Successful treatment of refractory thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus with combination of plasma exchange and low-dose rituximab

Lupus ◽  
2020 ◽  
Vol 29 (14) ◽  
pp. 1961-1967
Author(s):  
Wanlu Ma ◽  
Wei Bai ◽  
Xueyan Wu ◽  
Jiuliang Zhao ◽  
Mengtao Li ◽  
...  
Keyword(s):  
Plasma Exchange ◽  
Lupus Erythematosus ◽  
Low Dose ◽  
Immunosuppressive Agents ◽  
High Dose ◽  
Systemic Lupus ◽  
College Hospital ◽  
Usual Practice ◽  
Platelet Counts

Objectives Thrombotic thrombocytopenia purpura (TTP) associated with systemic lupus erythematous (SLE) (i.e., SLE-TTP) is a rare life-threatening disease often requiring intensive immunosuppressive agents, in addition to high-dose corticosteroids and plasma exchange (PEX). The optimal therapy of rituximab is unclear, but 375 mg/m2 weekly for 4 weeks is the usual practice, adopted from regimens for non-Hodgkin’s lymphoma. We reported two cases of refractory SLE-TTP that showed good efficacy and prognosis with combination of methylprednisolone (MP) pulse, plasma exchange and low-dose rituximab (100 mg weekly for 4 weeks) treatment. Methods Clinical data and treatment outcomes were reviewed of two patients diagnosed with refractory SLE-TTP at Peking Union Medical College Hospital between July 2017 and July 2018. Results Both patients had SLE and presented with microangiopathic anemia and thrombocytopenia. Laboratory assays revealed high anti-nuclear antibody titers, reduced complement 3 and 4 levels, proteinuria, significantly elevated lactate dehydrogenase, schistocytes on peripheral blood smear, low ADAMTS13 activity, and the presence of ADAMTS13 inhibitor. In both patients, platelet counts remained below 50 × 109/L after MP pulse and 6 PEXs, confirming the diagnosis of refractory SLE-TTP. Low-dose rituximab (100 mg weekly for 4 weeks) was administered in both cases, resulting in normalization of platelet counts and significant reductions in B-lymphocyte counts. No TTP relapse or SLE flare occurred during 24 months of follow-up. Conclusions Our cases confirmed the efficacy and good follow-up outcomes of low-dose rituximab treatment (100 mg weekly for 4 weeks) for refractory SLE-TTP.

Systemic Lupus Erythematosus and Related Disorders

2014 ◽  
pp. 257-270
Author(s):  
Bonnie L. Bermas
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Central Nervous System ◽  
Nervous System ◽  
Lupus Erythematosus ◽  
Immunosuppressive Agents ◽  
Future Research ◽  
High Dose ◽  
Childbearing Age ◽  
Systemic Lupus ◽  
Multisystem Disease

Systemic lupus erythematosus (SLE) is a multisystem disease that preferentially affects women of childbearing age. This disorder is both more common and more severe in individuals of African and Asian ancestry. The etiology of SLE is not well understood, although genetics and environmental stimuli clearly are involved. Whether this disease is caused by a T-cell, B-cell, or other immunologic malfunction is debated, but all would agree that clearly autoantibodies such as antinuclear antibodies and anti–double-stranded DNA contribute to the pathophysiology of this disorder. This multisystem disease can affect the skin, joints, lungs, heart, kidneys, and central nervous system. Most of the morbidity and mortality is from renal and central nervous system (CNS) involvement, although accelerated atherosclerosis has recently been appreciated as a major contributor to disease burden. The treatment of SLE has improved over the past decade with less reliance on high-dose corticosteroids and more emphasis on immunosuppressive agents. It is our hope that future research into the pathophysiology of this disorder and the development of more specific therapy, such as biologics, will improve the outcome of this disease.

