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Systemic lupus m

Anne F M Jansen, Ruud P H Raijmakers, Stephan P Keijmel, Renate G van der Molen, Gerald M Vervoort, Jos W M van der Meer, Marcel van Deuren, Chantal P Bleeker-Rovers
Q fever infection can lead to chronic Q fever, a potentially lethal disease occurring in 1-5% of patients infected with Coxiella burnetii, characterized by the persistence of this intracellular bacterium. It usually presents as endocarditis, infected vascular aneurysms, or infected vascular prostheses. This systematic review of the literature discusses the various autoimmune syndromes and B-cell dyscrasias in acute and chronic Q fever patients, that may interfere with or impede recognition and diagnosis of Q fever...
June 13, 2018: European Journal of Internal Medicine
Anne Troldborg, Steffen Thiel, Marten Trendelenburg, Justa Friebus-Kardash, Josephine Nehring, Rudi Steffensen, Søren Werner Karlskov Hansen, Magdalena Janina Laska, Bent Deleuran, Jens Christian Jensenius, Anne Voss, Kristian Stengaard-Pedersen
OBJECTIVE: The pathogenesis of systemic lupus erythematosus (SLE) involves complement activation. Activation of complement through the classical pathway (CP) is well established. However, complement activation through pattern recognition not only happens through the CP, but also through the lectin pathway (LP). We investigated the hypothesis that the LP is activated in SLE and involved in the pathogenesis of the disease. METHODS: Using immunoassays developed in-house, we measured concentrations of LP proteins in a cohort of 372 patients with SLE and 170 controls...
June 15, 2018: Journal of Rheumatology
Tanvi Arkatkar, Holly M Jacobs, Samuel W Du, Quan-Zhen Li, Kelly L Hudkins, Charles E Alpers, David J Rawlings, Shaun W Jackson
B cells are known to promote the pathogenesis of systemic lupus erythematosus (SLE) via the production of pathogenic anti-nuclear antibodies. However, the signals required for autoreactive B cell activation and the immune mechanisms whereby B cells impact lupus nephritis pathology remain poorly understood. The B cell survival cytokine B cell activating factor of the TNF Family (BAFF) has been implicated in the pathogenesis of SLE and lupus nephritis in both animal models and human clinical studies. Although the BAFF receptor has been predicted to be the primary BAFF family receptor responsible for BAFF-driven humoral autoimmunity, in the current study we identify a critical role for signals downstream of Transmembrane Activator and CAML Interactor (TACI) in BAFF-dependent lupus nephritis...
June 12, 2018: Kidney International
A Le Bot, P Jégo, E Donal, E Flécher, M Revest, P Tattevin
Non-infective endocarditis, also referred to as non-bacterial thrombotic endocarditis, represent a wide range of rare pathologies, often severe. This review gathered the data available in the literature, to decipher the major information collected on the pathophysiology, the diagnosis and the treatment of these heterogeneous diseases, often misdiagnosed. Characteristics of non-infective endocarditis are similar to infective endocarditis in terms of valvular lesions (mostly left-sided, with regurgitations and vegetations), and their complications (embolism)...
June 11, 2018: La Revue de Médecine Interne
Cynthia O Anyanwu, Lacy L Sommer, Halyna Kuzyshyn, Jeanette M Camacho, Hala M Eid, Warren R Heymann
The isomorphic response is the appearance of new lesions from a preexisting skin condition at a site of trauma. Discoid lupus erythematosus (DLE) may develop on traumatized skin and also may arise at sites of a prior cutaneous eruption. We report the case of a 20-year-old woman with a history of systemic lupus erythematosus (SLE) and DLE who developed a painful, multidermatomal, vesicular rash on the left breast and back consistent with herpes zoster (HZ) during treatment with systemic immunosuppression. Four months after the HZ resolved, the patient developed new-onset DLE lesions within the prior HZ-affected dermatomes...
