scholarly journals P1792SUCCESSFUL TRANSPLANTATION OF STEM CELLS AND KIDNEY IN REFRACTORY LUPUS NEPHRITIS: REMOTE OUTCOMES

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Aleksandr Demin ◽  
Larisa Demina
Keyword(s):  
Stem Cells ◽  
Lupus Nephritis ◽  
Creatinine Clearance ◽  
Disease Activity ◽  
Immunosuppressive Therapy ◽  
High Dose ◽  
Sledai Score ◽  
Organ Systems ◽  
Nephritic Syndrome ◽  
Conventional Immunosuppression

Abstract Background and Aims Lupus nephritis is a common part of systemic lupus erythematosus (SLE), severe autoimmune disease that affects multiple organ systems and associated with pure prognosis. Patients with lupus nephritis who experience persisted disease activity despite conventional immunosuppression are at high risk of early death. Re-setting of the immune system and self-tolerance by high-dose immunosuppressive therapy with autologous stem cell transplantation (ASCT) is a new approach in the treatment of refractory SLE. Remote outcomes of this method and effectiveness relapse treatment are still unclear and were the aims of this study. Method We report a sick woman 39 years old, with refractory severe lupus nephritis. Standard therapy was ineffective, SLEDAI score remains 22 so patient was underwent high dose immunosuppressive therapy with ASCT and included in European Group for Blood and Marrow Transplantation European League against Rheumatism (EBMT/EULAR) registry on 53 pts who received ASCT for SLE between 1996 and 2005. Ethical approval was obtained for this study from Cambridge University Hospital Ethics Committee. Results The ASCT induced complete clinical and serological remission (SLEDAI score was 0) for 3 years. Than relapse with nephritic syndrome and anti-dsDNA positivity occurred and caused renal failure, creatinine clearance decreased to 21. Despite therapy including prednisolone, 45 mg daily, and mycophenolate mofetil, 2000 mg daily, disease activity persisted, creatinine clearance remained decreasing and 3 years later became 15 ml/min. During 1 year patient was on regular hemodialysis, then renal transplantation performed. Now, follow up is 15 years after ASCT and 8 years after kidney transplantation. Patient received a standard post-transplant immunosuppression with prednisolone, 5 mg daily, tacrolimus, 2.5 mg daily, and azathioprine, 100 mg daily, and her conditions remains stable, she has functioning renal allograft, SLEDAI score is 2 (anti-dsDNA positivity in low titer). Conclusion We present the successful remote outcomes of first case of immunoablation and double transplantation of autologous stem cells and allogeneic kidney in severe refractory to conventional immunosuppression SLE with lupus nephritis.

scholarly journals Urinary Neutrophil Gelatinase-Associated Lipocalin to Monitor Lupus Nephritis Disease Activity

2015 ◽  
Vol 10 ◽  
pp. BMI.S27625 ◽  
Author(s):  
Hani Susianti ◽  
Jullyanny W. Wijaya ◽  
Ati Rastini ◽  
Kusworini Handono ◽  
Atma Gunawan ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Transforming Growth Factor ◽  
Activity Index ◽  
Disease Activity Index ◽  
Transforming Growth Factor Β1 ◽  
Sledai Score ◽  
Neutrophil Gelatinase Associated Lipocalin ◽  
Neutrophil Gelatinase

Background This study was conducted to determine whether there is an association between urinary neutrophil gelatinase-associated lipocalin (uNGAL) and urinary transforming growth factor-β1 (uTGF-β1) with lupus nephritis (LN) disease activity. Methods Urine samples from 18 LN patients were collected every month for six months then examined for uNGAL, uTGF-β1, and renal domain Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score. Results The uNGAL levels were significantly different between active and inactive LN (P < 0.05). uTGF-β1 levels were not different between active and inactive LN (P > 0.05). There was a significant correlation between uNGAL levels and renal domain SLEDAI score (r= 0.417, P < 0.05). There was no correlation between uTGF-β1 levels and renal domain SLEDAI score (r = 0.031, P > 0.05). Conclusion uNGAL is better than uTGF-β1 for differentiation of active and inactive LN. uNGAL can be considered as a biomarker to monitor LN disease activity.

scholarly journals Cytokine Profile in Juvenile Systemic Lupus Erythematosus

2021 ◽  
pp. 72-78
Author(s):  
A. Elfar ◽  
Amal El-Bendary ◽  
M. A. Abdel-Hafez ◽  
N. Abo El Hana
Keyword(s):  
Lupus Nephritis ◽  
Disease Activity ◽  
Urinary Protein ◽  
Cytokine Profile ◽  
Control Group ◽  
Healthy Children ◽  
Healthy Controls ◽  
Sledai Score ◽  
Significant Difference ◽  
Active Patients

