Toxic epidermal necrolysis after therapeutic plasma exchange in pediatric lupus patients and associated risk factors analysis

Lupus ◽  
2021 ◽  
pp. 096120332098112
Author(s):  
Jing Lu ◽  
Liqun Dong ◽  
Lijuan Zhang ◽  
Hui Zhang ◽  
Lin Wang ◽  
...  
Keyword(s):  
Risk Factors ◽  
Systemic Lupus Erythematosus ◽  
Toxic Epidermal Necrolysis ◽  
Plasma Exchange ◽  
Skin Rash ◽  
Lupus Erythematosus ◽  
Effective Means ◽  
Clinical Manifestations ◽  
Therapeutic Plasma Exchange ◽  
Systemic Lupus

Background Therapeutic plasma exchange (TPE) is an effective means of treating systemic lupus erythematosus in children and is safe for most pediatric patients with systemic lupus erythematosus, but severe complications such as toxic epidermal necrolysis (TEN) may occur, which is a life-threatening condition. Methods In this study, three systemic lupus erythematosus (SLE) children developed toxic epidermal necrolysis after TPE. We analyzed their medical history, clinical manifestations, SLEDAI scores, and immunological characteristics, compared to 117 cases of SLE patients without TEN after TPE, trying to find the possible risk factors. Results The three children with TEN after plasma exchange appeared to have a higher proportion of male (male: female = 2:1), fever (100% Vs 32.5%), erythema on the cheek (100% Vs 54.7%), itching rash (100% Vs 54.7%), ruptured rash (100% Vs 54.7%), oral ulcer (100% Vs 54.7%) and higher LDH level (1826.0 ± 1113.1 Vs 721.1 ± 799.5 U/L), but lower white blood cell count (5.5 ± 3.3 Vs 7.2 ± 4.2 × 109/L), neutrophil count (4.7 ± 3.7 Vs 5.2 ± 3.6 × 109/L), lymphocyte count (0.6 ± 0.5 Vs 1.5 ± 0.8 × 109/L), platelet count (133.7 ± 58.1 Vs 178.5 ± 103.1 × 109/L) and C-reactive protein (all normal Vs 47.9% elevated). Autoantibody spectrum revealed that positive anti-SSA seemed more common (100% Vs 42.7%) in the three children. Relative risk analysis revealed that male (OR 21.4, 95%CI 1.78–257.186), ruptured skin rash (OR 56.5, 95%CI 4.199–760.196) and rash with itching (OR 24, 95%CI 1.98–290.896) are the risk factors of SLE patients developing TEN after plasma exchange. Conclusions We should pay particular attention to TEN after plasma exchange in SLE patients (3/120, 2.5%). This condition may be related to male, ruptured skin rash and rash with itching. For SLE patients with risk factors. We should arrange plasmapheresis more carefully.

Predictors of Incident Seizure in Systemic Lupus Erythematosus

2016 ◽  
Vol 43 (3) ◽  
pp. 565-575 ◽  
Author(s):  
XiangYang Huang ◽  
Laurence S. Magder ◽  
Michelle Petri
Keyword(s):  
Risk Factors ◽  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Renal Involvement ◽  
Multivariable Analysis ◽  
Clinical Manifestations ◽  
Systemic Lupus ◽  
Malar Rash ◽  
History Of

Objective.The risk factors for incident seizures in systemic lupus erythematosus (SLE) were prospectively determined in a cohort study.Methods.A total of 2203 patients with SLE followed longitudinally in the Hopkins Lupus Cohort were analyzed. Demographic variables, clinical manifestations, laboratory tests, and SLE disease activity were recorded at each quarterly visit. Adjusted estimates of association of risk factors for onset of seizure were derived using pooled logistic regression. We examined incident seizures in 3 ways: at the time of diagnosis, more than 45 days after the diagnosis of SLE, and after cohort entry.Results.Of 2203 patients with no history of seizure prior to SLE diagnosis, 157 (7.13%) had the first seizure occurrence at the time of (37 patients, 1.68%) or after diagnosis (120 patients, 5.45%) of SLE. The risk of seizure occurring around the time of SLE diagnosis was higher in patients with a history of malar rash (p = 0.002), proteinuria (p = 0.004), and psychosis (p < 0.001). Multivariable analysis of the first seizure occurring after the diagnosis of SLE showed that history of low C3 (p = 0.0078), psychosis (p < 0.0001), cranial or peripheral neuropathy (p = 0.0043), anti-Sm antibody (p = 0.0551), renal involvement (p = 0.0177), and current corticosteroid dose (p < 0.0001) were independently associated with a higher incidence of seizure. Disease activity was not predictive after adjusting for corticosteroids.Conclusion.Risk of seizure after diagnosis of SLE is increased in those patients with prior psychosis, neuropathy, proteinuria, anti-Sm, low C3, and use of corticosteroids.