May 2018: Cutis; Cutaneous Medicine for the Practitioner
Debora N Setoue, Ana C Pitta, Fernanda J Fiorot, Mariana M Nastri, Glaucia V Novak, Beatriz C Molinari, Juliana C Oliveira, Natali W Gormezano, Ana P Sakamoto, Maria T Terreri, Rosa M Pereira, Claudia Saad-Magalhães, Adriana M Sallum, Katia Kozu, Melissa M Fraga, Daniela P Piotto, Gleice Clemente, Roberto Marini, Hugo R Gomes, Carlos N Rabelo-Junior, Marta M Felix, Maria C Ribeiro, Rozana G Almeida, Ana P Assad, Silvana B Sacchetti, Leandra C Barros, Eloisa Bonfá, Clovis A Silva
OBJECTIVE: To evaluate symptomatic polyautoimmunity (PA) at childhood-onset systemic lupus erythematosus(cSLE) diagnosis, and its association with demographic data, disease activity, clinical manifestations and laboratorial abnormalities in a large Brazilian cSLE population. METHODS: A multicenter retrospective study was performed in 1463 cSLE(ACR criteria) patients from 27 Pediatric Rheumatology services. Symptomatic PA was defined according to the presence of more than one concomitant autoimmune disease(AD) and symptomatic multiple autoimmune syndrome(MAS) was defined as three or more AD...
June 7, 2018: Autoimmunity Reviews
Angélica M Delgado-Vega, Manuel Martínez-Bueno, Nina Y Oparina, David López Herráez, Helga Kristjansdottir, Kristján Steinsson, Sergey V Kozyrev, Marta E Alarcón-Riquelme
In an effort to identify rare alleles associated with SLE, we have performed whole exome sequencing of the most distantly related affected individuals from two large Icelandic multicase SLE families followed by Ta targeted genotyping of additional relatives. We identified multiple rare likely pathogenic variants in nineteen genes co-segregating with the disease through multiple generations. Gene co-expression and protein-protein interaction analysis identified a network of highly connected genes comprising several loci previously implicated in autoimmune diseases...
June 8, 2018: Scientific Reports
Kevin R Green, Jarelys M Hernandez-Jimenez, Carmen Liliana Isache, Rafik Jacob
A 33-year-old woman with history of HIV presented with 4 months of gradually progressing right hip pain and was found to have avascular necrosis (AVN) of both femoral heads. She had no other risk factors for AVN including sickle cell disease, systemic lupus erythematosus, prolonged steroid used or trauma. She initially failed conservative management and ultimately had bilateral hip core decompressions. After decompression therapy, the left femoral head collapsed and patient underwent a left total hip arthroplasty...
June 6, 2018: BMJ Case Reports
Rafael Pacheco-Costa, Hannah M Davis, Emily G Atkinson, Julian E Dilley, Innocent Byiringiro, Mohammad W Aref, Matthew R Allen, Teresita Bellido, Lilian I Plotkin
Aging is accompanied by imbalanced bone remodeling, elevated osteocyte apoptosis, and decreased bone mass and mechanical properties; and improved pharmacologic approaches to counteract bone deterioration with aging are needed. We examined herein the effect of mefloquine, a drug used to treat malaria and systemic lupus erythematosus and shown to ameliorate bone loss in glucocorticoid-treated patients, on bone mass and mechanical properties in young and old mice. Young 3.5-month-old and old 21-month-old female C57BL/6 mice received daily injections of 5 mg/kg/day mefloquine for 14 days...
June 4, 2018: Bone
Jared S Kahn, Sandhya Chowdary Deverapalli, David M Rosmarin
BACKGROUND: Discoid lupus erythematosus (DLE) and dermatomyositis (DM) are inflammatory autoimmune diseases that manifest primarily in the skin but can be linked to systemic complications. Although there is an in-depth understanding of the clinical presentation of these two diseases, much less is known regarding the pathophysiology. This has limited the effective treatment options for patients. OBJECTIVE: An understanding of the pathogenesis of each disease in greater detail will lead to newer targeted medications with less morbidity...
