Measures in assessment of pediatric systemic lupus erythematosus: an experience of retrospective observational study

2017 ◽  
Vol 8 (5) ◽  
pp. 35-43
Author(s):  
Ekaterina M. Kuchinskaya ◽  
Vyacheslav G. Chasnyk ◽  
Mikhail M. Kostik
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Mycophenolate Mofetil ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Favorable Outcome ◽  
Induction Treatment ◽  
Systemic Lupus ◽  
Intravenous Methylprednisolone ◽  
Pediatric Systemic Lupus Erythematosus ◽  
Initial Treatment Period

Systemic lupus erythematosus in children (juvenile-onset SLE, jSLE) is a multisystemic disease with an unpredictable course and a more severe phenotype compared to adults. The patterns of jSLE are extremely heterogeneous, so an enrollment to controlled studies may be rather complicated. Due to this problem and some additional ones, there are no standards for treatment of jSLE yet. The attending physician is fully responsible for the induction and maintenance therapeutical options including durability and aggressiveness. Objectives: finding of jSLE individual course’s features prognostically connected with the disease outcome. Methods: 45 children admitted to the SPbGPMU hospital with the systemic lupus erythematosus diagnosed at the age of 4-17 years were enrolled in this retrospective study. Primary SLE manifestations, the activity of disease according to SELENA-SLEDAI and ECLAM scales during initial treatment period and flares after it, the fact of remission achievement in 6 months were evaluated in each patient. Results: a few organ involvements were considered to be connected with outcome’s characteristics, for example lupus nephritis and early disease oncet are unfavorable predictive factors. The positive connection of favorable outcome with cyclophosphamide, intravenous methylprednisolone and mycophenolate mofetil was found; the negative connection between initial disease activity and flares after induction treatment was also noticed. Conclusion: the patient with initially high disease activity treated aggressively with high cumulative doses of cyclophosphamide, intravenous methylprednisolone and mycophenolate mofetil has more chances of the favorable outcome (the achievement of remission without further flares).

scholarly journals POS0685 MYCOPHENOLATE MOFETIL IN SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS: FIVE-YEARS DRUG SURVIVAL IN RENAL AND NON-RENAL INVOLVEMENT

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 588.2-588
Author(s):  
G. Olivieri ◽  
F. Ceccarelli ◽  
F. Natalucci ◽  
F. R. Spinelli ◽  
C. Alessandri ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Mycophenolate Mofetil ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Treatment Duration ◽  
Retention Rate ◽  
Renal Involvement ◽  
Joint Involvement ◽  
Systemic Lupus ◽  
Median Treatment

Background:The updated EULAR recommendations for the management of systemic lupus erythematosus (SLE) underline the use of Mycophenolate Mofetil (MMF) in the treatment of different disease related manifestations (1). Several randomized controlled trials have demonstrated the efficacy of MMF in lupus nephritis (LN) patients but only case series and open-labelled trials have analyzed the use of this drug in other than LN features. Moreover, no data are available about the MMF retention rate in a real-life setting.Objectives:The present study aims at evaluating the 5-years drug retention rate (DRR) of MMF in a large monocentric SLE cohort. Secondly, we investigated the influence of MMF in disease activity changes and chronic damage progression.Methods:We performed a longitudinal study including all the SLE patients (ACR 1997 criteria) starting MMF treatment in our Lupus Clinic. Data about indications, mean dosage, duration of treatment and reasons for drug withdrawal were registered. The DRR was estimated using the Kaplan–Meier method. Disease activity and chronic damage were assessed by SLE Disease Activity Index 2000 (SLEDAI-2K) and SLICC Damage Index (SDI), respectively.Results:The present analysis included 162 SLE patients (M/F 22/140, median age at the disease diagnosis 25.5 years, IQR 13). At the beginning of MMF treatment, we registered a median age of 34 months (IQR 21) and a median disease duration of 72 months (IQR 123). The most frequent indications for prescribing MMF were LN (101 patients, 62.3%) and musculoskeletal manifestations (39, 24.1%), followed by neuropsychiatric involvement (10, 6.2%), and others disease related manifestations (12, 7.4%; in particular skin involvement, hematological features, myositis, vasculitis). MMF was administered at a mean daily dosage of 2.1±0.6 grams; no differences in dosage were found between the different indications (p=ns).At the longitudinal analysis, we registered a median treatment duration of 30 months (IQR 55). Figure 1 reported data about DRR: in particular, at 60 months follow-up we observed a DRR of 61.1% for LN patients, which was similar to that registered for patients without renal involvement (NLN) (60.5%; p=ns). Interestingly, the DRR at 60 months was higher in the subgroup of patients treated for joint involvement (75.4%), even without reaching a statistically significant difference. During the observation period, 92 patients (59.2%) discontinued MMF (median treatment duration at discontinuation 25 months, IQR 35). Interestingly, the main cause of withdrawal was the achievement of persistent remission, observed in 20 patients (21.7%), followed by loss of efficacy (19 patients, 20.5%), drug intolerance and pregnancy planning (17 patients for both reasons, 18,4%). Furthermore, our analysis confirmed MMF efficacy, as demonstrated by the significant reduction in SLEDAI-2k values after 4, 12 and 24 months of treatment (p< 0.0001 for all the time-points in comparison with baseline). In addition, MMF resulted able to control chronic damage progression, as demonstrated by the lack of significant increase in SDI values (baseline: 0.6, IQR 1; last observation: 0.93, IQR 1; p=ns).Conclusion:The evaluation of a large SLE cohort demonstrated a good retention rate for MMF. In particular, our results demonstrated that MMF is also a safe and effective drug for SLE manifestation other than LN, in particular for joint involvement. Moreover, it is able to control disease activity and to prevent the progression of chronic damage.References:[1]Fanouriakis A et al. Ann Rheum Dis. 2019 Jun;78(6):736-745.Disclosure of Interests:None declared

