneumonia por Pneumocystis/complicações , Injúria Renal Aguda/complicações , Terapia de Imunossupressão , Síndrome Nefrótica/etiologia
15.
Clin J Am Soc Nephrol ; 17(12): 1823-1834, 2022 12.
Artigo em Inglês | MEDLINE | ID: mdl-36198505

RESUMO

Idiopathic nephrotic syndrome often responds to immunosuppressive treatment. Nevertheless, this syndrome-and the drugs used to treat it-remain important causes of patient morbidity. Idiopathic nephrotic syndrome is usually caused by minimal change disease or FSGS, diseases that primarily affect the podocytes. In spite of decades of research, the underlying causes of both diseases remain incompletely understood. There is, however, a large body of observational and experimental data linking the immune system with both minimal change disease and FSGS, including associations with systemic infections and hematologic malignancies. Perhaps most compellingly, many different immunomodulatory drugs are effective for treating idiopathic nephrotic syndrome, including biologic agents that have well-defined immune targets. In fact, the unexpected efficacy of targeted therapeutic agents has provided important new insights into the pathogenesis of these diseases. Given the large number of drugs that are available to deplete or block specific cells and molecules within the immune system, a better understanding of the immunologic causes of idiopathic nephrotic syndrome may lead to better diagnostic and therapeutic approaches.


Assuntos
Glomerulosclerose Segmentar e Focal , Nefrose Lipoide , Síndrome Nefrótica , Humanos , Síndrome Nefrótica/tratamento farmacológico , Síndrome Nefrótica/patologia , Nefrose Lipoide/tratamento farmacológico , Nefrose Lipoide/complicações , Glomerulosclerose Segmentar e Focal/tratamento farmacológico , Sistema Imunitário/patologia
16.
Eur Rev Med Pharmacol Sci ; 26(20): 7556-7560, 2022 10.
Artigo em Inglês | MEDLINE | ID: mdl-36314326

RESUMO

BACKGROUND: The aim of the study was to summarize the clinical features, diagnosis and treatment of leucine-rich glioma inactivation protein 1 (LGI-1) antibody-associated encephalitis coexistence of minimal change nephrotic syndrome (MCNS), moreover, to strengthen the awareness of the disease. Increasing number of studies describe coexistence of autoimmune encephalitis and other systemic autoimmune diseases. CASE REPORT: Here we report a case of a patient with anti- LGI1 antibody-associated encephalitis, who presented with cognitive dysfunction, faciobrachial dystonic seizures (FBDS), sleep disturbance, and hyponatremia. Treatment with immunoglobulins, corticosteroids, levetiracetam and oxcarbazepine was proven effective for this patient. The patient had a history of MCNS diagnosed by renal biopsy and responded to treatment with low dose of oral corticosteroids. CONCLUSIONS: This case expanded the spectrum of autoimmune comorbidities in patients with anti-LGI1 encephalitis.


Assuntos
Encefalite , Glioma , Encefalite Límbica , Nefrose Lipoide , Humanos , Encefalite Límbica/complicações , Encefalite Límbica/diagnóstico , Leucina , Nefrose Lipoide/complicações , Nefrose Lipoide/diagnóstico , Nefrose Lipoide/tratamento farmacológico , Autoanticorpos , Peptídeos e Proteínas de Sinalização Intracelular , Encefalite/diagnóstico , Encefalite/tratamento farmacológico , Glioma/tratamento farmacológico , Corticosteroides/uso terapêutico
17.
J Vet Intern Med ; 36(6): 2187-2190, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-36151875

RESUMO

A 3-year-old entire female Springer Spaniel, with a previous diagnosis of meningoencephalitis of unknown origin diagnosed 2 years before presentation and treated with long term administration of prednisolone, developed proteinuria. Laboratory findings revealed hypoalbuminemia, hypercholesterolemia, and proteinuria. Further investigations excluded underlying causes. Renal biopsies were performed. The glomeruli and the tubulointerstitial compartment did not show any anomalies on light microscopy and immunofluorescence staining did not reveal abnormalities. Transmission electron microscopy revealed moderate podocyte injury consisting of foot process effacement and microvillus transformation of the cytoplasm. The dog was diagnosed with primary minimal change disease of the podocytes and treated with telmisartan and mycophenolate mofetil. Abnormalities of serum albumin, cholesterol, and proteinuria resolved within 4 weeks. Minimal change disease has been reported in dogs, but this is a case report of proteinuria secondary to minimal change disease successfully treated with mycophenolate mofetil and telmisartan.


Assuntos
Doenças do Cão , Nefrose Lipoide , Cães , Feminino , Animais , Nefrose Lipoide/tratamento farmacológico , Nefrose Lipoide/veterinária , Nefrose Lipoide/complicações , Ácido Micofenólico/uso terapêutico , Telmisartan/uso terapêutico , Proteinúria/tratamento farmacológico , Proteinúria/veterinária , Glomérulos Renais/patologia , Doenças do Cão/tratamento farmacológico , Doenças do Cão/patologia
18.
Nephrol Ther ; 18(6): 549-556, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-36123282