June 5, 2018: International Journal of Dermatology
Andrew J Monteith, Heather A Vincent, SunAh Kang, Patrick Li, Tauris M Claiborne, Zenon Rajfur, Ken Jacobson, Nathaniel J Moorman, Barbara J Vilen
Lysosomes maintain immune homeostasis through the degradation of phagocytosed apoptotic debris; however, the signaling events regulating lysosomal maturation remain undefined. In this study, we show that lysosome acidification, key to the maturation process, relies on mTOR complex 2 (mTORC2), activation of caspase-1, and cleavage of Rab39a. Mechanistically, the localization of cofilin to the phagosome recruits caspase-11, which results in the localized activation of caspase-1. Caspase-1 subsequently cleaves Rab39a on the phagosomal membrane, promoting lysosome acidification...
June 4, 2018: Journal of Immunology: Official Journal of the American Association of Immunologists
Evangelos Theodorou, Adrianos Nezos, Eleni Antypa, Dimitrios Ioakeimidis, Michael Koutsilieris, Maria Tektonidou, Haralampos M Moutsopoulos, Clio P Mavragani
BACKGROUND: Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease with an increased atherosclerotic risk compared to healthy population, partially explained by traditional cardiovascular (CV) risk factors. Recent data suggest B-cell activating factor (BAFF) as an important contributor in the pathogenesis of both SLE and atherosclerosis. The aim of the current study is to explore whether serum BAFF levels along with variants of the BAFF gene increase lupus related atherosclerotic risk...
May 30, 2018: Journal of Autoimmunity
N Tanaka, Y Kunihiro, M Kubo, R Kawano, K Oishi, K Ueda, T Gondo
AIM: To identify characteristic high-resolution computed tomography (CT) findings for individual collagen vascular disease (CVD)-related interstitial pneumonias (IPs). MATERIALS AND METHODS: The HRCT findings of 187 patients with CVD, including 55 patients with rheumatoid arthritis (RA), 50 with systemic sclerosis (SSc), 46 with polymyositis/dermatomyositis (PM/DM), 15 with mixed connective tissue disease, 11 with primary Sjögren's syndrome, and 10 with systemic lupus erythematosus, were evaluated...
May 29, 2018: Clinical Radiology
Atakan Tanacan, Gokcen Orgul, Ahmet Bülent Dogrul, Fatih Aktoz, Osman Abbasoglu, M Sinan Beksac
A 26-year-old primigravid patient, at 35 weeks and 2 days of gestation, was referred to Hacettepe University Hospital for pancreatic mass, giant cervical myoma, maternal systemic lupus erythematosus, thrombocytopenia, and onset of preterm labor. At 36 weeks and 1 day of gestation (6 days after admission to the hospital), regular uterine contractions started and cervical dilatation with effacement was observed. Because of breech presentation and giant cervical myoma, a cesarean section was performed on the primigravid patient under general anesthesia...
2018: Case Reports in Obstetrics and Gynecology
Antonio Julià, Francisco Javier López-Longo, José J Pérez Venegas, Silvia Bonàs-Guarch, Àlex Olivé, José Luís Andreu, Mª Ángeles Aguirre-Zamorano, Paloma Vela, Joan M Nolla, José Luís Marenco de la Fuente, Antonio Zea, José María Pego-Reigosa, Mercedes Freire, Elvira Díez, Esther Rodríguez-Almaraz, Patricia Carreira, Ricardo Blanco, Víctor Martínez Taboada, María López-Lasanta, Mireia López Corbeto, Josep M Mercader, David Torrents, Devin Absher, Sara Marsal, Antonio Fernández-Nebro
BACKGROUND: Systemic lupus erythematosus (SLE) is a common systemic autoimmune disease with a complex genetic inheritance. Genome-wide association studies (GWAS) have significantly increased the number of significant loci associated with SLE risk. To date, however, established loci account for less than 30% of the disease heritability and additional risk variants have yet to be identified. Here we performed a GWAS followed by a meta-analysis to identify new genome-wide significant loci for SLE...