Synergistic effect of cumulative corticosteroid dose and immunosuppressants on avascular necrosis in patients with systemic lupus erythematosus

Lupus ◽  
2018 ◽  
Vol 27 (10) ◽  
pp. 1644-1651 ◽  
Author(s):  
H H Kwon ◽  
S Y Bang ◽  
S Won ◽  
Y Park ◽  
J H Yi ◽  
...  
Keyword(s):  
Risk Factors ◽  
Systemic Lupus Erythematosus ◽  
Avascular Necrosis ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Individual Risk ◽  
High Dose ◽  
Systemic Lupus ◽  
Corticosteroid Dose ◽  
Increased Risk

Objectives Avascular necrosis (AVN) is one of the most common causes of organ damage in patients with systemic lupus erythematosus (SLE) and often causes serious physical disability. The aims of this study were to investigate clinical risk factors associated with symptomatic AVN and to analyze their synergistic effects in a large SLE cohort in Korea. Methods Patients with SLE were enrolled and followed from 1998 to 2014 in the Hanyang BAE Lupus cohort, and damage was measured annually according to the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI). AVN was confirmed by imaging study if patients had symptoms. To determine risk factors for AVN, clinical, laboratory and therapeutic variables were analyzed by logistic regression. Relative excess risk due to interaction (RERI), attributable proportion (AP), and synergy index (S) were calculated to measure interactions between significant variables. Results Among 1219 SLE patients, symptomatic AVN was the most common type of musculoskeletal damage (10.8%, n = 132). SLE patients with AVN showed an earlier onset age, demonstrated AVN more commonly in conjunction with certain other clinical manifestations such as renal and neuropsychiatric disorders, and received significantly higher total cumulative corticosteroid dose and immunosuppressive agents than did patients without AVN. However, in multivariable analysis, only two variables including use of a cumulative corticosteroid dose greater than 20 g (odds ratio (OR) 3.62, p = 0.015) and use of immunosuppressants including cyclophosphamide or mycophenolate mofetil (OR 4.51, p < 0.001) remained as significant risk factors for AVN. Patients with cumulative corticosteroid dose > 20 g and immunosuppressant use had a 15.44-fold increased risk for AVN, compared with patients without these risk factors ( p < 0.001). RERI, AP and S, which define the strength of interactions between two risk factors, were 9.01 (95% confidence interval (CI) 1.30–16.73), 0.58 (95% CI 0.36–0.81) and 2.66 (95% CI 1.42–4.99), respectively, supporting the presence of synergistic interactions in the development of symptomatic AVN in our Korean lupus cohort. Conclusions An individual risk assessment for AVN development should be made prior to and during treatment for SLE, especially in patients with high-dose corticosteroid and immunosuppressant use regardless of clinical manifestations and disease activity.

scholarly journals Therapeutic Plasma Exchange as Management of Complicated Systemic Lupus Erythematosus and Other Autoimmune Diseases

2019 ◽  
Vol 2019 ◽  
pp. 1-10 ◽  
Author(s):  
David Aguirre-Valencia ◽  
Juan Naranjo-Escobar ◽  
Iván Posso-Osorio ◽  
María Carmenza Macía-Mejía ◽  
Ivana Nieto-Aristizábal ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Autoimmune Diseases ◽  
Plasma Exchange ◽  
Lupus Erythematosus ◽  
Clinical Characteristics ◽  
Diffuse Alveolar Hemorrhage ◽  
Therapeutic Plasma Exchange ◽  
Systemic Lupus ◽  
Systemic Autoimmune Diseases ◽  
Main Complication