Background: Cytokines have an important role in immune system dysregulation in SLE because they act on the differentiation, maturation, and activation of several effector cells, culminating in inflammation and subsequent tissue damage.The aim of the work was to evaluate cytokine profile (IL2, IL10 and IL13) in children with SLE and their possible role in the pathogenesis of lupus nephritis. Methods: This is a cross sectional case-control study conducted on 60 children with SLE and 30 healthy children of matched age and sex served as a control group. The presence of lupus nephritis was confirmed by renal biopsy and histopathological examination. The SLE Disease Activity Index (SLEDAI) score for each patient was used to evaluate disease activity. Serum IL2, IL-10 & IL-13levels were measured using ELISA. Results: There was a significant increase in serum IL-10 levels in SLE patients compared to healthy controls and in patients with lupus nephritis compared to patients without lupus nephritis. Also, there were significant positive correlation between IL-10 and SLEDAI Score and between IL-10 and 24-hour urinary protein collection. There was no statistically significant difference in IL-2 levels in SLE patients compared to healthy controls. However, IL2 levels were significantly lower in active patients without lupus nephritis compared to active patients with lupus nephritis. There was no correlation between IL-2 and 24-hour urinary protein collection. The levels of IL13 were significantly higher in SLE patients compared to healthy controls and in patients with lupus nephritis compared to patients without lupus nephritis. There were significant positive correlations between Il 13 and SLEDAI Score and between IL-13and 24-hour urinary protein collection. Conclusions: Soluble IL10 and IL-13 could be used as a measure of disease activity. Further studies are needed to evaluate SLE pathophysiology including measurement of cytokine profile.

scholarly journals Immunosuppressive therapy in lupus nephritis: The Euro-Lupus Nephritis Trial, a randomized trial of low-dose versus high-dose intravenous cyclophosphamide

2002 ◽  
Vol 46 (8) ◽  
pp. 2121-2131 ◽  
Author(s):  
Frédéric A. Houssiau ◽  
Carlos Vasconcelos ◽  
David D'Cruz ◽  
Gian Domenico Sebastiani ◽  
Enrique de Ramon Garrido ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Randomized Trial ◽  
Immunosuppressive Therapy ◽  
Low Dose ◽  
High Dose ◽  
Intravenous Cyclophosphamide

scholarly journals SLE Plasma Profiling Identifies Unique Signatures of Lupus Nephritis and Discoid Lupus

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Michael A. Smith ◽  
Jill Henault ◽  
Jodi L. Karnell ◽  
Melissa L. Parker ◽  
Jeffrey M. Riggs ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Activity Index ◽  
Disease Activity Index ◽  
Complement C3 ◽  
Type I ◽  
Tissue Remodelling ◽  
Organ Systems ◽  
Organ Specific

Abstract Systemic lupus erythematosus (SLE) impacts multiple organ systems, although the causes of many individual SLE pathologies are poorly understood. This study was designed to elucidate organ-specific inflammation by identifying proteins that correlate with SLE organ involvement and to evaluate established biomarkers of disease activity across a diverse patient cohort. Plasma proteins and autoantibodies were measured across seven SLE manifestations. Comparative analyses between pathologies and correlation with the SLE Disease Activity Index (SLEDAI) were used to identify proteins associated with organ-specific and composite disease activity. Established biomarkers of composite disease activity, SLE-associated antibodies, type I interferon (IFN), and complement C3, correlated with composite SLEDAI, but did not significantly associate with many individual SLE pathologies. Two clusters of proteins were associated with renal disease in lupus nephritis samples. One cluster included markers of infiltrating leukocytes and the second cluster included markers of tissue remodelling. In patients with discoid lupus, a distinct signature consisting of elevated immunoglobulin A autoantibodies and interleukin-23 was observed. Our findings indicate that proteins from blood samples can be used to identify protein signatures that are distinct from established SLE biomarkers and SLEDAI and could be used to conveniently monitor multiple inflammatory pathways present in different organ systems.

scholarly journals Assessment of SLEDAI Score in Children with Lupus Nephritis Class III-IV in Vietnam National Children’s Hospital

2020 ◽  
Vol 36 (2) ◽  
Author(s):  
Duong Thi Thanh Binh ◽  
Nguyen Thu Huong ◽  
Nguyen Thi Kien ◽  
Pham Van Dem ◽  
Tran Minh Dien
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Activity Index ◽  
Disease Activity Index ◽  
Class Iii ◽  
Systemic Lupus ◽  
Sledai Score ◽  
Pediatric Systemic Lupus Erythematosus