May 30, 2018: Arthritis Research & Therapy
N-S Lai, H-C Yu, K-Y Huang, C-H Tung, H-B Huang, M-C Lu
Objective To investigate whether the aberrant expression of non-coding RNAs (ncRNAs) in T cells from patients with systemic lupus erythematosus (SLE) could contribute to the pathogenesis of lupus. Methods Expression profiles of RNA transcripts in T cells from three patients with SLE and three controls were analyzed by microarray analysis. Potentially aberrant-expressed ncRNAs were validated using T cell samples from 23 patients with SLE and 17 controls. Transfection studies and microarray analyses were conducted to search for any gene expression that is regulated by specific ncRNAs...
January 1, 2018: Lupus
K Suzuki, M Miyamoto, T Miyamoto, T Matsubara, Y Inoue, M Iijima, S Mizuno, J Horie, K Hirata, T Shimizu, T Kanbayashi
Objective Involvement of the hypothalamus is rare in patients with systemic lupus erythematosus (SLE). In this study, we measured cerebrospinal fluid (CSF) orexin-A levels in SLE patients with hypothalamic lesions to investigate whether the orexin system plays a role in SLE patients with hypothalamic lesions who present with excessive daytime sleepiness (EDS). Methods Orexin-A levels were measured in CSF from four patients with SLE who presented with hypothalamic lesions detected by MRI. Three patients underwent repeated CSF testing...
January 1, 2018: Lupus
Patricia Jordán-González, Lee Ming Shum, Lorena González-Sepúlveda, Luis M Vilá
Objective: Infections are a major cause of morbidity and mortality in systemic lupus erythematosus. Clinical outcomes of systemic lupus erythematosus patients hospitalized due to infections vary among different ethnic populations. Thus, we determined the outcomes and associated factors in a group of Hispanics from Puerto Rico with systemic lupus erythematosus admitted due to severe infections. Methods: Records of systemic lupus erythematosus patients admitted to the Adult University Hospital, San Juan, Puerto Rico, from January 2006 to December 2014 were examined...
2018: SAGE Open Medicine
Aisha M Dahir, Simon F Thomsen
Vitiligo is a common skin disorder characterized by idiopathic, progressive cutaneous hypomelanosis. Vitiligo is associated with several comorbid autoimmune, systemic, and dermatological diseases, primarily thyroid disease, alopecia areata, diabetes mellitus, pernicious anemia, systemic lupus erythematosus, rheumatoid arthritis, Addison's disease, inflammatory bowel disease, Sjögren's syndrome, dermatomyositis, scleroderma, ocular and audiological abnormalities, psoriasis, and atopic dermatitis. It is essential to increase awareness of these comorbidities in order to improve the disease burden and quality of life of patients with vitiligo...
May 28, 2018: International Journal of Dermatology
A Doria, D Bass, A Schwarting, A Hammer, D Gordon, M Scheinberg, N L Fox, J Groark, W Stohl, C Kleoudis, D Roth
Objective To evaluate the safety, tolerability and efficacy of subcutaneous (SC) belimumab in patients with systemic lupus erythematosus (SLE) beyond 1 year. Methods This was a 24-week, open-label extension following a 52-week, double-blind, placebo-controlled trial of belimumab SC. Patients who completed the double-blind phase were eligible to enter the open-label phase. All patients received weekly belimumab 200 mg SC plus standard SLE therapy. Outcome measures included safety and efficacy (SLE Response Index (SRI) and SLE Flare Index (SFI) rates), and changes in biomarker and B cell levels...
January 1, 2018: Lupus
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