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E Hasegawa, H Sato, Y Wada, K Takai, A Wakamatsu, Y Nozawa, T Nakatsue, T Kuroda, Y Suzuki, M Nakano, I Narita
Purpose TAFRO syndrome is a novel disorder manifesting as fever, anasarca, thrombocytopenia, renal insufficiency and organomegaly, and its etiology has not been clarified. The aim of this study was to elucidate similarities and differences between systemic lupus erythematosus (SLE) and TAFRO syndrome. Methods We examined 46 consecutive patients diagnosed with SLE and determined whether they meet the proposed diagnostic criteria for TAFRO syndrome (2015 version). Results Of the 46 patients with SLE, four (8...
January 1, 2017: Lupus
#2
E K Tannor, W D Bates, M R Moosa
Purpose Clinically, repeat renal biopsies (RRBs) have been performed in lupus nephritis to identify changes in class, plan treatment and assist in prognostication. We set out to compare the histopathological features and outcomes of disease flare and protocol biopsy patients. Methods A retrospective descriptive study was conducted on repeat biopsies performed between January 1984 and December 2015 in lupus nephritis patients. Disease flares and protocol biopsies were compared. Results Of 614 systemic lupus erythematosus (SLE) renal biopsies, 127 (20...
January 1, 2017: Lupus
#3
Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension.
Sean Gaine, Kelly Chin, Gerry Coghlan, Richard Channick, Lilla Di Scala, Nazzareno Galiè, Hossein-Ardeschir Ghofrani, Irene M Lang, Vallerie McLaughlin, Ralph Preiss, Lewis J Rubin, Gérald Simonneau, Olivier Sitbon, Victor F Tapson, Marius M Hoeper
Patients with connective tissue disease-associated pulmonary arterial hypertension (PAH-CTD) have a poor prognosis compared with other aetiologies. The underlying CTD can influence treatment response and outcomes. We characterised the GRIPHON study PAH-CTD subgroup and evaluated response to selexipag.Of 334 patients with PAH-CTD, PAH was associated with systemic sclerosis (PAH-SSc) in 170, systemic lupus erythematosus (PAH-SLE) in 82 and mixed CTD/CTD-other in 82. For the primary composite endpoint of morbidity/mortality, hazard ratios (HR) and 95% CI were calculated using Cox proportional hazard models...
August 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
#4
Gangduo Wang, Jianling Wang, G A Shakeel Ansari, M Firoze Khan
Tetrachloroethene (perchloroethylene, PCE), an ubiquitous environmental contaminant, has been implicated in inducing autoimmunity/autoimmune diseases (ADs), including systemic lupus erythematosus (SLE) and scleroderma in humans. However, experimental evidence suggesting the potential of PCE in mediating autoimmunity is lacking. This study was, therefore, undertaken to explore PCE's potential in inducing/exacerbating an autoimmune response. Six-week old female MRL+/+ mice, in groups of 6 each, were treated with PCE (0...
August 14, 2017: Toxicology and Applied Pharmacology
#5
S Sam Lim, Alan J Kivitz, Doug McKinnell, M Edward Pierson, Faye S O'Brien
PURPOSE: We elicited patient experiences from clinical trial simulations to aid in future trial development and to improve patient recruitment and retention. PATIENTS AND METHODS: Two simulations of draft Phase II and Phase III anifrolumab studies for systemic lupus erythematosus (SLE)/lupus nephritis (LN) were performed involving African-American patients from Grady Hospital, an indigent care hospital in Atlanta, GA, USA, and white patients from Altoona Arthritis and Osteoporosis Center in Altoona, PA, USA...
2017: Patient Preference and Adherence
#6
Joseph C Lownik, Andrea J Luker, Sheela R Damle, Lauren Folgosa Cooley, Riham El Sayed, Andreas Hutloff, Costantino Pitzalis, Rebecca K Martin, Mohey Eldin M El Shikh, Daniel H Conrad
The proper regulation of ICOS and ICOS ligand (ICOSL) has been shown to be essential for maintaining proper immune homeostasis. Loss of either protein results in defective humoral immunity, and overexpression of ICOS results in aberrant Ab production resembling lupus. How ICOSL is regulated in response to ICOS interaction is still unclear. We demonstrate that a disintegrin and metalloproteinase (ADAM)10 is the primary physiological sheddase of ICOSL in mice and humans. Using an in vivo system in which ADAM10 is deleted only on B cells, elevated levels of ICOSL were seen...
