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    Gastrointestinal system manifestations in juvenile systemic lupus erythematosus.
    Clin Rheumatol 2017 Feb 16. Epub 2017 Feb 16.
    Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Sıhhiye, 06100, Ankara, Turkey.
    Systemic lupus erythematosus (SLE) is an autoimmune disease which may involve gastrointestinal system (GIS). The aim of this study was to present GIS manifestations of pediatric SLE patients. The medical files of 69 children with SLE followed between January 2011 and January 2016 were reviewed. Read More

    Low Level of Haptoglobin in Lupus.
    J Investig Med High Impact Case Rep 2017 Jan-Mar;5(1):2324709616689106. Epub 2017 Jan 1.
    Johns Hopkins University, Baltimore, MD, USA.
    Haptoglobin levels are measured in systematic lupus erythematosus patients as part of the workup for anemia, with low levels indicating hemolysis. Haptoglobin is an acute phase protein. We present 2 lupus patients who were found to have low haptoglobin levels in the absence of other evidence of hemolysis. Read More

    [Systemic lupus erythematosus presenting as severe alveolar hypoventilation and the shrinking lung syndrome].
    Rev Mal Respir 2017 Feb 8. Epub 2017 Feb 8.
    Service des maladies respiratoires, unité Inserm 903, hôpital Maison-Blanche, CHU de Reims, 45, rue Cognacq-Jay, 51092 Reims cedex, France. Electronic address:
    Introduction: The shrinking lung syndrome (SLS) is a rare complication of systemic lupus erythematosus.

    Case Report: A 69-year-old man presented with exertional dyspnoea, muscle weakness, and weight loss of 15kg in 6months. Pulmonary function tests revealed a restrictive lung disorder, with a dramatic decrease in maximal inspiratory pressure (17% of theoretical value), and alveolar hypoventilation (pH 7. Read More

    Cardiovascular magnetic resonance imaging pattern at the time of diagnosis of treatment naïve patients with connective tissue diseases.
    Int J Cardiol 2017 Feb 1. Epub 2017 Feb 1.
    Onassis Cardiac Surgery Center, Athens, Greece.
    Background-aim: Cardiac involvement at diagnosis of connective tissue disease (CTD) has been described by echocardiography. We hypothesized that cardio-vascular magnetic resonance (CMR) detects occult lesions at CTD diagnosis.

    Patients-methods: CMR was performed early after diagnosis in 78 treatment-naïve CTDs (aged 43±11, 59F/19M) without cardiac involvement [5 Takayasu arteritis (TA), 4 Churg Strauss syndrome (CSS), 5 Wegener granulomatosis (WG), 16 systemic lupus erythematosus (SLE), 12 rheumatoid arthritis (RA), 8 mixed connective tissue diseases (MCTD), 12 ankylosing spondylitis (AS), 3 polymyalgia rheumatica (PMR), 8 systemic sclerosis (SSc) and 5 dermatomyositis (DM)]. Read More

    Pathways of impending disease flare in African-American systemic lupus erythematosus patients.
    J Autoimmun 2017 Feb 2. Epub 2017 Feb 2.
    Arthritis and Clinical Immunology, Oklahoma Medical Research Foundation, 825 NE 13th Street, Oklahoma City, OK, 73104, USA; Departments of Medicine and Pathology, Oklahoma Clinical and Translational Science Institute, University of Oklahoma Health Sciences Center, 940 Stanton L. Young Boulevard, BMSB 451, Oklahoma City, OK, 73104, USA. Electronic address:
    Immune dysregulation in systemic lupus erythematosus (SLE) contributes to increased disease activity. African-American (AA) SLE patients have an increased prevalence of complications from disease flares and end-organ damage that leads to increased morbidity and early mortality. We previously reported alterations in inflammatory and regulatory immune mediator levels prior to disease flare in European American (EA) SLE patients. Read More

    Association of Therapy for Autoimmune Disease With Myelodysplastic Syndromes and Acute Myeloid Leukemia.
    JAMA Oncol 2017 Feb 2. Epub 2017 Feb 2.
    Department of Hematology and Oncology, Mayo Clinic, Phoenix, Arizona.
    Importance: Therapy-related myeloid neoplasms are a potentially life-threatening consequence of treatment for autoimmune disease (AID) and an emerging clinical phenomenon.

