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lupus, end of life

Margarida Figueiredo-Braga, Caleb Cornaby, Miguel Bernardes, Marta Figueiredo, Cristina Dos Santos Mesquita, Lúcia Costa, Brian D Poole
BACKGROUND: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects a large number of people throughout the world. Anxiety, depression and fatigue are common symptoms of SLE that substantially contribute to decreased quality of life. This study investigates the interplay between physical and psychiatric manifestations of lupus. To this end, an SLE patient cohort was examined for correlations between clinical presentation, laboratory tests, and psychological indicators...
2018: PloS One
Mohamed Moustafa Rizk, Eman Tayae Elsayed, Ahmed Fathi ElKeraie, Ireny Ramzy
Lupus nephritis (LN) is a major cause of morbidity and mortality in systemic lupus erythematosus (SLE). Previous studies suggest that mutant A20 binding inhibitor of NF-κB 1 (ABIN1) protein encoded by tumor necrosis factor alpha-induced protein 3 interacting protein 1 (TNIP1) gene is associated with LN via NF-κB dysregulation. The aim of the current study was to evaluate the association of TNIP1 gene SNP rs7708392 with SLE and LN in Egyptian patients. 5' nuclease Allelic discrimination was used to evaluate the frequency of TNIP1 SNP rs7708392 in 53 patients with LN, 57 SLE patients without nephritis and 85 healthy controls...
March 27, 2018: Biochemical Genetics
Alessandra Soriano, Nazareno Smerieri, Stefano Bonilauri, Loredana De Marco, Alberto Cavazza, Carlo Salvarani
Granulomatosis with polyangiitis (GPA) is a small-vessel necrotizing granulomatous vasculitis typically involving upper airways, lungs, and kidneys, which may lead to end-organ damage and life-threatening complications. Major infections during GPA course represent a considerable concern in the management of the disease. Cytomegalovirus (CMV) infection and disease are rare but significant complications in the course of GPA being associated with high morbidity and mortality rates. Colonic perforation due to CMV colitis is exceedingly rare and has so far almost exclusively been documented in HIV, renal transplant, and systemic lupus erythematosus patients...
January 4, 2018: Clinical Rheumatology
Audrey A Margery-Muir, Christine Bundell, Delia Nelson, David M Groth, John D Wetherall
Factors are reviewed that contribute to the contemporary view of a disproportionate prevalence and incidence of SLE in females. Recent studies on the epidemiology of SLE report that global incidences and prevalences of SLE for Caucasian and Black populations are of the order of 5.5 and 13.1 per year and 81 and 212 per 100,000 persons respectively. Both parameters displayed age dependent variation over a 90-year lifespan. The female to male (F:M) incidence of SLE varied with age, being approximately 1 during the first decade of life, followed by a sharp increase to 9 during the 4th decade, thence declining in subsequent decades before an increase during the 7th or 8th decade...
March 2017: Autoimmunity Reviews
Melinda Zsuzsanna Szabó, Emese Kiss
The authors present the latest guideline for the treatment of lupus nephritis and their own single-centre results with mycofenolate mofetil treated lupus nephritis. Lupus nephritis and mainly its proliferative form is a frequent and potentially life-threatening manifestation of systemic lupus erythematosus that can lead to end-stage renal disease. The treatment of lupus nephritis greatly improved in the last decades; mycofenolate mofetil has become an alternative of cyclophosphamide both in remission induction and as a maintenance regimen as well in the treatment of Class III and IV glomerulonephritis...
August 2016: Orvosi Hetilap
L B Lewandowski, L E Schanberg, N Thielman, A Phuti, A A Kalla, I Okpechi, P Nourse, P Gajjar, G Faller, P Ambaram, H Reuter, G Spittal, C Scott
BACKGROUND: Systemic lupus erythematosus (SLE) is a life-threatening multisystem autoimmune disease that is more severe in patients of African ancestry and children, yet pediatric SLE on the African continent has been understudied. This study describes a cohort of pediatric SLE (PULSE) patients in South Africa. METHODS: Patients with a diagnosis of SLE (1997 American College of Rheumatology criteria) diagnosed prior to age 19 years in Cape Town, South Africa, were enrolled in this cross-sectional study from September 2013 to December 2014...
August 3, 2016: Lupus
John P O'Laughlin, Parag H Mehta, Brian C Wong
We present a case of a syncopal episode resulting from significant QT interval prolongation in a patient on hydroxychloroquine for the treatment of systemic lupus erythematosus and end stage renal disease. The patient had been treated with hydroxychloroquine for two years prior to presentation. After thorough workup for secondary causes of QT interval prolongation hydroxychloroquine was discontinued and the patient's QT interval shortened. The patient was treated with mexiletine to prevent sudden ventricular arrhythmias, which was unique compared to other documented cases in which lidocaine was used...