RESUMO

The present study aimed to investigate the efficacy and safety of tacrolimus for treating incipient minimal change disease in adults. The clinical data of 52 adult patients with minimal change disease of nephrotic syndrome diagnosed by renal biopsy in the First affiliated hospital of Zhengzhou University between August 2013 and August 2015 were retrospectively analyzed. According to the treatment plan, the patients were divided into a tacrolimus group and a glucocorticoid group. The efficacy and safety of tacrolimus in the treatment of minimal change disease in adult patients was analyzed and compared with that of glucocorticoids. The results revealed that the baseline characteristics of the two groups were similar (P>0.05). At 24 weeks, there was a significant difference in serum albumin between the two groups (P<0.01). The serum albumin levels of tacrolimus group was higher compared with the glucocorticoid group. In addition, the complete remission rates in the tacrolimus and glucocorticoid groups were 93.75 and 77.8%, respectively (P=0.095), and the mean complete remission time was 6.33±4.21 and 5.14±2.45 weeks, respectively (P=0.175). The relapse rate was 12.5 and 22.2% in the tacrolimus and glucocorticoid groups, respectively (P=0.368). During the follow-up, in tacrolimus group, the incidence of new onset diabetes or impaired glucose tolerance, osteoporosis, infection, abnormal liver function, Cushing's syndrome, acne and gastrointestinal symptoms were significantly less than those of glucocorticoids (P<0.05). In conclusion, tacrolimus treatment after short-time intravenous methylprednisolone is an effective treatment option with fewer adverse effects in adult onset minimal change disease.


Assuntos
Nefrose Lipoide , Síndrome Nefrótica , Adulto , Humanos , Tacrolimo/efeitos adversos , Nefrose Lipoide/tratamento farmacológico , Metilprednisolona/efeitos adversos , Estudos Retrospectivos , Glucocorticoides/uso terapêutico , Imunossupressores/uso terapêutico , Síndrome Nefrótica/etiologia , Resultado do Tratamento , Albumina Sérica/análise
19.
J Osteopath Med ; 122(10): 499-501, 2022 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-35700437

RESUMO

This case follows a 54-year-old woman with a medical history of hypertension who experienced reactivation of minimal change disease (MCD) after receiving the Pfizer vaccine against COVID-19. She had her first episode of MCD 15 days after receiving the influenza vaccine in 2018. She remained in remission for over 3 years following treatment with steroids. She experienced foamy urine and leg edema after receiving the first dose of the Pfizer vaccine, but she did not consult medical professionals until she received the second dose. She wanted to be fully vaccinated because she worked in healthcare. Her initial diagnosis of MCD in 2018 was made following a kidney biopsy. The diagnosis of reactivation following COVID-19 vaccine was made with labs and presenting symptoms. At presentation, her urine protein was 9,977 mg/day. She was treated with prednisone 50 mg/day following her relapse with improvement in her urine protein to 85 mg/g within 4 weeks of starting treatment. She is currently undergoing treatment with prednisone with improvement in her presenting symptoms, which included foaming of urine and edema of legs. This case demonstrates the importance of vigilance in patients with a history of MCD when receiving the COVID-19 vaccine, particularly if they have a history of such reactions to other vaccines. Patients should discuss the benefits and risks of receiving the vaccine with their medical professionals and stay cognizant about the possibility of reactivation after receiving the COVID-19 vaccine.


Assuntos
Tratamento Farmacológico da COVID-19 , Vacinas contra COVID-19 , Nefrose Lipoide , Vacinas contra COVID-19/efeitos adversos , Edema , Feminino , Humanos , Vacinas contra Influenza/uso terapêutico , Pessoa de Meia-Idade , Nefrose Lipoide/tratamento farmacológico , Nefrose Lipoide/etiologia , Prednisona/uso terapêutico
20.
Sci Rep ; 12(1): 9782, 2022 06 13.
Artigo em Inglês | MEDLINE | ID: mdl-35697724

RESUMO

Previous studies reported conflicting results regarding an association between serum albumin concentration and the cumulative incidence of remission of proteinuria in adult patients with minimal change disease (MCD). The present study aimed to clarify the clinical impact of serum albumin concentration and the cumulative incidence of remission and relapse of proteinuria in 108 adult patients with MCD at 40 hospitals in Japan, who were enrolled in a 5-year prospective cohort study of primary nephrotic syndrome, the Japan Nephrotic Syndrome Cohort Study (JNSCS). The association between serum albumin concentration before initiation of immunosuppressive treatment (IST) and the cumulative incidence of remission and relapse were assessed using multivariable-adjusted Cox proportional hazards models. Remission defined as urinary protein < 0.3 g/day (or g/gCr) was observed in 104 (96.3%) patients. Of 97 patients with remission within 6 month of IST, 42 (43.3%) developed relapse defined as ≥ 1.0 g/day (or g/gCr) or dipstick urinary protein of ≥ 2+. Serum albumin concentration was significantly associated with remission (multivariable-adjusted hazard ratio [95% confidence interval] per 1.0 g/dL, 0.57 [0.37, 0.87]), along with eGFR (per 30 mL/min/1.73 m2: 1.43 [1.08, 1.90]), whereas they were not associated with relapse. A multivariable-adjusted model showed that patients with high eGFR level (≥ 60 mL/min/1.73 m2) and low albumin concentration (≤ 1.5 g/dL) achieved significantly early remission, whereas those with low eGFR (< 60 mL/min/1.73 m2) and high albumin concentration (> 1.5 g/dL) showed significantly slow remission. In conclusion, lower serum albumin concentration and higher eGFR were associated with earlier remission in MCD, but not with relapse.


Assuntos
Nefrose Lipoide , Síndrome Nefrótica , Adulto , Estudos de Coortes , Humanos , Imunossupressores/uso terapêutico , Nefrose Lipoide/complicações , Nefrose Lipoide/tratamento farmacológico , Síndrome Nefrótica/tratamento farmacológico , Estudos Prospectivos , Proteinúria/tratamento farmacológico , Recidiva , Indução de Remissão , Estudos Retrospectivos , Albumina Sérica
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