August 16, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
#7
Panagiotis Tsikouras, Georg-Friedrich von Tempelhoff, Werner Rath
Venous thromboembolism (VTE) remains a leading cause of direct maternal deaths in the developed countries. The incidence of VTE has increased significantly during the past two decades. The absolute risk of VTE is estimated 0.6-2.2 per 1000 deliveries. Compared with age-matched non-pregnant women, the daily risk of VTE is increased 7- to 10-fold for antepartum VTE, but it is 15- to 35-fold for postpartum VTE. The incidence of pulmonary embolism (PE) during the first 6 weeks postpartum is nearly 15-fold higher compared to the incidence in pregnancy, and remains significantly increased up to 12 weeks postpartum...
August 2017: Zeitschrift Für Geburtshilfe und Neonatologie
#8
E S Park, S S Ahn, S M Jung, J J Song, Y-B Park, S-W Lee
We investigated renal outcome of kidney-transplantation in 19 Korean recipients with biopsy-proven lupus nephritis and compared it with 18 Korean age- and gender-matched recipients without lupus nephritis who were diagnosed with end-stage renal disease caused by renal diseases other than lupus nephritis in a single centre. We reviewed histological findings of kidneys and calculated cumulative dose of immunosuppressive agents. We assessed renal flare of systemic lupus erythematosus, recurrence of lupus nephritis and graft failure as prognosis...
January 1, 2017: Lupus
#9
A A Imam, H E Ibrahim, M A A Farghaly, U M Alkholy, H H Gawish, N Abdalmonem, A M Sherif, Y F Ali, M E Hamed, N M Waked, M M Fathy, A M Khalil, M A Noah, M S Hegab, B R Ibrahim, R M Nabil, L A Fattah
Background Childhood-onset systemic lupus erythematosus (cSLE) is a lifelong autoimmune disorder. The vitamin D receptor (VDR) gene is a potential candidate gene for cSLE susceptibility. In this study, we aimed to investigate the FokI polymorphism in the VDR gene in Egyptian children and adolescents with SLE, to determine whether this polymorphism could be a genetic marker for cSLE susceptibility or disease activity and we also measured the serum level of 25-hydroxyvitamin D [25(OH) D] to assess its relation to such polymorphism...
January 1, 2017: Lupus
#10
A R Poliwczak, E Waszczykowska, B Dziankowska-Bartkowiak, M Koziróg, K Dworniak
Background Systemic lupus erythematosus is a progressive autoimmune disease. There are reports suggesting that patients even without overt signs of cardiovascular complications have impaired autonomic function. The aim of this study was to assess autonomic function using heart rate turbulence and heart rate variability parameters indicated in 24-hour ECG Holter monitoring. Methods Twenty-six women with systemic lupus erythematosus and 30 healthy women were included. Twenty-four hour ambulatory ECG-Holter was performed in home conditions...
January 1, 2017: Lupus
#11
S Baba, Y Katsumata, Y Okamoto, Y Kawaguchi, M Hanaoka, H Kawasumi, H Yamanaka
We aimed to validate the reliability of the Medical Outcomes Study Short Form-36 (SF-36) among Japanese patients with systemic lupus erythematosus (SLE). Japanese patients with SLE ( n = 233) completed the SF-36 and other related demographic questionnaires, and physicians simultaneously completed the SLE Disease Activity Index 2000 (SLEDAI-2K) and the Systemic Lupus International Collaborating Clinics Damage Index (SDI). Patients were prospectively followed for a repeat assessment the following year. The SF-36 subscales demonstrated acceptable internal consistency (Cronbach's α of 0...
