scholarly journals Is podocytopathy another image of renal affection in p-SLE?

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Hend H. Abdelnabi
Keyword(s):  
Lupus Erythematosus ◽  
Renal Involvement ◽  
Histopathological Analysis ◽  
Immune Deposits ◽  
Foot Process ◽  
Lupus Podocytopathy ◽  
Focal Segmental Sclerosis ◽  
Nephrotic Range Proteinuria

Abstract Background Lupus podocytopathy (LP) is a renal affection described in systemic lupus erythematosus (SLE) patients with nephrotic range proteinuria, characterized by diffuse foot process effacement without immune deposits and glomerular proliferation. This study describes LP, its pathological features and outcomes of pediatric (p-SLE) patients in comparison to the usual lupus nephritis (LN) cases. Methodology A retrospective cohort study conducted on a 10-year registration (2010–2019) of 140 p-SLE patients at the Pediatric Department, Tanta University. Histopathological analysis with light microscopy (LM) and immunofluorescence (IF) of all renal biopsies were evaluated according to the International Society of Nephrology Renal Pathology Society (ISN/RPS) grading system. In addition, some biopsies were examined with electron microscopy (EM). Results Eighty-six p-SLE cases (61.4%) had renal involvement; seventy-nine biopsies (91.86%) of them met the classification criteria of LN as defined by ISN/RPS system. Five biopsies were normal (MCD) and two showed focal segmental sclerosis (FSGN) that did not meet any known classification of LN. Hence, they were reevaluated using EM that revealed diffuse effaced podocytes without glomerular sub-epithelial, endocapillary or basement membrane immune deposits, and were classified as having lupus podocytopathy, representing (8.14%) of all LN biopsies. Those seven cases showed good response to steroids with a complete remission duration of 3.40 ± 1.95 weeks. However, some case had 1–3 relapses during the duration of follow up. Conclusions LP is a spectrum of p-SLE, not an association as it is related to disease activity and its initial presentation.

scholarly journals Delayed Onset Minimal Change Disease as a Manifestation of Lupus Podocytopathy

2021 ◽  
Vol 11 (4) ◽  
pp. 747-754
Author(s):  
Rasha Aly ◽  
Xu Zeng ◽  
Ratna Acharya ◽  
Kiran Upadhyay
Keyword(s):  
Renal Function ◽  
Lupus Erythematosus ◽  
Minimal Change Disease ◽  
Normal Serum ◽  
Minimal Change ◽  
Renal Histology ◽  
Foot Process ◽  
Lupus Podocytopathy

Lupus podocytopathy (LP) is an uncommon manifestation of systemic lupus erythematosus (SLE) and is not included in the classification of lupus nephritis. The diagnosis of LP is confirmed by the presence of diffuse foot process effacement in the absence of capillary wall deposits with or without mesangial immune deposits in a patient with SLE. Here we describe a 13-year-old female who presented with nephrotic syndrome (NS) seven years after the diagnosis of SLE. The renal function had been stable for seven years since the SLE diagnosis, as manifested by the normal serum creatinine, serum albumin and absence of proteinuria. Renal biopsy showed evidence of minimal change disease without immune complex deposits or features of membranous nephropathy. Renal function was normal. The patient had an excellent response to steroid therapy with remission within two weeks. The patient remained in remission five months later during the most recent follow-up. This report highlights the importance of renal histology to determine the accurate etiology of NS in patients with SLE. Circulating factors, including cytokines such as interleukin 13, may play a role in the pathophysiology of LP and needs to be studied further in future larger studies.

scholarly journals Recurrent podocytopathy in a patient with systemic lupus erythematosus

2017 ◽  
Vol 5 ◽  
pp. 2050313X1769599
Author(s):  
Shereen Paramalingam ◽  
Daniel D Wong ◽  
Gursharan K Dogra ◽  
Johannes C Nossent
Keyword(s):  
Electron Microscopy ◽  
Systemic Lupus Erythematosus ◽  
Nephrotic Syndrome ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Systemic Lupus Erythematosus Activity ◽  
Growing Body ◽  
Foot Process ◽  
Lupus Podocytopathy

