Predictors of mortality in children with lupus nephritis

Background Renal involvement during the clinical course ofsystemic lupus erythematosus (SLE) is generally considered to bethe most important factor influencing disease prognosis in termsof morbidity and mortality. Various factors have been reported toinfluence the prognosis of lupus nephritis (LN).Objective To analyze clinical signs and laboratory parameters thatmight serve as predictors associated with mortality in pediatricLN.Methods Retrospectively, medical records of children with LNat Soetomo Hospital from 1998 to 2011 were studied. Diagnosisof SLE was based on Revised American Rheumatism Associationcritera, while patients with clinical manifestations of hypertension,abnormal urinalysis, and serum creatinin > 1 mg/dL wereconsidered as lupus nephritis. Cox proportional hazard modelingwas used to assess for associations of clinical signs and laboratoryparameters with mortality. Kaplan-Meier survival analysis wasused to assess the cumulative survival from the time of diagnosisto the outcome.Results There were 57 children with LN of whom 43 (75%) weregirls. The female-to-male ratio was 3:1. Subjects’ mean age was 10.6(SD 6.87) years. The mean time of observation was 51 (SD 74.54)months and 23 (40%) children died. Age, gender, hypertension,hematuria, proteinuria, and anemia were not significant aspredictors for mortality. However, hypertensive crisis (HR=2.79;95%CI 1.16 to 6.75; P=0.02) and initial glomerular filtration rate(GFR) of <75 mL/min/1.73m2 (HR=3.01; 95%CI 1.23 to 7.34;P=0.01) were significant predictors of mortality in children with LN.The mean survival time of LN with hypertensive crisis and initialGFR <75 mL/min/1.73m2 was 36.9 (SD 12.17) months.Conclusion Hypertensive crisis and GFR <75 mL/min/1.73m2 aresignificant predictors of mortality in children with LN.

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AB0750 FUNCTIONAL CONDITION OF KIDNEYS IN THE LONG-TERM COURSE OF SLE IN ADOLESCENTS

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.3930 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 1403.2-1403

Author(s):

L. Bohmat ◽

N. Shevchenko ◽

I. Bessonova

Keyword(s):

Renal Function ◽

Lupus Nephritis ◽

Lupus Erythematosus ◽

Disease Duration ◽

Renal Involvement ◽

Clinical Manifestations ◽

Clinical Analysis ◽

Average Dose ◽

One Year

Background:Lupus nephritis is the most severe and adverse systemic lupus erythematosus (SLE) syndrome. According to modern recommendations, the clinical manifestations of active nephritis should be taken under medical control in 6 months after the start of the disease’s treatment1.Objectives:The aim of this study was to examine the functional status of the kidneys in children with SLE in the course of the disease for more than one year.Methods:The analysis included case histories of 43 patients with SLE, mostly females (41), aged 7 to 18 years (mean age 14.4 years) with disease duration of 4.75 ± 0.58 years of whom 22 were less than three years, 21 - more than three years. All children received corticosteroid therapy, at the time of the examination the average dose was 13.85 ± 1.86 mg per day in terms of prednisolone. The second component of therapy was azathioprine (average dose 97.61 ± 2.11 mg). All children received hydroxychloroquine (5 mg/kg per day).To determine the functional state of the kidneys a clinical analysis of urine, a study of the scope of specific gravity of urine during the day (Zymnytsky test), the content of creatinine and urea in serum to determine the glomerular filtration rate (GFR), the level of microalbuminuria per day were evaluated.Results:Renal involvement in the developed SLE occurred in 73.08% of patients. Among them, therapy during the first 6 months was considered quite effective in 58.06% of patients. It was found that in children with disease duration from one to three years proteinuria was registered in 68.18%, a decrease in GFR in 4.45% and hyperfiltration in 9.09%. In the group of children with duration of SLE more than three years revealed deeper changes in renal function; there was proteinuria in 90.47%, the frequency of GFR decreased was in 19.04%, a decrease of renal concentration function was in 14.28% of cases.Indicators of renal function in children with SLE depending on the duration of the disease (M ± m)IndicatorDuration of the diseasefrom 1 year to 3 years n = 22over 3 yearsn = 21Creatinine, mmol/l0,080 ± 0,0140,090 ± 0,018Мочевина, mmol/l5,66 ± 1,425,63 ± 1,61GFR, ml/min117,05 ± 19,68100,20 ± 18,98 *Microalbuminuria, mg/day24,41 ± 13,1334,73 ± 24,76Density min1,007 ± 0,0051,006 ± 0,005Density max1,024 ± 0,0051,019 ± 0,005 ***р<0,03;**р<0,01 the probability of differences when comparing between groupsConclusion:Long-term follow-up of children with SLE over one year reveals a prolongation of renal dysfunction, which worsens after three years, and is the basis for the development of irreversible renal impairment.References:[1]European evidence-based recommendations for the diagnosis and treatment of childhood-onset lupus nephritis: the SHARE initiative /Noortje Groot, Nienke de Graeff, Stephen D Marks et all. //Ann Rheum Dis. 2017 Dec;76(12):1965-1973.Disclosure of Interests:None declared