Systemic lupus erythematosus-related acute pancreatitis

Lupus ◽  
2020 ◽  
Vol 30 (1) ◽  
pp. 5-14
Author(s):  
Alina Dima ◽  
Daniel Vasile Balaban ◽  
Ciprian Jurcut ◽  
Mariana Jinga
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Acute Pancreatitis ◽  
Abdominal Pain ◽  
Plasma Exchange ◽  
Lupus Erythematosus ◽  
High Dose ◽  
Systemic Lupus ◽  
Serum Lipase ◽  
Autoimmune Pathology ◽  
Short Disease Duration

Introduction Systemic lupus erythematosus (SLE) is a complex autoimmune pathology that can involve any organ. Lupus-related acute pancreatitis (AP) is, together with lupus mesenteric vasculitis, an important cause of SLE-induced acute abdominal pain. Methods A literature search was conducted using the terms “Pancreatitis” and “Lupus Erythematosus, Systemic” on PubMed/Medline and Web of Science from January 2007 to January 2020. Clinical characteristics, diagnostic approach, and treatment principles in SLE-related AP are presented in this review. Results Mainly retrospective reports were identified. The reported incidence of SLE-associated AP ranges from 0.9 to more than 5% of patients. A total of 264 SLE patients were found in the selected research, with a net female predominance (sex ratio 9:1) and mean age of 31.4 years. Abdominal pain was virtually present in all cases. AP occurrence was more frequent in SLE patients with short disease duration, high activity scores, and multiorgan involvement. The AP definition was based on currently available guidelines and after exclusion of any other known causes (including iatrogenic, i.e. drugs), a diagnosis of “idiopathic” SLE-related AP might be sustained. Management is difficult, as there is no standardized therapeutic approach. Of note, glucocorticoid use remains still controversial as, especially for high doses, subsequent pancreatic injury may occur. Monitoring serum lipase levels after high dose steroids might be considered. One study reported beneficial prognostic effect of plasma exchange. Moreover, AP in SLE might raise awareness about macrophage activation syndrome association. Mortality up to one third of AP cases in SLE was reported. Conclusion The SLE-related AP is a rare, but severe, life-threatening complication. Corticosteroids must be used with caution. Plasma exchange could be considered in selected cases.

scholarly journals FRI0171 THE CHANGES OF IMMUNE FUNCTION AND CLINICAL INDEXES WITH SYSTEMIC LUPUS ERYTHEMATOSUS AFTER IMMUNOREGULATORY COMBINATION THERAPIES

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 670.3-670
Author(s):  
X. Liu ◽  
X. Liu ◽  
H. Hou ◽  
X. LI
Keyword(s):  
Systemic Lupus Erythematosus ◽  
T Cells ◽  
Lupus Erythematosus ◽  
Low Dose ◽  
Remission Rate ◽  
Treg Cells ◽  
Control Group ◽  
Combination Therapies ◽  
Systemic Lupus

Background:Recent studies have reported that some drugs such as low-dose interleukin-2, rapamycin, metformin, retinoic acid and coenzyme Q10 could promote the proliferation and functional recovery of regulatory T cells (Treg) in patients with autoimmune diseases. However, the effects on the balance of Treg cells and pro-inflammatory lymphocytes and long-term efficacy have rarely been reported.Objectives:To evaluate the changes of peripheral lymphocyte subsets, conventional drugs and remission rate in patients with systemic lupus erythematosus (SLE) after immunomodulatory combination therapies.Methods:A total of 189 patients with SLE from the Second Affiliated Hospital of Shanxi Medical University from January 2016 to October 2019 were enrolled, who were divided into well-controlled group and untargeted control group taking a full consideration of the patient’s symptoms, signs and related laboratory findings. We measured the absolute counts of B, NK, CD8+T and helper T 1 (Th1), helper T 2 (Th2), helper T 17 (Th17) and Treg cells in peripheral blood of patients before immunomodulatory combination therapies and during the 3 months and 6 months of follow-up and 190 sex- and age- matched control individuals using flow cytometry. Moreover, the ratios of various cells to Treg cells were calculated.Results:Compared with healthy controls, Treg cells in SLE patients were significantly lower before the treatment with immunomodulator, while the ratios of various pro-inflammatory lymphocytes to Treg cells (such as Th2/Treg, Th17/Treg, CD8+T/Treg, etc.) were higher. After 3 months and 6 months with immunomodulatory therapy, the absolute number of Treg cells in peripheral blood of SLE patients increased obviously reaching to normal level. Accordingly, the ratios of various pro-inflammatory lymphocytes to Treg cells recovered. At the same time, the dose of glucocorticoid and disease-modifying antirheumatic drugs (DMARDs) decreased distinctly. Additionally, the well-controlled group was able to maintain a high remission rate, and the untargeted control group could achieve a higher response rate after immunomodulatory treatment.Conclusion:The imbalance between pro-inflammatory lymphocytes and Treg cells caused by the significant decrease of Treg cells may be the main cause of SLE. And immunomodulatory combination therapies we came up with may reverse the imbalance of proinflammatory lymphocytes and Treg cells, which is an potential and effective treatment for SLE.References:[1]Noack M, Miossec P. Th17 and regulatory T cell balance in autoimmune and inflammatory disease[J]. Autoimmun Rev, 2014, 13(6): 668-677.[2]Yu A, Snowhite I, Vendrame F, et al. Selective IL-2 responsiveness of regulatory T cells through multiple intrinsic mechanisms supports the use of low-dose IL-2 therapy in type 1 diabetes. Diabetes. 2015;64: 2172–2183.[3]Schuiveling M, Vazirpanah N, Radstake TRDJ, Zimmermann M, Broen JCA. Metformin, A New Era for an Old Drug in the Treatment of Immune Mediated Disease?[J]. Curr Drug Targets, 2017;18:1-15.Table 1.The changes of remission rate in the no-remission group during follow-up.Follow-up periodTotal patientsRemissionNo-remissionRemission rate(%)Baseline9209203 Months72333945.8a6 Months74423256.8aa: Compared with baseline; b: Compared with 3 months.Acknowledgments:We would like to express our sincere gratitude to all our coworkers and collaborators, Jing Luo, Xiangcong Zhao, Chen Zhang, Qi Wu, Congcong Liang, and Rui Fu for their technical support.Disclosure of Interests:None declared