January 1, 2017: Lupus
#12
REVIEW
Leendert A Trouw, Matthew C Pickering, Anna M Blom
Complement activation is associated with common rheumatic diseases such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA) and systemic vasculitis. Evidence linking complement activation to these diseases includes the presence of complement deposition in affected tissues, decreased levels of complement proteins and high levels of complement activation fragments in the blood and/or synovial fluid of patients with these diseases, as well as data from experimental models. Eculizumab, a monoclonal antibody that inhibits the complement component C5, is now approved for the treatment of rare conditions involving complement hyperactivation, and the success of this therapy has renewed interest in understanding the utility of complement inhibition in rheumatological practice, particularly for SLE...
August 10, 2017: Nature Reviews. Rheumatology
#13
Maria G Tektonidou, Laura B Lewandowski, Jinxian Hu, Abhijit Dasgupta, Michael M Ward
OBJECTIVE: To determine trends in survival among adult and paediatric patients with systemic lupus erythematosus (SLE) from 1950 to the present. METHODS: We performed a systematic literature review to identify all published cohort studies on survival in patients with SLE. We used Bayesian methods to derive pooled survival estimates separately for adult and paediatric patients, as well as for studies from high-income countries and low/middle-income countries. We pooled contemporaneous studies to obtain trends in survival over time...
August 9, 2017: Annals of the Rheumatic Diseases
#14
Chris Chamberlain, Peter J Colman, Ann M Ranger, Linda C Burkly, Geoffrey I Johnston, Christian Otoul, Christian Stach, Miren Zamacona, Thomas Dörner, Murray Urowitz, Falk Hiepe
OBJECTIVES: Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease associated with diffuse immune cell dysfunction. CD40-CD40 ligand (CD40L) interaction activates B cells, antigen-presenting cells and platelets. CD40L blockade might provide an innovative treatment for systemic autoimmune disorders. We investigated the safety and clinical activity of dapirolizumab pegol, a polyethylene glycol conjugated anti-CD40L Fab' fragment, in patients with SLE. METHODS: This 32-week randomised, double-blind, multicentre study (NCT01764594) evaluated repeated intravenous administration of dapirolizumab pegol in patients with SLE who were positive for/had history of antidouble stranded DNA/antinuclear antibodies and were on stable doses of immunomodulatory therapies (if applicable)...
August 5, 2017: Annals of the Rheumatic Diseases
#15
M Benucci, A Damiani, F Li Gobbi, V Grossi, M Infantino, A Arena, M Manfredi
Jaccoud's arthropathy (JA) is a chronic, non erosive, rheumatoid-like deformity associated with rheumatic fever (RF) and systemic lupus erythematosus and with other diseases such as psoriatic arthritis, connective tissue diseases, hypocomplementemic urticarial vasculitis, infections, sarcoidosis and neoplasia. We described a case of JA in a patient with cutaneous psoriasis but with a particular disease evolution associated with idiopathic retropritoneal fibrosis (IRF), evaluated with computed tomography, magnetic resonance and 18F-FDG PET/ CT...
August 3, 2017: Reumatismo
#16
Desiree R Azizoddin, Geraldine Zamora-Racaza, Sarah R Ormseth, Lekeisha A Sumner, Chelsie Cost, Julia R Ayeroff, Michael H Weisman, Perry M Nicassio
Our analyses examined whether reserve capacity factors would explain the relationship between socioeconomic status (SES) and symptoms of depression/anxiety in patients with systemic lupus erythematosus (SLE). We assessed disease activity, depression/anxiety symptoms, and intrapersonal and interpersonal reserve capacity measures in 128 patients with SLE. Multiple meditational analyses revealed that intrapersonal and interpersonal psychosocial aspects of reserve capacity fully mediated the relationship between SES and depression/anxiety...