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Propylthiouracil-induced diffuse proliferative lupus nephritis: review of immunological complications.

Journal of the American Society of Nephrology ◽

10.1681/asn.v871205 ◽

1997 ◽

Vol 8 (7) ◽

pp. 1205-1210

Author(s):

G V Prasad ◽

S Bastacky ◽

J P Johnson

Keyword(s):

Lupus Nephritis ◽

Lupus Erythematosus ◽

Renal Involvement ◽

Clinical Manifestations ◽

Graves Disease ◽

Specific Intervention ◽

Systemic Lupus ◽

Drug Induced ◽

Proliferative Lupus Nephritis ◽

Acute Renal Insufficiency

Propylthiouracil (PTU), used to treat Graves' disease, occasionally induces a lupus-like syndrome. A 39-year-old woman developed clinical manifestations of systemic lupus erythematosus with rash, serositis, myocarditis, and acute renal insufficiency, associated with serologies for lupus, after 3 wk of exposure to the drug. Renal biopsy revealed diffuse proliferative lupus nephritis. This article reviews the side effects of PTU and the literature on PTU-induced nephrotoxicity. Possible mechanisms and management of drug-induced lupus nephritis are also reviewed. To facilitate early and specific intervention, clinicians should be aware of the propensity of PTU to cause lupus-like syndromes with renal involvement.

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Interleukin-34 as a marker for subclinical proliferative lupus nephritis

Lupus ◽

10.1177/0961203320914976 ◽

2020 ◽

Vol 29 (6) ◽

pp. 607-616

Author(s):

Asmaa SM Abdel-Rehim ◽

Nesrine A Mohamed ◽

Marwa M Shakweer

Keyword(s):

Lupus Nephritis ◽

Lupus Erythematosus ◽

Activity Index ◽

Renal Involvement ◽

Clinical Manifestations ◽

Surrogate Marker ◽

Disease Activity Index ◽

Class Iii ◽

Group I ◽

Dsdna Antibodies

Background Lupus nephritis (LN) is an ominous manifestation of systemic lupus erythematosus (SLE). Clinical renal affection is present in about 70% of lupus patients, and more patients have histological evidence of renal involvement without clinical manifestations. This study aimed to investigate the potential role of serum interleukin-34 (IL-34) as an early marker for the detection of silent LN. Methods Thirty-three lupus patients with silent LN (group I), 37 patients with clinical LN (group II) and 20 controls were included. The SLE Disease Activity Index (SLEDAI), IL-34, anti-dsDNA antibodies and renal biopsy were assessed in all patients. Results Serum IL-34 levels were significantly higher in all lupus patients compared to healthy controls ( p < 0.001) and showed a significant positive correlation with SLEDAI score. SLE patients with positive anti-dsDNA antibodies had more active disease according to SLEDAI and higher levels of IL-34 than those with negative anti-dsDNA antibodies. In both studied groups, serum IL-34 levels were significantly higher in patients with proliferative LN (class III and class IV) than those with non-proliferative lupus (class II and class V) and controls. Yet, in both groups, IL-34 was not useful in differentiating active from chronic renal affection. Conclusion In lupus patients with insignificant proteinuria, serum levels of IL-34 distinguished the different histological classes of subclinical LN. Serum IL-34 may be used as a surrogate marker for early renal affection in silent LN, especially the proliferative type.