Intravenous immunoglobulins in systemic lupus erythematosus: from the bench to the bedside

Lupus ◽  
2009 ◽  
Vol 18 (10) ◽  
pp. 884-888 ◽  
Author(s):  
G Zandman-Goddard ◽  
M Blank ◽  
Y Shoenfeld
Keyword(s):  
Systemic Lupus Erythematosus ◽  
B Cell ◽  
Lupus Erythematosus ◽  
Immunosuppressive Agents ◽  
Working Party ◽  
Neurological Involvement ◽  
High Dose ◽  
Systemic Lupus ◽  
First Choice ◽  
European Working

This article is an update on the clinical and research data available on systemic lupus erythematosus (SLE) and intravenous immunoglobulin (IVIg) therapy that includes some studies performed under the umbrella of the European Working Party on SLE. Various mechanisms of IVIg may play a role, some synergistically, in the modulation of SLE. Recently it has been suggested that IVIg also suppresses the expansion of autoreactive B lymphocytes through signalling of the FcgRIIB, idiotype-mediated inhibition of B cell receptors and neutralisation of cytokines such as the B cell survival factors (B cell activation factor (BAFF and APRIL). In case reports and in open trials, high-dose IVIg (2 g/kg over a 5-day period) has consistently been shown to be a beneficial and safe adjunct therapeutic agent for over 20 manifestations in patients with SLE. It can be given as a first choice of therapy in some cases, for example, in neurological involvement and in those patients who refuse certain immunosuppressive agents such as cyclophosphamide, or in patients who have concomitant infections. Furthermore, IVIg may have a steroid-sparing effect although this characteristic needs further investigation. Specific IVIg (an anti-idiotype to anti-DNA, phosphorylcholine and antiphospholipids) has been shown to be effective in experimental murine models. Hence, extractable IVIg that is directed to the specific pathogenic immunoglobulins will enable the more specific therapy for patients with lupus.

scholarly journals Damage accrual and mortality over long-term follow-up in 300 patients with systemic lupus erythematosus in a multi-ethnic British cohort

Rheumatology ◽  
2019 ◽  
Author(s):  
Beatriz Tejera Segura ◽  
Brett Sydney Bernstein ◽  
Thomas McDonnell ◽  
Chris Wincup ◽  
Vera M Ripoll ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
High Dose ◽  
Concomitant Disease ◽  
Systemic Lupus ◽  
Increased Risk ◽  
Damage Accrual ◽  
Long Term Follow Up