scholarly journals MAGE-B2 Autoantibody: A New Biomarker for Pediatric Systemic Lupus Erythematosus

2008 ◽  
Vol 35 (12) ◽  
pp. 2430-2438 ◽  
Author(s):  
ALICE D.C. HOFTMAN ◽  
LEI-QIAN TAI ◽  
SHEILA TZE ◽  
DAVID SELIGSON ◽  
RICHARD A. GATTI ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Activity Index ◽  
Disease Activity Index ◽  
Systemic Lupus ◽  
Western Blots ◽  
Pediatric Systemic Lupus Erythematosus ◽  
Embryonic Antigen ◽  
Pediatric Sle

ObjectiveMelanoma-associated antigen gene B2 (MAGE-B2) encodes an embryonic antigen normally silenced after birth except in testis and placenta. We identified the MAGE-B2 gene and autoantibodies in pediatric patients with systemic lupus erythematosus (SLE) glomerulonephritis. We investigated the prevalence of MAGE-B2 autoantibodies in association with active SLE, to determine a pathogenetic role of MAGE-B2 protein through its distribution in cells and tissues.MethodsAcross-sectional study analyzed the frequency of MAGE-B2 autoantibodies in 40 patients with pediatric SLE, 23 adult controls, and 16 patients with pediatric juvenile rheumatoid arthritis (JRA) using Western blots containing recombinant MAGE-B2. SLE Disease Activity Index 2000 (SLEDAI-2K) and British Isles Lupus Assessment Group (BILAG) index measured SLE disease activity. Tissue distribution of MAGE-B2 protein was assessed by immunohistochemistry, immunofluorescence, and Western blots.ResultsSeventeen (43%) of 40 pediatric SLE patients had MAGE-B2 autoantibodies as compared to 0 of 16 JRA patients and 2 of 23 adult controls. SLE disease activity was significantly higher in MAGE-B2 autoantibody-positive versus autoantibody-negative patients (SLEDAI-2K, mean 10.9 vs 5.2, p = 0.013; BILAG, mean 15.3 vs 6.3, p = 0.023). Active nephritis was more prevalent (60% vs 24%) inMAGE-B2 autoantibody-positive than autoantibody-negative SLE patients. MAGE-B2 protein was visualized in SLE kidney proximal convoluted tubules and in tumor epithelial cells, but not in lymphoblastoid cells.ConclusionMAGE-B2 autoantibody appears to be a clinically relevant biomarker for pediatric SLE disease activity and nephritis.

scholarly journals SYMPTOMATIC COVID INFCETIONS IN ADULT AND PEDIATRIC SYSTEMIC LUPUS ERYTHEMATOSUS AND RELATION TO DISEASE ACTIVITY