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THE CLINICAL, LABORATORY MANIFESTATIONS AND HISTOPATHOLOGY IN NEPHROTIC PATIENTS WITH LUPUS NEPHRITIS

Journal of Medicine and Pharmacy ◽

10.34071/jmp.2018.2.9 ◽

2018 ◽

pp. 52-58

Author(s):

Le Thuan Nguyen ◽

Bui Bao Hoang

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Nephritis ◽

Lupus Erythematosus ◽

Clinical Laboratory ◽

Activity Index ◽

Clinical Manifestations ◽

Systemic Lupus ◽

Cross Sectional ◽

Organ Systems ◽

Tubulointerstitial Lesions

Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disease involving multiple organ systems. The kidney appears to be the most commonly affected organ, especially nephrotic is a serious kidney injury. The clinical, laboratory manifestations and histopathology are very useful for diagnosis, provide the means of predicting prognosis and guiding therapy in nephrotic patients with lupus nephritis. Methods: Descriptive cross-sectional study of nephrotic patients with lupus treated in the Department of Nephrology Trung Vuong Hospital and Cho Ray Hospital between May/2014 and May/2017. Renal histopathological lesions were classified according to International Society of Nephrology/Renal Pathology Society - ISN/RPS ’s 2003. The clinical, laboratory manifestations and histopathological features were described. Results: Of 32 LN with nephritic range proteinuria cases studied, 93.7% were women. The 3 most common clinical manifestations were edema (93.8%), hypertension (96.8%) and pallor (68.9%), musculoskeletal manifestions (46.9%), malar rash (40.6%). There was significant rise in laboratory and immunological manifestions with hematuria (78.1%), Hb < 12g/dL (93.5%), increased Cholesterol (100%), and Triglycerid (87.5%), Creatinine > 1.4 mg/dL (87.5%), increased BUN 71.9%, ANA (+) 93.8%, Anti Ds DNA(+) 96.9%, low C3: 96.9%, low C4: 84.4%. The most various and severe features were noted in class IV with active tubulointerstitial lesions and high activity index. Conclusion: Lupus nephritis with nephrotic range proteinuria has the more severity of histopathological feature and the more severity of the more systemic organ involvements and laboratory disorders were noted. Key words: Systemic lupus, erythematosus (SLE) lupus nepphritis, clinical

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The presence of lupus nephritis additionally increases the risk of preeclampsia among pregnant women with systemic lupus erythematosus

Lupus ◽

10.1177/09612033211004716 ◽

2021 ◽

pp. 096120332110047

Author(s):

Katarina Bremme ◽

Sonja Honkanen ◽

Iva Gunnarsson ◽

Roza Chaireti

Keyword(s):

Caesarean Section ◽

Lupus Nephritis ◽

Pregnant Women ◽

Lupus Erythematosus ◽

Premature Birth ◽

Renal Involvement ◽

Significant Risk ◽

Obstetric Outcomes ◽

Increased Risk ◽

Renal Lupus

Introduction Pregnant women with systematic lupus erythematosus (SLE) have an increased risk of obstetric complications, such as preeclampsia and premature births. Previous studies have suggested that renal involvement could further increase the risk for adverse obstetric outcomes. Aims: The aim of this study was to compare the obstetric outcomes in a Swedish cohort of patients with SLE with and without lupus nephritis (LN). Patients and methods The study was conducted as a retrospective observational study on 103 women with SLE, who gave birth at the Karolinska University Hospital between the years 2000-2017. Thirty-five women had previous or active LN and 68 women had non-renal lupus. Data was collected from digital medical records. The outcomes that were analysed included infants born small for gestational age (SGA), premature birth, preeclampsia, SLE- or nephritis flare and caesarean section. Results Women with LN, both with previous and with renal flare during pregnancy suffered from pre-eclampsia more often compared to women with non-renal lupus (25.7% vs 2.9%, p = 0.001) and this complication was associated with premature birth (p = 0.021) and caesarean section (p = 0.035). Conclusions Lupus nephritis is a significant risk factor for adverse obstetric outcomes in women with SLE, including preeclampsia. Those patients could benefit from more frequent antenatal controls and more vigorous follow-up.

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Apolipoprotein E Gene Polymorphisms in Saudi Patients with Systemic Lupus Erythematosus

Clinical Medicine Insights Arthritis and Musculoskeletal Disorders ◽

10.4137/cmamd.s38090 ◽

2016 ◽

Vol 9 ◽

pp. CMAMD.S38090 ◽

Cited By ~ 3

Author(s):

Hannan Al-Rayes ◽

Ghaleb Huraib ◽

Saeed Julkhuf ◽

Misbahul Arfin ◽

Mohammad Tariq ◽

...