Abstract Objective Damage in patients with systemic lupus erythematosus is irreversible change in organs due to disease activity, concomitant disease or medication side-effects. It is measured using the Systemic Lupus International Collaborative Clinics Damage Index (SDI) and is associated with increased mortality. Previous reports have suggested associations between damage accrual and various ethnic, disease and treatment factors, but there is a dearth of long-term follow-up data from large multi-ethnic cohorts. We describe a study of damage and mortality in 300 patients from London, UK followed for up to 40 years. Methods We carried out retrospective analysis of medical records and SDI scores of 300 patients followed for up to 40 years (median 13.3 years). Characteristics of the groups who did and did not develop damage and those who died or survived to the end of follow-up were compared using univariable and multivariable analysis. Kaplan-Meier analysis was used to analyse factors affecting mortality and accrual of damage. Results Damage developed in 231/300 (77%) of patients. There was a linear accrual of damage over 40 years follow-up. Factors associated with damage were African/Caribbean ethnicity, renal and cerebral involvement, early use of high-dose corticosteroids or immunosuppressants, anti-RNP and antiphospholipid antibodies. Damage was strongly associated with mortality. Of 87 patients who died, 93% had damage compared with 70% of survivors (P < 0.001). Conclusion Development of damage is strongly associated with increased mortality. We identified groups at increased risk of developing damage, including those treated with high-dose steroids and immunosuppressants within the first two years.

Outcome of Percutaneous Nephrostomy in Children With Ureteropelvic Junction Obstruction

2020 ◽  
Vol 20 (2) ◽  
pp. 61-64
Author(s):  
Mohammad Mahfuzur Rahman Chowdhury ◽  
Rifat Zaman ◽  
Md Amanur Rasul ◽  
Akm Shahadat Hossain ◽  
Shafiqul Alam Chowdhury ◽  
...  
Keyword(s):  
Renal Function ◽  
Low Dose ◽  
Ureteropelvic Junction Obstruction ◽  
Percutaneous Nephrostomy ◽  
Ureteropelvic Junction ◽  
College Hospital ◽  
Medical College ◽  
Split Renal Function

Introduction and objectives: Congenital ureteropelvic junction obstruction (UPJO) is the most common cause of hydronephrosis. Management protocols are based on the presence of symptoms and when the patient is asymptomatic the function of the affected kidney determines the line of treatment. Percutaneous nephrostomy (PCN) became a widely accepted procedure in children in the 1990s. The aim of the study was to evaluate the results of performing percutaneous nephrostomy (PCN) in all patients with UPJO and split renal function (SRF) of less than 10% in the affected kidney, because the management of such cases is still under debate. Methods:This prospective clinical trial was carried out at Dhaka Medical College Hospital from January 2014 to December 2016. Eighteen consecutive patients who underwent PCN for the treatment of unilateral UPJO were evaluated prospectively. In these children, ultrasonography was used for puncture and catheter insertion. Local anesthesia with sedation or general anesthesia was used for puncture. Pig tail catheters were employed. The PCN remained in situ for at least 4 weeks, during which patients received low-dose cephalosporin prophylaxis. Repeat renography was done after 4 weeks. When there was no significant improvement in split renal function (10% or greater) and PCN drainage (greater than 200 ml per day) then nephrectomies were performed otherwise pyeloplasties were performed. The patients were followed up after pyeloplasty with renograms at 3 months and 6 months post operatively. Results: All the patients had severe hydronephrosis during diagnosis and 14 patients with unilateral UPJO were improved after PCN drainage and underwent pyeloplasty. The rest four patients that did not show improvement in the SRF and total volume of urine output underwent nephrectomy. In the patients with unilateral UPJO who improved after PCN drainage, the SRF was increased to 26.4% ±8.6% (mean± SD) after four weeks and pyeloplasty was performed. At three and six months follow-up, SRF value was 29.2% ±8.5% and 30.8.2% ±8.8% respectively. Conclusion: Before planning of nephrectomy in poorly functioning kidneys (SRF < 10%) due to congenital UPJO, PCN drainage should be done to asses improvement of renal function. Bangladesh Journal of Urology, Vol. 20, No. 2, July 2017 p.61-64

scholarly journals Severe deafness in systemic lupus erythematosus: its immediate relief by plasma exchange.

BMJ ◽  
1982 ◽  
Vol 284 (6326) ◽  
pp. 1374-1374 ◽  
Author(s):  
T J Hamblin ◽  
G J Mufti ◽  
A Bracewell
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Plasma Exchange ◽  
Lupus Erythematosus ◽  
Systemic Lupus
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