Podocytopathy in systemic lupus erythematosus is characterised by diffuse foot process effacement without significant peripheral capillary wall immune deposits as seen on electron microscopy. Lupus podocytopathy falls outside the scope of the current International Society of Nephrology and the Renal Pathology Society classification of lupus nephritis. We present a case of relapsing podocytopathy with nephrotic syndrome occurring simultaneously with two extra-renal and serological disease flares, which makes it likely that podocytopathy was related to systemic lupus erythematosus activity. This case adds to the growing body of evidence that lupus podocytopathy must be considered in the differential diagnosis of systemic lupus erythematosus patients presenting with nephrotic syndrome.

scholarly journals A case of lupus podocytopathy and a review of the literature

2021 ◽  
Vol 35 (1) ◽  
pp. 39-42
Author(s):  
José Silvano ◽  
◽  
Augusta Praça ◽  
Inês Ferreira ◽  
Ana Nunes ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Minimal Change Disease ◽  
Kidney Injury ◽  
Mesangial Proliferation ◽  
Foot Process ◽  
Complex Deposition ◽  
Lupus Podocytopathy

Nephrotic syndrome in systemic lupus erythematosus patients with histological evidence of minimal change disease, mesangial proliferation or focal and segmental glomerulosclerosis on light microscopy, represents a distinct clinical entity – lupus podocytopathy. This entity is characterized by a diffuse foot process effacement on electron microscopy and by absence of subepithelial or subendothelial immune -complex deposition. We report the case of a 49 -year -old woman admitted on suspicion of lupus nephritis flare, characterized by nephrotic syndrome and acute kidney injury, whose renal biopsy revealed histological features of lupus podocytopathy. Six months after discharge, under prednisolone and azathioprine, she presented 300 mg/day proteinuria, normal kidney function, without hematuria. A review of the pathogenesis, clinical features, diagnostic criteria, treatment and prognosis of lupus podocytopathy is provided. This case highlights the mounting evidence that lupus podocytopathy encompasses distinct clinical and morphological features, that should be included in the upcoming pathological classification of lupus nephritis.

Electron microscopy study of 496 cases of lupus nephritis: A single-center experience

Lupus ◽  
2021 ◽  
pp. 096120332098453
Author(s):  
Bahar Bagheri ◽  
Seyed Mohammad Owji ◽  
Simin Torabinezhad ◽  
Hadi Raeisi Shahraki ◽  
Amirhossein Kamalinia ◽  
...  
Keyword(s):  
Electron Microscopy ◽  
Lupus Nephritis ◽  
Light Microscopy ◽  
Lupus Erythematosus ◽  
Interstitial Fibrosis ◽  
Renal Involvement ◽  
Correct Classification ◽  
Tubular Atrophy ◽  
Diagnosis And Classification

Introduction Renal involvement is seen in about 40-82% of systemic lupus erythematosus (SLE) Asian patients. The exact diagnosis and classification of lupus nephritis are important for treatment and prognosis. This study aimed to investigate the value of electron microscopy (EM) in the diagnosis and classification of lupus nephritis compared with light microscopy. Method In this cross-sectional referral-center 16-year study of lupus nephritis, the final diagnosis was based on the EM study. Primary light microscopy findings were compared with EM diagnosis. Moreover, Immunofluorescence patterns distribution was assessed. Results From 496 patients diagnosed with lupus nephritis based on EM, 225(45.4%) of patients were categorized in class IV, followed by 98(19.7%), 93(18.8%), 46(9.3%), and 14(2.8%) who were categorized into classes of II, III, V, and VI respectively. Only 1(0.2%) patient belonged to class I, and 19(3.8%) cases were diagnosed with mixed two classes. Using EM was essential for diagnosing 25.6% of cases taking the correct classification by light microscopy into account; however, disregarding correct classification, this could change to a 7.4% contribution rate of EM. The most common cause of misdiagnosis, disregarding incorrect classification, was inadequate or wrong tissue. Positive associations were detected between tubular atrophy and interstitial fibrosis of both electron and light microscopy with different classes (P < 0.001). Conclusion While light microscopy is highly accurate for diagnosing lupus nephritis regardless of correct classification, EM contributes substantially to the correct classification of lupus nephritis types.