Keyword(s):

Apolipoprotein E ◽

Lupus Erythematosus ◽

Neuropsychiatric Symptoms ◽

Renal Involvement ◽

Clinical Manifestations ◽

Biological Properties ◽

Apoe Polymorphism ◽

Genotype 3 ◽

Apolipoprotein E Gene ◽

Apoe Gene Polymorphism

Apolipoprotein E (APOE) is a glycosylated protein with multiple biological properties. APOE gene polymorphism plays a central role in lipid metabolism and has recently been suggested to regulate inflammation. Our objective is to evaluate whether APOE polymorphism affects susceptibility to SLE. APOE genotyping was performed using ApoE StripAssay™ kit. Results indicated significantly higher frequencies of allele ∊4 and genotype ∊3/∊4 and lower frequencies of allele ∊3 and genotype ∊3/∊3 in SLE patients than controls. APOE ∊2 allele was found in three patients, whereas it was absent in controls. The frequencies of allele ∊4 and genotype ∊3/∊4 were significantly higher in SLE patients with renal involvement and those of alleles ∊2, ∊4 and genotypes ∊2/∊3, ∊3/∊4 were higher in patients with neuropsychiatric symptoms. It is concluded that APOE allele ∊4 is associated with susceptibility risk/clinical manifestations of SLE and ∊2 may increase its severity while ∊3 is protective for SLE in Saudis.

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A comparative study on clinical and serological characteristics between patients with rhupus and those with systemic lupus erythematosus and rheumatoid arthritis

Lupus ◽

10.1177/0961203320938456 ◽

2020 ◽

Vol 29 (10) ◽

pp. 1216-1226

Author(s):

Beatriz Frade-Sosa ◽

Javier Narváez ◽

Tarek Carlos Salman-Monte ◽

Raul Castellanos-Moreira ◽

Vera Ortiz-Santamaria ◽

...

Keyword(s):

Rheumatoid Arthritis ◽

Systemic Lupus Erythematosus ◽

Autoimmune Diseases ◽

Lupus Erythematosus ◽

Disease Duration ◽

Renal Involvement ◽

Clinical Manifestations ◽

Classification Criteria ◽

Systemic Lupus ◽

Cross Sectional

Background The concomitant presence of two autoimmune diseases – systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) – in the same patient is known as rhupus. We evaluated a group of patients with rhupus to clarify further their clinical, serological and immunogenic features in a multi-centre cohort. In addition, the study aimed to explore the utility of the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) SLE classification criteria in our group of patients with rhupus. Methods This was a cross-sectional study. We included rhupus patients from 11 different rheumatology departments, and compared them to SLE and RA patients at a ratio of 2:1. All information was recorded following a pre-established protocol. Results A total of 200 patients were included: 40 rhupus patients and 80 each of SLE and RA patients as controls. Disease duration was similar among SLE and rhupus groups (around 13 years), but the RA group had a significantly lower disease duration. Main clinical manifestations were articular (94.2%), cutaneous (77.5%) and haematological (72.5%). Rhupus patients had articular manifestations similar to those expected in RA. Only 10% of rhupus patients had renal involvement compared with 25% of those with SLE ( p < 0.05), while interstitial lung disease was more common in patients affected by RA. The 2019 EULAR/ACR SLE criteria were met in 92.5% of the rhupus patients and in 96.3% of the SLE cohort ( p > 0.05). Excluding the joint domain, there were no differences between the numbers of patients who met the classification criteria. Conclusion Rhupus patients follow a particular clinical course, with full expression of both SLE and RA in terms of organ involvement, except for a lower prevalence of kidney affection. The new 2019 EULAR/ACR SLE criteria are not useful for differentiating SLE and rhupus patients. A new way of classifying autoimmune diseases is needed to identify overlapping clusters.

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An uncommon cause of diffuse alveolar hemorrhage in a young male

Monaldi Archives for Chest Disease ◽

10.4081/monaldi.2019.1067 ◽

2019 ◽

Vol 89 (2) ◽

Author(s):

Anshul Mittal ◽

Jagdish Chander Suri ◽

Shibdas Chakrabarti ◽

Pranav Ish

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Nephritis ◽

Lupus Erythematosus ◽

Renal Involvement ◽

Young Male ◽

Diffuse Alveolar Hemorrhage ◽

Alveolar Hemorrhage ◽

Systemic Lupus ◽

Threatening Condition ◽

Life Threatening

It is uncommon for Systemic lupus erythematosus (SLE) to present with diffuse alveolar hemorrhage (DAH) as the initial presentation. To diagnose this in a young male with no renal involvement is further uncommon. We report a case of a 16-year-old boy, who presented with hemoptysis and was eventually diagnosed as DAH with underlying SLE. Treatment with steroids and immunosuppressant helped in rapid recovery from this potentially life-threatening condition. This case highlights the need of defining diagnostic criteria for SLE in patients presenting as DAH and formulating guidelines for treatment of the same, especially in absence of co-existing lupus nephritis.