2016: Case Reports in Cardiology
David A Hart
Considerable research effort has been invested in attempting to understand immune dysregulation leading to autoimmunity and target organ damage. In systemic lupus erythematosus (SLE), patients can develop a systemic disease with a number of organs involved. One of the major target organs is the kidney, but patients vary in the progression of the end-organ targeting of this organ. Some patients develop glomerulonephritis only, while others develop rapidly progressive end organ failure. In murine models of SLE, renal involvement can also occur...
2016: Biology of Sex Differences
Morton Scheinberg, Flavio Fernando Nogueira de Melo, Adrian Nogueira Bueno, Carolyne Mendes Costa, Maria Lucia Alvares de Azevedo Bahr, Enio Ribeiro Reis
The objective of the study was to evaluate prospectively real-life experience on the effect of belimumab on patients with active systemic lupus erythematosus (SLE). Forty-eight patients with active SLE were evaluated after 1 year of continuous treatment. Thirty-eight patients were still on treatment at the end of 1 year, and it was possible to observe significant clinical improvement in the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score with a decrease from 12 ± 3.0 to 2.5 ± 2...
July 2016: Clinical Rheumatology
Anne Contis, Helene Vanquaethem, Marie-Elise Truchetet, Lionel Couzi, Claire Rigothier, Christophe Richez, Estibaliz Lazaro, Pierre Duffau
Lupus nephritis is a life-threatening complication of systemic lupus erythematosus. The standard treatment for this condition, including corticosteroids and cyclophosphamide, results in a 70 % remission rate at 12 months, but it is also associated with significant morbidity. Rituximab, a chimeric anti-CD20 antibody, could be useful, given the central role of B cells in the pathogenesis of systemic lupus erythematosus. Case reports and retrospective series have reported that rituximab is effective for refractory lupus nephritis...
February 2016: Clinical Rheumatology
Christina Lam, Stephanie W Liu, Henry B Townsend, Alisa N Femia, Abrar A Qureshi, Ruth Ann Vleugels
IMPORTANCE: Patients with cutaneous lupus erythematosus (CLE) experience significant morbidity and poor quality of life. In the absence of a dermatologist's examination, no reliable tool exists to confirm whether a patient has CLE for use in epidemiologic studies. OBJECTIVE: To determine whether the Cutaneous Lupus Screening (CLUSE) tool can detect cases of CLE by measuring its performance in individuals with dermatologist-diagnosed CLE compared with individuals without CLE...
January 2016: JAMA Dermatology
Kirby A M Brennan, Ann-Marie Creaven
PURPOSE: Because symptoms are not immediately visible to others, systemic lupus erythematosus (SLE) is often considered an invisible illness. This invisibility can reduce the social support received from network members and adversely affect the quality of life. In the light of this, social support from formal support groups and from medical professionals can be particularly important; however, literature examining support from these sources is scarce. The purpose of this study was to explore the nature and impact of social support from medical professionals and from support groups for individuals with SLE...
May 2016: Quality of Life Research
Christine Fox, Karen Walker-Bone
At the end of 2013, 35 million people worldwide were infected with HIV. The prognosis of HIV has been transformed by combination antiretroviral therapy (cART). Providing compliance is good, the use of cART has normalised the life expectancy of HIV-infected people leading to a growing population of people with chronic infection. Management of HIV patients has therefore needed to adapt in order to not only control viral activity but also manage long-term complications of HIV and cART. Rheumatological manifestations of HIV were first described in 1989...
April 2015: Best Practice & Research. Clinical Rheumatology
John G Hanly, Aidan G O'Keeffe, Li Su, Murray B Urowitz, Juanita Romero-Diaz, Caroline Gordon, Sang-Cheol Bae, Sasha Bernatsky, Ann E Clarke, Daniel J Wallace, Joan T Merrill, David A Isenberg, Anisur Rahman, Ellen M Ginzler, Paul Fortin, Dafna D Gladman, Jorge Sanchez-Guerrero, Michelle Petri, Ian N Bruce, Mary Anne Dooley, Rosalind Ramsey-Goldman, Cynthia Aranow, Graciela S Alarcón, Barri J Fessler, Kristjan Steinsson, Ola Nived, Gunnar K Sturfelt, Susan Manzi, Munther A Khamashta, Ronald F van Vollenhoven, Asad A Zoma, Manuel Ramos-Casals, Guillermo Ruiz-Irastorza, S Sam Lim, Thomas Stoll, Murat Inanc, Kenneth C Kalunian, Diane L Kamen, Peter Maddison, Christine A Peschken, Soren Jacobsen, Anca Askanase, Chris Theriault, Kara Thompson, Vernon Farewell
OBJECTIVE: To determine nephritis outcomes in a prospective multi-ethnic/racial SLE inception cohort. METHODS: Patients in the Systemic Lupus International Collaborating Clinics inception cohort (≤15 months of SLE diagnosis) were assessed annually for estimated glomerular filtration rate (eGFR), proteinuria and end-stage renal disease (ESRD). Health-related quality of life was measured by the Short Form (36 questions) health survey questionnaire (SF-36) subscales, mental and physical component summary scores...