scholarly journals A nationwide study of SLE in Japanese identified subgroups of patients with clear signs patterns and associations between signs and age or sex

Lupus ◽  
2014 ◽  
Vol 23 (13) ◽  
pp. 1435-1442 ◽  
Author(s):  
C Terao ◽  
R Yamada ◽  
T Mimori ◽  
K Yamamoto ◽  
T Sumida
Keyword(s):  
Lupus Erythematosus ◽  
Disease Duration ◽  
Renal Involvement ◽  
Disease Status ◽  
First Year ◽  
Clinical Markers ◽  
Autoantibody Production ◽  
Nationwide Study ◽  
Negatively Associated

We performed a nationwide study to determine the distributions of the signs and clinical markers of systemic lupus erythematosus (SLE) and identify any patterns in their distributions to allow patient subclassification. We obtained 256,999 patient-year records describing the disease status of SLE patients from 2003 to 2010. Of these, 14,779 involved patients diagnosed within the last year, and 242,220 involved patients being followed up. Along with basic descriptive statistics, we analyzed the effects of sex, age and disease duration on the frequencies of signs in the first year and follow-up years. The patients and major signs were clustered using the Ward method. The female patients were younger at onset. Renal involvement and discoid eczema were more frequent in males, whereas arthritis, photosensitivity and cytopenia were less. Autoantibody production and malar rash were positively associated with young age, and serositis and arthritis were negatively associated. Photosensitivity was positively associated with a long disease duration, and autoantibody production, serositis and cytopenia were negatively associated. The SLE patients were clustered into subgroups, as were the major signs. We identified differences in SLE clinical features according to sex, age and disease duration. Subgroups of SLE patients and the major signs of SLE exist.

Applying SLICC and ACR/EULAR systemic lupus erythematosus classification criteria in a cohort of patients with undifferentiated connective tissue disease

Lupus ◽  
2020 ◽  
pp. 096120332097693
Author(s):  
Kyle R Drehmel ◽  
Alan R Erickson ◽  
Bryant R England ◽  
Kaleb D Michaud ◽  
Harlan R Sayles ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Medical Center ◽  
Statistical Significance ◽  
Academic Medical Center ◽  
Renal Involvement ◽  
Classification Criteria ◽  
Academic Medical ◽  
Dsdna Antibody ◽  
Record Review

Background/objective New classification criteria for SLE have recently been developed. How these criteria affect the classification of patients with the SLE-mimicking condition UCTD is poorly understood. This study investigated the reclassification of UCTD patients using newly derived SLE criteria. Methods Patients with UCTD were identified within a single academic medical center using ICD9/10 codes. Medical record review was performed to confirm UCTD diagnosis and identify disease features present at diagnosis. The SLICC and ACR/EULAR criteria were applied, after which we compared the proportion of patients reclassified as SLE and determined which disease features were associated with reclassification. Results A total of 129 patients were included in the study. When applying the SLICC and ACR/EULAR criteria, 18 (14.0%) and 26 patients (20.2%) were reclassified as SLE. Comparison with McNemar’s test trended toward statistical significance (p = 0.057). Cohen’s kappa coefficient was 0.62 (p < 0.001), indicating substantial agreement between these criteria. Disease features associated with reclassification as SLE were renal involvement, leukopenia, thrombocytopenia, anti- dsDNA antibody, hypocomplementemia, non-scarring alopecia (SLICC), and arthritis (ACR/EULAR). Conclusions Both the SLICC and ACR/EULAR criteria exhibit increased SLE classification. These newer classification criteria could be used to increase the number of SLE patients in future clinical studies.

scholarly journals The Various Forms of Nephrotic Syndrome in a Patient with Systemic Lupus Erythematosus