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Pattern and Frequency of Anti-nuclear Antibody Positivity in Paediatric Rheumatic Diseases

Bangladesh Journal of Child Health ◽

10.3329/bjch.v43i1.41212 ◽

2019 ◽

Vol 43 (1) ◽

pp. 21-26

Author(s):

Mohammad Imnul Islam ◽

Kamrul Laila ◽

Shahana A Rahman

Keyword(s):

Rheumatic Diseases ◽

Lupus Erythematosus ◽

Staining Pattern ◽

Renal Involvement ◽

Clinical Manifestations ◽

Speckled Pattern ◽

Nuclear Antigens ◽

Antibody Positivity ◽

Homogeneous Pattern ◽

Paediatric Rheumatic Diseases

Background: Anti-nuclear antibodies (ANAs) are specific antibodies directed against a variety of nuclear antigens detected in the serum of patients with many rheumatic and non-rheumatic diseases.These antibodies are not only involved in the pathogenesis, but also constitute the basis for diagnosis and treatment of paediatric rheumatic diseases. The objective of the study was to identify the patterns and frequency of ANA positivity in Paediatric Rheumatic Diseases. Methodology: It was a retrospective study. Fourteen hundred and sixty eight records of paediaric rheumatology patients were analyzed. Statistical analysis were done to observe the frequency and association of different patterns of ANA in Juvenile idiopathic arthritis and systemic lupus erythematosus patients. Results: Among the 1468 patients of PRDs, frequency of JIA cases was the highest (65 %) followed by SLE, Scleroderma, juvenile dermatomyositis, and others. Among the 261 PRD patients ANA positivity was 65%. ANA positivity was 100%, 92%, 40% and 31.5% in Mixed connective tissue disease, SLE, JDM and Scleroderma patients respectively. Homogenous staining pattern was found in 59% and speckled pattern in 22.9%. There was significant association between ANA positivity and uveitis in oligoarticular JIA patients. Significant association was also found between homogeneous patterns of ANA and renal involvement in SLE patients. Conclusion: ANA positivity was highest in MCTD cases followed by SLE cases. Majority of SLE cases had homogeneous pattern of ANA.Staining patterns of ANA had significant association with the clinical manifestations in SLE and JIA cases. Bangladesh J Child Health 2019; VOL 43 (1) :21-26

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Cell-free DNA profiling in patients with lupus nephritis

Lupus ◽

10.1177/0961203320957717 ◽

2020 ◽

Vol 29 (13) ◽

pp. 1759-1772

Author(s):

Anna Truszewska ◽

Agnieszka Wirkowska ◽

Kamila Gala ◽

Piotr Truszewski ◽

Łucja Krzemień-Ojak ◽

...

Keyword(s):

Lupus Nephritis ◽

Lupus Erythematosus ◽

Copy Number ◽

Severe Disease ◽

Renal Involvement ◽

Mtdna Copy Number ◽

Cell Free Dna ◽

Free Dna ◽

Mitochondrial Fractions ◽

Kidney Involvement

Background Increased level of cell-free DNA (cf-DNA) is associated with systemic lupus erythematosus (SLE) and might be related to disease activity. The aim of this study was to evaluate whether cfDNA integrity, size distribution and concentration of different cfDNA fractions is associated with lupus activity and kidney involvement. Methods Blood samples were collected from 43 SLE patients and 50 healthy controls. Nuclear and mitochondrial fractions of cfDNA and intracellular DNA were quantified by real-time qPCR. Sizing and quantification of total cfDNA level was performed on Bioanalyzer. Results We determined four parameters that characterized cfDNA profile: fragmentation index, ratio of intra- to extracellular mtDNA copy number, cfDNA concentration, and presence of 54–149 bp and 209–297 bp fragments. Patients with healthy-like cfDNA profile had higher eGFR ( P = 0.009) and more often no indications for kidney biopsy or less advanced lupus nephritis (LN) ( P = 0.037). In contrary, SLE patients with distinct cfDNA profile (characterized by increased cfDNA concentration and fragmentation, higher discrepancy between intra- to extracellular mtDNA copy number, and the presence of 54–149 bp and 209–297 bp fragments) had lower eGFR ( P = 0.005) and more often advanced LN or history of renal transplantation ( P = 0.001). Conclusions We showed that cfDNA profiling may help to distinguish SLE patients with renal involvement and severe disease course from patients with more favorable outcomes. We suggest cfDNA profile a promising SLE biomarker.

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