August 3, 2017: Journal of Clinical Psychology in Medical Settings
#17
Daniel Seoane-Mato, Carlos Sánchez-Piedra, Lucía Silva-Fernández, Francisca Sivera, Francisco J Blanco, Fernando Pérez Ruiz, Antonio Juan-Mas, José M Pego-Reigosa, Javier Narváez, Neus Quilis Martí, Raúl Cortés Verdú, Fred Antón-Pagés, Víctor Quevedo Vila, Gustavo Añez Sturchio, Fermín Medina Varo, María Del Mar Ruiz Tudela, Antonio Romero Pérez, Javier Ballina, Anahy Brandy García, Dolores Fábregas Canales, Teresa Font Gayá, Carolina Bordoy Ferrer, Beatriz González Álvarez, Laura Casas Hernández, Fátima Álvarez Reyes, Mónica Delgado Sánchez, Cristina Martínez Dubois, Simón Ángel Sánchez-Fernández, Luisa Marena Rojas Vargas, Paula Virginia García Morales, Alejandro Olivé, Paula Rubio Muñoz, Marta Larrosa, Noemí Navarro Ricos, Eduard Graell Martín, Eugenio Chamizo, Lara Chaves Chaparro, Sara Rojas Herrera, Jordi Pons Dolset, Miguel Ángel Polo Ostariz, Susana Ruiz-Alejos Garrido, Cristina Macía Villa, Ana Cruz Valenciano, María Luisa González Gómez, Mercedes Morcillo Valle, Deseada Palma Sánchez, María José Moreno Martínez, Marta Mayor González, Joana Atxotegi Sáenz de Buruaga, Irati Urionagüena Onaindia, Boris Anthony Blanco Cáceres, Federico Díaz-González, Sagrario Bustabad
AIMS: To describe the methodology of the EPISER 2016 (study of the prevalence of rheumatic diseases in adult population in Spain), as well its strengths and limitations. The aim of this study is to estimate the prevalence of rheumatoid arthritis (RA), psoriatic arthritis (PsA), ankylosing spondylitis (AS), systemic lupus erythematosus (SLE), Sjögren's syndrome (SS), osteoarthritis (knee, hip, hands, and cervical and lumbar spine), fibromyalgia, gout and clinical osteoporotic fracture...
July 31, 2017: Reumatología Clinica
#18
Sasha Bernatsky, Ann E Clarke, Omid Zahedi Niaki, Jeremy Labrecque, Laura E Schanberg, Earl D Silverman, Kristen Hayward, Lisa Imundo, Hermine I Brunner, Kathleen A Haines, Randy Q Cron, Kiem Oen, Linda Wagner-Weiner, Alan M Rosenberg, Kathleen M O'Neil, Ciarán M Duffy, Emily von Scheven, Lawrence Joseph, Jennifer L Lee, Rosalind Ramsey-Goldman
OBJECTIVE: To determine cancer incidence in a large pediatric-onset systemic lupus erythematosus (SLE) population. METHODS: Data were examined from 12 pediatric SLE registries in North America. Patients were linked to their regional cancer registries to detect cancers observed after cohort entry, defined as date first seen in the clinic. The expected number of malignancies was obtained by multiplying the person-years in the cohort (defined from cohort entry to end of followup) by the geographically matched age-, sex-, and calendar year-specific cancer rates...
August 1, 2017: Journal of Rheumatology
#19
S Cooray, H Zhang, R Breen, G Carr-White, R Howard, M Cuadrado, D D'Cruz, G Sanna
Central nervous system (CNS) tuberculosis (TB) is a rare but catastrophic event in patients with systemic lupus erythematosus (SLE). Here we report a case of cerebral TB in a patient with lupus myocarditis and nephritis, following cyclophosphamide immunosuppression. To our knowledge this is the first reported case of cerebral TB in SLE in a non-endemic country. A 31-year-old female with SLE and a history of regular travel to Kenya presented to our centre with clinical features of acute heart failure. She was diagnosed with severe lupus myocarditis, and a renal biopsy also confirmed lupus nephritis...
January 1, 2017: Lupus
#20
B T Ma, H X Yin, H Wu, J B Chang, X Zhang, L Wang, X M Leng, W B Ma, Y N Li, R Z Wang, J J Wei
Objective: To investigate the pathogenesis and management of the hydrocephalus in patients with systemic lupus erythematosus (SLE). Methods: Eight cases of hospitalized lupus patients with hydrocephalus in Peking Union Medical College Hospital from Jan 1990 to Mar 2017 were analyzed retrospectively.We collected the patients' medical records including medical history, CT and/or MRI images and analysis of cerebrospinal fluid via lumbar puncture in order to investigate the pathogenesis of the hydrocephalus in patients with SLE and summarize treatment experience...
July 18, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
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