February 2016: Rheumatology
M Tuğcu, Ç Ruhi, B Boynueğri, U Kasapoğlu, Ö Can, G Kiliçoğlu, G Şahin, M Titiz
Differential diagnosis of post-transplant infections should include rare/uncommon foci and pathogens. We present a rare case of life-threatening infection, a splenic abscess in a 53-year-old woman who was transplanted with a cadaveric kidney 5 months previously. The patient was admitted to our clinic with chills, shivering, and fever. She required a kidney transplant because of end-stage renal disease secondary to systemic lupus erythematosus, which had previously been treated by means of peritoneal dialysis for 7 years, until encapsulated sclerosing peritonitis developed, at which time therapy was changed to hemodialysis for 1 year...
June 2015: Transplantation Proceedings
Beatrice Goilav, Chaim Putterman, Tamar B Rubinstein
Lupus nephritis (LN), the renal involvement in systemic lupus erythematosus, is currently diagnosed by histopathology obtained by percutaneous renal biopsy and is associated with increased morbidity and mortality in both adults and children. LN is more prevalent and severe in children, requiring aggressive and prolonged immunosuppression. The consequences of the diagnosis and its treatment have devastating long-term effects on the growth, well-being and quality of life of affected children. The paucity of reliable clinical indicators of the presence and severity of renal involvement have contributed to a halt in the reduction of progression to end-stage renal disease in recent years...
2015: Biomarkers in Medicine
Glauce L Lima, Juliane Paupitz, Nadia E Aikawa, Liliam Takayama, Eloisa Bonfa, Rosa M R Pereira
OBJECTIVE: Vitamin D has an important immunomodulatory effect, but there are no trials that directly address the boosting of serum levels of 25-hydroxyvitamin D (25[OH]D) in juvenile-onset systemic lupus erythematosus (SLE). The aim of this study was to evaluate the effect of vitamin D supplementation on disease activity and fatigue in juvenile-onset SLE. METHODS: This study was a randomized, double-blind, placebo-controlled, 24-week trial. Forty juvenile-onset SLE patients were randomized (1:1) to receive oral cholecalciferol 50,000 IU/week (juvenile-onset SLE-VitD) or placebo (juvenile-onset SLE-PL)...
January 2016: Arthritis Care & Research
Christa Ky, Brian Swasdibutra, Shaadi Khademi, Sheetal Desai, Vivian Laquer, Sergei A Grando
Cutaneous lupus erythematosus (CLE) is a chronic inflammatory autoimmune skin disease. Evidence-based therapy for CLE is lacking in the most part. Intravenous immunoglobulin (IVIg) is being increasingly utilized as off-label therapy for a variety of autoimmune and inflammatory conditions, especially in dermatology. The usefulness of IVIg in CLE is not well established. The goal of the present study was to obtain the proof-of-concept evidence that IVIg can control acute CLE and thus replace current systemic immunosuppressive therapy that causes severe side effects and adverse reactions...
March 16, 2015: Dermatology Reports
Adriano Taddeo, Laleh Khodadadi, Caroline Voigt, Imtiaz M Mumtaz, Qingyu Cheng, Katrin Moser, Tobias Alexander, Rudolf A Manz, Andreas Radbruch, Falk Hiepe, Bimba F Hoyer
INTRODUCTION: Autoantibodies contribute significantly to the pathogenesis of systemic lupus erythematosus (SLE). Unfortunately, the long-lived plasma cells (LLPCs) secreting such autoantibodies are refractory to conventional immunosuppressive treatments. Although generated long before the disease becomes clinically apparent, it remains rather unclear whether LLPC generation continues in the established disease. Here, we analyzed the generation of LLPCs, including autoreactive LLPCs, in SLE-prone New Zealand Black/New Zealand White F1 (NZB/W F1) mice over their lifetime, and their regeneration after depletion...
2015: Arthritis Research & Therapy
Adriano Taddeo, Laleh Khodadadi, Caroline Voigt, Imtiaz M Mumtaz, Qingyu Cheng, Katrin Moser, Tobias Alexander, Rudolf A Manz, Andreas Radbruch, Falk Hiepe, Bimba F Hoyer
INTRODUCTION: Autoantibodies contribute significantly to the pathogenesis of systemic lupus erythematosus (SLE). Unfortunately, the long-lived plasma cells (LLPCs) secreting such autoantibodies are refractory to conventional immunosuppressive treatments. Although generated long before the disease becomes clinically apparent, it remains rather unclear whether LLPC generation continues in the established disease. Here, we analyze the generation of LLPCs, including autoreactive LLPCs, in lupus-prone New Zealand Black/New Zealand White F1 (NZB/W F1) mice over their lifetime, and their regeneration after depletion...
December 2015: Arthritis Research & Therapy
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