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Sophia Lionaki ◽  
George Liapis ◽  
Kalliope Vallianou ◽  
Chrysovalantis Vergadis ◽  
Ioannis Boletis
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Nephrotic Syndrome ◽  
Lupus Erythematosus ◽  
Disease Onset ◽  
Systemic Lupus ◽  
Kidney Involvement ◽  
Lupus Podocytopathy ◽  
Systemic Symptoms ◽  
Serological Activity

Kidney involvement is frequent in patients with systemic lupus erythematosus (SLE), although it may not be present from disease onset. Renal lupus itself is highly heterogenous with respect to the combination and/or severity of clinical and/or laboratory manifestations. This is a case of a 45-year-old Caucasian female with an established diagnosis of SLE, who presented four times with new onset of proteinuria during a follow-up time of ten years, since the diagnosis of SLE. Specifically, she experienced two episodes of lupus membranous nephropathy, and after she achieved remission, she developed twice overt nephrotic syndrome associated with new and biopsy proven lupus podocytopathy. All these episodes of nephrotic syndrome were combined with systemic symptoms, attributed to lupus itself, while serological activity of lupus was also noted. This case highlights the importance of performing a kidney biopsy in all patients with SLE who have new renal manifestations, including nephrotic proteinuria.

AB0246 FACTORS ASSOCIATED WITH THE TIME OF PRESENTATION OF CARDIOVASCULAR EVENTS IN A COHORT OF COLOMBIAN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1423-1424
Author(s):  
S. Herrera ◽  
J. C. Diaz-Coronado ◽  
D. Rojas-Gualdrón ◽  
L. Betancur-Vasquez ◽  
D. Gonzalez-Hurtado ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Cardiovascular Risk ◽  
Lupus Erythematosus ◽  
Renal Involvement ◽  
P Value ◽  
Systemic Lupus ◽  
P Values ◽  
Interval Censored ◽  
Age And Sex

Background:Systemic lupus erythematosus is a systemic disease characterized by a compromise of vital organs. The autoimmune activity has been linked to accelerated endothelial damage and increased cardiovascular risk and its outcomes such as heart attack, stroke, and peripheral arterial disease(1). Patients with Lupic nephritis have been characterized by requiring aggressive immunosuppressive therapies apart from prolonged and progressive use of corticosteroids, what you have shown can accelerate these outcomes(2). Other factors such as secondary arterial hypertension, dyslipidemia among others are factors to consider (3).Objectives:To analyze clinical and immunological characteristics associated with time to severe renal involvement in patients with Systemic Lupus Erythematous in a Colombian cohort followed for one year, between January 2015 and December 2018.Methods:Retrospective follow-up study based on clinical records of patients with SLE diagnosis that fulfilled either 1987 American College of Rheumatology Classification Criteria for SLE or 2011 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria for SLE. Patients with cardiovascular disease outcomes such as angina, acute myocardial infarction, stroke, transient cerebral ischemia and chronic arterial occlusive disease were included. Patients who did not have at least two follow-up measurements or had structural heart disease, valvulopathies, arrhythmias, myocarditis, pericarditis were excluded. The main outcome was defined as the time from diagnosis to cardiovascular diseases.Clinical and immunological characteristics were analyzed. Descriptive statistical analyses of participant data during the first evaluation are reported as frequencies and percentages for categorical variables, and as medians and interquartile ranges for quantitative variables. Age and sex adjusted survival functions and Hazard Ratios (HR) with 95% confidence intervals and p-values were estimated using parametric Weibull models for interval-censored data. P values < 0.05 were considered statistically significantResults:547 patients were analyzed: 29 were left-censored as they presented renal involvement at entry, 22 were interval censored as outcome occurred between study visits, and 496 were right-censored as involvement was not registered during follow-up. 528 (96.5%) patients were female, median age at entry was 46 (IQR = 23) and median age to diagnosis was 29.4 (IQR = 20.9). Statistically significant age and sex adjusted variables were High Blood Pressure (HBP) HR = 2.0 (95%CI 1.1-3.6; p-value <0.018) and cumulative prednisolone dose (>10 gr vs <2 gr) HR = 2.4 (95%CI 1. 1-5.1; p-value = 0.023). Figure 1 shows the age and sex adjusted survival function for HBPConclusion:HBP and cumulative steroid doses accelerate the onset of cardiovascular diseases in patients with lupus more than two times. Maintaining blood pressure in goals and performing early clearance of glucocorticoids could improve outcomes in these patients who are already considered a high cardiovascular riskReferences:[1]Hans-Joachim Anders, Ramesh Saxena, Ming-Hui Zhao. Lupus Nephritis. Nat Rev Dis Primers. 2020 Jan 23;6(1):7.[2]Shanthini Kasturi, Lisa R Sammaritano. Corticosteroids in Lupus. Rheum Dis Clin North Am, 42 (1), 47-62, viii[3]César Magro-Checa, Juan Salvatierra, José Luis Rosales-Alexander, et al. Cardiovascular risk in systemic lupus erythematosus: implicated factors and assessment methods. Semin Fund Esp Reumatol. 2012;13(3):95–102Disclosure of Interests:Sebastian Herrera Speakers bureau: academic conference, Juan camilo Diaz-Coronado: None declared, Diego Rojas-Gualdrón: None declared, Laura Betancur-Vasquez: None declared, Daniel Gonzalez-Hurtado: None declared, Juanita Gonzalez-Arango: None declared, laura Uribe-Arango: None declared, Maria Fernanda Saavedra Chacón: None declared, Jorge Lacouture-Fierro: None declared, Santiago Monsalve: None declared, Sebastian Guerra-Zarama: None declared, Juan david Serna: None declared, Julian Barbosa: None declared, Deicy Hernandez-Parra: None declared, Ana Sierra: None declared, Ricardo Pineda.Tamayo: None declared