Introduction. Autoimmune diseases include a diverse and complex group of pathologies with a broad clinical spectrum due to the production of autoantibodies, which generates multisystemic compromise. Therapeutic plasma exchange (TPE) is a good additive treatment for immunosuppression due to its action over the autoantibodies. Objectives. To describe the main clinical characteristics and outcomes of patients with systemic lupus erythematosus and other systemic autoimmune diseases managed with TPE. Methodology. This descriptive retrospective study enrolled patients with systemic autoimmune diseases who received TPE. Results. In total, 66 patients with a median age of 33.5 years (24-53 years) were included; the majority were females [n=51 (77.27%)]. Forty (60.61%) patients were diagnosed with systemic lupus erythematosus. In these cases, the main indication for TPE was diffuse alveolar hemorrhage (DAH; n=20, 30.3%) and neurolupus (n=9, 13.6%). No TPE-related deaths occurred, and the main complication was hemorrhage, without significant differences among the four types of TPE solutions used. The overall outcome was improvement in 41 (62.12%) patients. Conclusion. TPE is safe and effective in patients with severe manifestations of autoimmune diseases.

scholarly journals AB0132 ALTERATIONS IN PERIPHERAL T-CELLS AND B-CELLS SUBSETS IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS AND SJÖGREN’S SYNDROME UNDERGOING THERAPEUTIC PLASMA EXCHANGE OR IMMUNOADSORPTION

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1367.2-1367
Author(s):  
Y. Jiang ◽  
Q. Wei ◽  
Q. Lv ◽  
X. Zhang ◽  
W. Zhu ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
T Cells ◽  
T Cell ◽  
B Cells ◽  
Plasma Exchange ◽  
Lupus Erythematosus ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Therapeutic Plasma Exchange ◽  
Systemic Lupus

Background:Systemic lupus erythematosus (SLE) and Sjögren’s syndrome (SS) are systemic autoimmune diseases characterized by a broad spectrum of clinical manifestations and disease course. Alternative therapies such as therapeutic plasma exchange (TPE), immunoadsorption are recommended to the patients who lack a good response to standard therapy [1].Objectives:Our observational study was to explored whether abnormalities in T-cells, B-cells and their subtypes were present in the patients who had TPE or immunoadsorption in patients with SLE and SS compared with healthy controls (HC).Methods:Demographic, clinical variables and autoantibodies were recorded. Flow cytometry was used to establish the frequencies of lineage subsets. Monoclonal antibodies against 21 surface markers such as CD3, CD4, CD8, were used to distinguish and evaluate T-cells’ and B-cells’ subpopulation. SLE acvity was measured using systemic lupus erythematosus disease activity index (SLEDAI). Comparisons between subgroups were undertaken using paired T-test, Mann-Whitney U test and ANOVA.Figure 1.Altered expression of CD4+ T-cell subsets in the patients with SLE and SS after treated with plasma exchange or immunoadsorptionResults:6 SS patients and 1 SLE patient underwent immune adsorption, while the other 5 SLE patients had plasma exchange all for three times. There was no significant difference among SLE, SS and HC in the proportion of T-cells and B-cells. The proportion of CD3-CD19+CD27+IgD+ B-cells were reduced in SLE, while CD3+CD4+CD25+CD127- T-cells were elevated in SS. The proportion of CD3+CD4+CD45RA+CCR7+T-cells were increased (p= 0.045), while CD3+CD4+CD25+CD127- T-cells were declined (p= 0.027) and CD3+CD4+CXCR5+PD-1+ T-cells went down after the therapies (p≤ 0.030). The proportion of CD3-CD19+IgD-IgM-CD27+CD38+ B-cells was also reduced after TPE or immunoadsorption (p= 0.032) with ANA titers and IgG decreasing dramatically. SLEDAI scores were reduced after the therapy in SLE patients.Conclusion:The T-cell and B-cell’s profiles were proved to have alteration after TPE or immunoadsorption which shed light on the complicated mechanisms of these relatively novel therapy in SLE and SS.References:[1]Bertsias GK, Tektonidou M, Amoura Z, et al. Joint European League Against Rheumatism and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of adult and paediatric lupus nephritis. ANN RHEUM DIS 2012;71:1771-1782.Acknowledgments:None.Disclosure of Interests:None declared

Therapeutic plasma exchange and Cyclosporin A in severe systemic lupus erythematosus

1989 ◽  
Vol 10 (2) ◽  
pp. 147-154 ◽  
Author(s):  
Rolf Bambauer ◽  
Bernd Reinelt ◽  
Hans Pees ◽  
Gerhard Schwarze ◽  
Hans Erich Keller ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Cyclosporin A ◽  
Plasma Exchange ◽  
Lupus Erythematosus ◽  
Therapeutic Plasma Exchange ◽  
Systemic Lupus ◽  
Severe Systemic Lupus Erythematosus
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