2021 ◽  
Author(s):  
Simone Appenzeller ◽  
Kaik da Silva Gomes ◽  
Mariana Moraes da Silva Lucino ◽  
Ana Carolina Londe ◽  
Diego de Paula Ferreira Nunes ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Pediatric Systemic Lupus Erythematosus

scholarly journals SIGLEC1 (CD169) is a sensitive biomarker for the deterioration of the clinical course in childhood systemic lupus erythematosus

Lupus ◽  
2020 ◽  
Vol 29 (14) ◽  
pp. 1914-1925
Author(s):  
Sae Lim von Stuckrad ◽  
Jens Klotsche ◽  
Robert Biesen ◽  
Mareike Lieber ◽  
Julia Thumfart ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Clinical Course ◽  
Youden Index ◽  
Systemic Lupus ◽  
Pediatric Systemic Lupus Erythematosus ◽  
Elevated Expression ◽  
Membrane Bound

Background To analyse the validity of membrane-bound SIGLEC1 (CD169) as a sensitive biomarker for monitoring disease activity in pediatric systemic lupus erythematosus (SLE). Methods 27 children and adolescents with SLE were followed for a mean of 13.5 months. During consecutive routine visits SLEDAI-2k, C3, C4 and ds-DNA values were determined. Additionally, expression of SIGLEC1 on monocytes was determined by flow cytometry. The amount of PE-labelled CD169 mAb bound per monocyte was analyzed using QuantiBRITE™ PE tubes. Associations between biomarkers and the clinical course were investigated by regression analysis. Results In general, SIGLEC1 expression is high on SLE-derived monocytes (mean 6 359 (SD 6 056) molecules/monocyte, cut-off 2 500 molecules/monocyte), all patients with newly diagnosed SLE exhibit elevated expression (mean 13366 (SD 7 750) molecules/monocyte). Changes (Δ) in SIGLEC1 levels during the clinical course is the only biomarker that significantly correlates with the change in SLEDAI-2k (betaST = 0.28, p = 0.001). At follow-up visit, a clinically important worsening was experienced by 47.6% of patients with a Δ SIGLEC1 > 2 151 molecules/cell (OR 5.31) and 72.4% with a Δ SIGLEC1 > 756 molecules/cell (OR 8.90). Conversely, 36.4% of patients with a Δ SIGLEC1 < -2 818 molecules/cell (OR 4.16, percentiles as cut-off criteria) and 50.0% of patients with a Δ SIGLEC1 < -1 370 molecules/cell (OR 3.55, application of Youden index) showed clinical improvement. SIGLEC1 expression correlates inversely with the amount of therapeutically applied hydroxychloroquine (p < 0.001). Conclusions SIGLEC1 expression on monocytes is a sensitive biomarker for adjusting disease activity in childhood SLE and represents a promising and easily applicable tool for disease monitoring.

scholarly journals Novel Therapeutic Interventions in Systemic Lupus Erythematosus

2021 ◽  
Author(s):  
Panagiotis Athanassiou ◽  
Lambros Athanassiou ◽  
Ifigenia Kostoglou-Athanassiou
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Mycophenolate Mofetil ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Immunosuppressive Agents ◽  
Induction Treatment ◽  
Systemic Lupus ◽  
Beneficial Effects ◽  
Anti Cd20 ◽  
Steroid Sparing

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease. It is characterized by a variable clinical course ranging from mild to fatal disease. It can affect the kidneys. The aim of treatment in SLE is the prevention of flares and the prevention of accumulation of damage to the main organs affected as well as the prevention of drug side effects. The cornerstone of SLE treatment is hydroxychloroquine. Corticosteroids are used both as induction treatment in disease flares as well as in small doses as maintenance treatment. Immunosuppressants, such as azathioprine, methotrexate and mycophenolate mofetil are used as steroid sparing agents. Calcineurin inhibitors, namely tacrolimus and cyclosporin A may also be used as immunosuppressants and steroid sparing agents. Pulse methylprednisolone, along with mycophenolate mofetil and cyclophosphamide are used as induction treatment in lupus nephritis. Rituximab, an anti-CD20 biologic agent may be used in non-renal SLE. In patients insufficiently controlled with hydroxychloroquine, low dose prednisone and/or immunosuppressive agents, belimumab may be used with beneficial effects in non-renal disease and lupus nephritis.

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