This study describes clinical, paraclinical characteristics and treatment response in children with nephritis class II-IV caused by systemic lupus erythematosus and validates SLEDAI for the evaluation of disease activity and the appropriate treatment strategy. A cross-sectional descriptive study was carried out on 40 children, 37 girls (92%) and 3 boys (8%), with an average age of 11.7 years with lupus nephritis class III- IV in Vietnam National Children’s Hospital in 2019. The study results show that the average score of SLEDAI in the children with pericardial and pleural effusions was 20.94 ± 4.09; high blood pressure, 20.89 ± 4.23; and gross hematuria, 20.29 ± 5.03, which were higher than those in children without these manifestations with p< 0.05. The most common kidney manifestations were nephrotic-range nephritis with renal failure (40%) and Glomerulonephritis (35%), corresponding to an average SLEDAI score of 24.25 ± 5.52 and 24.33 ± 3.2, respectively (p = 0.001). SLEDAI had an inverse correlation with the C3 complement value (r -0.315, p <0.05). The average SLEDAI score decreased gradually from 18.75 ± 4.22 to 3.38 ± 3.95 points (p <0.001) after 12 months of treatment.  The study concludes that SLEDAI score was higher in patients with pleural and/or pericardial effusions, hypertension and gross hematuria, nephrotic-range nephritis with kidney failure or glomerulonephritis. SLEDAI score corresponded with the C3 complement value and the average SLEDAI score decreased gradually with treatment. Keywords: Lupus Nephritis class III- IV, SLEDAI. References [1] George Bertsias, Ricard Cervera và Dimitrios T Boumpas, Systemic Lupus Erythematosus: Pathogenesis and Clinical Features<sample chapter 20_mod 17_Systemic Lupus nephritis 2012.pdf> (2012), EULAR Textbook on Rheumatic Diseases, EULAR, 476-505.[2] D.M. Levy and S. Kamphuis, Systemic lupus erythematosus in children and adolescents. Pediatr Clin North Am59(2) (2012)345-64.[3] Thai Thien Nam, 2018, Lupus in National Children,s Hospital, [4] C.Bombardier, M.B. Hurwitz et al, Derivation of the SLEDAI: A disease activity index for lupus patients. The committee on prognosis studies in SLE, Arthritis Rheum 35(6) (1992) 630-640.[5] R. Shamim, S. Farman, S. Batool et al, Association of systemic lupus erythematosus disease activity index score with clinical and laboratory parameters in pediatric onset systemic lupus erythematosus. Pak J Med Sci. 36(3) (2020) 467-472.[6] Le Thuy Hang, Assesment of SLEDAI score and panthology in children with lupus nephritis, 2016, Pediatrician thesis, Hanoi Medical University.[7] S.K.S.M. Nazri, K.K. Wong and W.Z.W.A. Hamid, Pediatric systemic lupus erythematosus. Retrospective analysis of clinico-laboratory parameters and their association with Systemic Lupus Erythematosus Disease Activity Index score, Saudi Med J. 39(6) (2018) 627-631. [8] Nguyen Thuy Duong, clinical, paraclinical and pathology characteristics in children with nephritis caused by systemic lupus erythematosus, 2011, Master thesis, Hanoi Medical University.[9] S.N. Wong, W.K. Chan, J.Hui et al, Membranous lupus nephritis in Chinese children--a case series and review of the literature. Pediatr Nephrol, 24(10)(2009) 1989-1996.[10] N.T.N. Dung, H.T. Loan, S. Nielsen et al, Juvenile systemic lupus erythematosus onset patterns in Vietnamese children: a descriptive study of 45 children. Pediatric Rheumatology Online Journal, 10 (2010) 38-48.[11] T. Pusongchai, J. Jungthirapanich, S. Khositseth, Pediatric Systemic Lupus Erythematosus in Thammasat University Hospital, J Med Assoc Thai. 93(12) (2010) 283-290.    

scholarly journals Efficacy of high-dose methylprednisolone and cyclophosphamide in childhood-onset systemic lupus erythematosus

2020 ◽  
Vol 60 (3) ◽  
pp. 116-23
Author(s):  
Putu Ayunda Trisnia ◽  
Ketut Dewi KUmara Wati ◽  
Komang Ayu Witarini ◽  
Ida Bagus Ramajaya Sutawan ◽  
Hendra Santoso
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
High Dose ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
Sledai Score ◽  
Increased Risk ◽  
High Dose Methylprednisolone

