Pediatric lupus nephritis

Abstract Involvement of the kidneys by lupus nephritis (LN) is one of the most severe clinical manifestations seen in individuals with systemic lupus erythematosus (SLE). LN is more frequent and severe in pediatric patients and has been associated with higher morbidity and mortality rates. This narrative review aimed to describe the general aspects of LN and its particularities when affecting children and adolescents, while focusing on the disease's etiopathogenesis, clinical manifestations, renal tissue alterations, and treatment options.

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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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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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Predictors of mortality in children with lupus nephritis

Paediatrica Indonesiana ◽

10.14238/pi54.6.2014.338-43 ◽

2014 ◽

Vol 54 (6) ◽

pp. 338

Author(s):

Lukman Oktadianto ◽

Risky Vitria Prasetyo ◽

Ninik Asmaningsih Soemyarso ◽

Mohammad Sjaifullah Noer

Keyword(s):

Lupus Nephritis ◽

Lupus Erythematosus ◽

Renal Involvement ◽

Hypertensive Crisis ◽

Clinical Signs ◽

Clinical Manifestations ◽

Predictors Of Mortality ◽

Cox Proportional Hazard ◽

Kaplan Meier ◽

Mean Time

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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Alveolar Echinococcosis in Children

Case Reports in Pediatrics ◽

10.1155/2020/5101234 ◽

2020 ◽

Vol 2020 ◽

pp. 1-7

Author(s):

Emilija Jonaitytė ◽

Martynas Judickas ◽

Eglė Tamulevičienė ◽

Milda Šeškutė

Keyword(s):

Surgical Treatment ◽

Current Literature ◽

Echinococcus Multilocularis ◽

Cystic Echinococcosis ◽

Alveolar Echinococcosis ◽

Pediatric Patients ◽

Treatment Options ◽

Clinical Manifestations ◽

Diagnostic Methods

Alveolar echinococcosis (AE) is an infectious zoonotic disease that is caused by Echinococcus multilocularis. The disease is generally identified accidentally because of the long asymptomatic period, has a malignant behaviour, and mainly occurs in the liver. Usually it is diagnosed in adults and is very rare in pediatric patients. We report two cases of AE and 1 differential case between AE and cystic echinococcosis (CE) in children: two of them had lesions in the liver and one had rare extrahepatic presentation of a cyst in the spleen. All our patients received chemotherapy with albendazole because surgical treatment was not recommended. The children were followed-up from 10 to 30 months and no significant improvement was seen. In this report we discuss the difficulties we faced in the treatment and follow-up of these patients. We also review the main clinical manifestations, general diagnostic methods, and treatment options of AE according to the current literature.

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A Study on Clinical and Pathologic Features in Lupus Nephritis with Mainly IgA Deposits and a Literature Review

Clinical and Developmental Immunology ◽

10.1155/2013/289316 ◽

2013 ◽

Vol 2013 ◽

pp. 1-5 ◽

Cited By ~ 1

Author(s):

Liu Hongyan ◽

Zheng Yi ◽

Dong Bao ◽

Lu Yuewu ◽

Meng Juan

Keyword(s):

Lupus Nephritis ◽

Lupus Erythematosus ◽

Laboratory Tests ◽

Clinical Manifestations ◽

Classification Criteria ◽

Systemic Lupus ◽

Serum Iga ◽

Pathologic Features ◽

Previous Infection ◽

Positive Rate

Objective. To study the clinical and pathologic features of systemic lupus erythematosus (SLE) that has atypical lupus nephritis (LN) with mainly IgA deposits.Methods. We searched the SLE patients who had nephritis with mainly IgA deposits in our hospital and selected the information including clinical manifestations, laboratory tests, treatments, and prognosis.Results. From January 2009 to June 2012, 5 patients were definitely diagnosed as SLE according to both 1982 and 2009 ACR classification criteria. But renal biopsy showed that all cases had mainly IgA deposits and were free of IgG, C1q, and fibrinogen-related antigen deposits under immunofluorescent microscopy, which did not match with typical LN. There were 2 males and 3 females, aging from 31 to 64 years and with an average of years. The 5 cases had multiple-system involvements, mainly the renal system. Compared to primary IgAN, the atypical LN showed some differences: older than primary IgAN, more women than men, no previous infection history, lower incidence of serum IgA elevation, and ACL positive rate as high as 100%.Conclusion. Nephritis with mainly IgAN deposits, as an atypical LN, may be a special subtype of SLE.