Pediatric onset lupus nephritis in western India—is it different from the rest of the country?

Lupus ◽  
2021 ◽  
pp. 096120332110450
Author(s):  
Aliza Mittal ◽  
Harshita Bamnawat ◽  
Aasma Nalwa ◽  
Vikarn Vishwajeet ◽  
Prawin Kumar ◽  
...  
Keyword(s):  
Renal Failure ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Care Center ◽  
Renal Involvement ◽  
Tertiary Care ◽  
Neurological Involvement ◽  
Western India ◽  
Pediatric Systemic Lupus Erythematosus

Aim To determine the clinicopathological characteristics and outcomes of children diagnosed with lupus nephritis in a tertiary hospital in western Rajasthan and compare it with the data available from other parts of India. Material and methods: A retrospective review of children presenting to a tertiary care center in western Rajasthan, India, with a diagnosis of pediatric Systemic Lupus Erythematosus (p SLE), between July 2017 and July 2020 was done. Comparisons of pediatric lupus in western India to other parts of country were done. Results 19 children with SLE with Renal involvement were enrolled and followed up. The median age at presentation was 15 years (IQR-16–9.5) (73% females). 8/19 (42%) children presented with AKI, of which 62% children presented as rapidly progressive renal failure. Six (37.5%) patients required dialysis at presentation. 84.21% of children were evaluated with renal biopsy, 16 biopsies were done in 19 children, among which class II, III, and IV lupus nephritis were reported in 21%,42%, and 35% respectively(4 crescentic). Antiphospholipid antibodies were positive in 8/15(53%), children which is much higher than a reported incidence of 30% in other Indian studies. Ten patients (52%) had neurological involvement, with seizures being the most common form of presentation (60%). Seven patients (36%) developed hepatitis. We noted many uncommon presentations in the small group like Autoimmune Pancreatitis, Mononeuritis multiplex, and peripheral digital gangrene. Cyclophosphamide was used in 10 out of 19 patients for inducing remission with class 3 and 4 nephritis and MMF in 8 children. 55% patients attained remission (after completing induction), of which 4 relapsed during the follow up. Four patients were lost to follow-up. A total of 27% patients died and 10% patients developed end stage renal failure. It was seen that those who died had more cardiac and neurological involvement at presentation, higher grade of proteinuria, lower GFR, and need for dialysis at admission. Conclusion: We found a more severe form of clinical manifestation in pediatric SLE patients at the time of the first presentation in the form of severe renal and extrarenal manifestation compared to other parts of the country.

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