Background Systemic lupus erythematosus (SLE) is a chronic, multisystem, autoimmune disease. Untreated SLE often become progressive and lead to increased risk of mortality. Corticosteroid and cyclophosphamide remain the treatment of choice for severe SLE. Disease activity assessed with SLE Daily Activity Index (SLEDAI). Objective To compare the disease activity of childhood-onset severe SLE at the time of diagnosis, after completion of high dose methylprednisolone, and after three month of cyclophosphamide by using  SLEDAI. Methods This study was conducted in the Division of Pediatric Allergy and Immunology, Department of Child Health, Udayana University/Sanglah Hospital, Denpasar, Bali. Subjects were SLE patient aged 0-18 years who had severe clinical manifestations. Subject received therapy combination of high dose methylprednisolone and cyclophosphamide every 2 weeks for six doses. SLEDAI score was assessed at the time of diagnosis, after completion of high dose methylprednisolone, and after three month of cyclophosphamide. Results During the study period, 51 children were diagnosed as SLE. Twenty-one subjects were included for analysis. Median SLEDAI score at the time of diagnosis was 23 (range 13-39). SLEDAI score after three months of cyclophosphamide was decreased to 2 (range 0-14). Post hoc analysis with Wilcoxon signed-rank test showed the improvement of SLEDAI score at the time of diagnosis and after three months of cyclophosphamide was statistically significant  (Z=-4.016, P<0.0001). Conclusion SLEDAI score reduced after completion of high-dose methylprednisolone and three month of cyclophosphamide therapy.

scholarly journals Efficacy of high-dose methylprednisolone and cyclophosphamide in childhood-onset systemic lupus erythematosus

2020 ◽  
Vol 60 (3) ◽  
pp. 117-24
Author(s):  
Putu Ayunda Trisnia ◽  
Ketut Dewi KUmara Wati ◽  
Komang Ayu Witarini ◽  
Ida Bagus Ramajaya Sutawan ◽  
Hendra Santoso
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
High Dose ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
Sledai Score ◽  
Increased Risk ◽  
High Dose Methylprednisolone

Background Systemic lupus erythematosus (SLE) is a chronic, multisystem, autoimmune disease. Untreated SLE often become progressive and lead to increased risk of mortality. Corticosteroid and cyclophosphamide remain the treatment of choice for severe SLE. Disease activity assessed with SLE Daily Activity Index (SLEDAI). Objective To compare the disease activity of childhood-onset severe SLE at the time of diagnosis, after completion of high dose methylprednisolone, and after three month of cyclophosphamide by using  SLEDAI. Methods This study was conducted in the Division of Pediatric Allergy and Immunology, Department of Child Health, Udayana University/Sanglah Hospital, Denpasar, Bali. Subjects were SLE patient aged 0-18 years who had severe clinical manifestations. Subject received therapy combination of high dose methylprednisolone and cyclophosphamide every 2 weeks for six doses. SLEDAI score was assessed at the time of diagnosis, after completion of high dose methylprednisolone, and after three month of cyclophosphamide. Results During the study period, 51 children were diagnosed as SLE. Twenty-one subjects were included for analysis. Median SLEDAI score at the time of diagnosis was 23 (range 13-39). SLEDAI score after three months of cyclophosphamide was decreased to 2 (range 0-14). Post hoc analysis with Wilcoxon signed-rank test showed the improvement of SLEDAI score at the time of diagnosis and after three months of cyclophosphamide was statistically significant  (Z=-4.016, P<0.0001). Conclusion SLEDAI score reduced after completion of high-dose methylprednisolone and three month of cyclophosphamide therapy.

Microprobe analysis of inclusion bodies related to two benzofuran derivatives

1987 ◽  
Vol 45 ◽  
pp. 672-673
Author(s):  
T. L. Benning ◽  
P. Ingram ◽  
J. D. Shelburne
Keyword(s):  
Inclusion Bodies ◽  
Uranyl Acetate ◽  
Multiple Organ ◽  
High Dose ◽  
En Bloc ◽  
Tissue Samples ◽  
Transmission Electron ◽  
Organ Systems ◽  
Dense Inclusion ◽  
Melanin Complex

Two benzofuran derivatives, chlorpromazine and amiodarone, are known to produce inclusion bodies in human tissues. Prolonged high dose chlorpromazine therapy causes hyperpigmentation of the skin with electron-dense inclusion bodies present in dermal histiocytes and endothelial cells ultrastructurally. The nature of the deposits is not known although a drug-melanin complex has been hypothesized. Amiodarone may also cause cutaneous hyperpigmentation and lamellar lysosomal inclusion bodies have been demonstrated within the cells of multiple organ systems. These lamellar bodies are believed to be the product of an amiodarone-induced phospholipid storage disorder. We performed transmission electron microscopy (TEM) and energy dispersive x-ray microanalysis (EDXA) on tissue samples from patients treated with these drugs, attempting to detect the sulfur atom of chlorpromazine and the iodine atom of amiodarone within their respective inclusion bodies.A skin biopsy from a patient with hyperpigmentation due to prolonged chlorpromazine therapy was fixed in 4% glutaraldehyde and processed without osmium tetroxide or en bloc uranyl acetate for Epon embedding.

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