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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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Rubrics for mortality: a real-world observational long-term lupus nephritis cohort

Lupus ◽

10.1177/0961203320937472 ◽

2020 ◽

Vol 29 (12) ◽

pp. 1616-1622

Author(s):

Maria Francisca Moraes-Fontes ◽

Ana Carolina Ferreira ◽

Nuno Riso ◽

Helena Viana ◽

Fernanda Carvalho

Keyword(s):

Lupus Nephritis ◽

Renal Biopsy ◽

Lupus Erythematosus ◽

Demographic Data ◽

Damage Index ◽

Clinical Manifestations ◽

Class Iii ◽

Systemic Lupus ◽

Number Of Patients

In this study, we aimed to evaluate long-term patient survival according to demographic data, clinical manifestations of systemic lupus erythematosus (SLE) and previous and current treatments, collected retrospectively. Patient selection required a minimum of four American College of Rheumatology revised criteria for SLE, biopsy-proven lupus nephritis (LN) available for reclassification according to the modified National Institutes of Health proposal for activity and chronicity indices and a minimum follow-up of at least three years since the last renal biopsy. Selection criteria were fulfilled in 25 patients followed for a median of 21 years. Based on the last renal biopsy, an equal number of patients were thus classified as class I/II and IV ( n=8) and class III and V ( n = 4). The mortality rate for LN was 14%. Having ever been diagnosed with glomerulonephritis (GN) type III or type IV but not class IV alone ( p = 0.046), a higher histological chronicity index at the last renal biopsy ( p = 0.022), not attaining renal remission one year after induction therapy ( p = 0.004), end-stage renal disease on dialysis ( p = 0.033) and the extra-renal Systemic Lupus International Collaborating Clinics Damage Index score ( p = 0.017) were all significantly associated with mortality. Our results may provide important clues for strict observation protocols in particular categories of LN patients with long-standing disease.

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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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Lupus Nephritis

Kidney Protection ◽

10.1093/med/9780190611620.003.0031 ◽

2019 ◽

pp. 309-318

Author(s):

Aisha Shaikh ◽

Kirk N. Campbell

Keyword(s):

Lupus Nephritis ◽

Lupus Erythematosus ◽

Treatment Options ◽

Disease Entity ◽

Disease Remission ◽

Renal Histology ◽

Single Disease Entity ◽

Histological Remission ◽

Common Manifestation ◽

Histological Form

Lupus nephritis (LN) is common manifestation of systemic lupus erythematosus and is associated with significant morbidity and mortality. LN is a not a single-disease entity; on the contrary, it encompasses a wide array of renal histological patterns. The treatment options and outcomes vary with the type of renal histology. Proliferative LN remains the most aggressive histological form of LN and requires aggressive treatment. The goal of therapy for LN is to achieve clinical and histological remission and avoid progression to chronic kidney disease. Remission of LN not only improves renal outcomes but also results in significant improvement in overall patient mortality. Though much progress has been made in this arena, there is still a need for therapeutic agents that are less toxic and more effective than the currently available therapies.

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Secondary angle-closure glaucoma as an ocular presentation of systemic lupus erythematosus: a case report

Journal of International Medical Research ◽

10.1177/0300060520959492 ◽

2020 ◽

Vol 48 (9) ◽

pp. 030006052095949

Author(s):

Xincen Hou ◽

Wenping Pan ◽

Anli Wang ◽

Tao Yu ◽

Aiping Song

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Nephritis ◽

Lupus Erythematosus ◽

Clinical Manifestations ◽

Immunosuppressive Drugs ◽

Acute Angle ◽

Angle Closure Glaucoma ◽

Angle Closure ◽

High Dose ◽

Systemic Lupus

Systemic lupus erythematosus (SLE) is a chronic idiopathic autoimmune disease. SLE can involve almost any part of the eyes. However, bilateral angle-closure glaucoma due to lupus choroidopathy that is accompanied by polyserositis and nephropathy is rare. We report a 21-year-old woman whose clinical manifestations were diagnosed as bilateral angle-closure glaucoma caused by ciliochoroidal effusion. Subsequently, SLE and lupus nephritis were diagnosed on the basis of malar rash, photosensitivity, proteinuria, positive anti-Smith and anti-DNA antibodies, and a renal histopathological biopsy. After 1 month of treatment with steroids and immunosuppressive drugs, the patient’s intraocular pressure returned to normal, visual acuity improved, and lupus nephritis was effectively controlled. Bilateral secondary acute angle closure caused by SLE choroidal disease can be an ocular manifestation of SLE, and is usually accompanied by polyserositis and nephropathy. High-dose steroids and immunosuppressive therapy should be immediately and actively provided for this condition.

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