    Objective: To query the association of cytotoxic, anti-inflammatory, and immunomodulating agents to treat patients with AID with the risk for developing myeloid neoplasm.

    Design, Setting, And Participants: This retrospective case-control study and medical record review included 40 011 patients with an International Classification of Diseases, Ninth Revision, coded diagnosis of primary AID who were seen at 2 centers from January 1, 2004, to December 31, 2014; of these, 311 patients had a concomitant coded diagnosis of myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML). Read More

    Papulonodular mucinosis, Guillain-Barré syndrome and nephrotic syndrome in a patient with systemic lupus erythematosus: a case report.
    BMC Nephrol 2017 Feb 1;18(1):43. Epub 2017 Feb 1.
    Renal Division, Shanxi Medical University Second Hospital, Shanxi Kidney Disease Institute, No.382, Wuyi Road, Xinghualing Distirct, Taiyuan, Shanxi Province, China.
    Background: Awareness of the spectrum of clinical manifestations of systemic lupus erythematosus (SLE), especially uncommon changes, is essential for diagnosis and effective management of patients.

    Case Presentation: A 26-year-old Chinese man with SLE initially manifested cutaneous papulonodular mucinosis and developed acute Guillain-Barré syndrome and class V lupus nephritis 2 years later. His cutaneous nodules had not been idententified for 2 years and were resected by surgical procedures twice until SLE was diagnosed. Read More

    Survival and outcomes after lung transplantation for non-scleroderma connective tissue-related interstitial lung disease.
    J Heart Lung Transplant 2016 Dec 31. Epub 2016 Dec 31.
    Division of Pulmonary and Critical Care, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania; Division of Rheumatology, Brigham and Women's Hospital, Boston, Massachusetts. Electronic address:
    Background: Patients with non-scleroderma connective tissue-related lung disease (NS-CTLD), including rheumatoid arthritis, idiopathic inflammatory myopathies, Sjögren syndrome, mixed connective tissue disease, and systemic lupus erythematosus, may be at risk for worse outcomes after lung transplantation because of immune dysregulation or extrapulmonary manifestations of their underlying disease. We compared survival, acute and chronic rejection, and extrapulmonary organ dysfunction after transplantation in patients with NS-CTLD and idiopathic pulmonary fibrosis (IPF).

    Methods: This was a retrospective cohort study of patients with NS-CTLD and IPF who were listed in the Scientific Registry of Transplant Recipients and underwent lung transplantation from May 5, 2005, to March 1, 2016. Read More

    Neuroimaging features in posterior reversible encephalopathy syndrome: A pictorial review.
    J Neurol Sci 2017 Feb 8;373:188-200. Epub 2016 Dec 8.
    AP-HP, Pitié-Salpétrière Hospital, Department of Functional and Diagnostic Neuroradiology, F-75013 Paris, France. Electronic address:
    Posterior reversible encephalopathy syndrome (PRES) is a radioclinical entity associating nonspecific neurological symptoms (headache, seizures, impairment of alertness, visual disturbances…) occurring in evocative clinical condition (hypertension, eclampsia, immunosuppressor agents, systemic lupus erythematosus…). In the acute stage, the typical imaging finding is a vasogenic edema predominant in the subcortical parietal-occipital white matter. The purpose of this pictorial review is to illustrate the different neuroimaging features of PRES and present key radiological elements to assert diagnosis. Read More

    Clinical applications of multi-parametric CMR in myocarditis and systemic inflammatory diseases.
    Int J Cardiovasc Imaging 2017 Jan 27. Epub 2017 Jan 27.
    North West Heart Centre, University Hospital of South Manchester, Manchester, UK.
    Cardiac magnetic resonance (CMR) has changed the management of suspected viral myocarditis by providing a 'positive' diagnostic test and has lead to new insights into myocardial involvement in systemic inflammatory conditions. In this review we analyse the use of CMR tissue characterisation techniques across the available studies including T2 weighted imaging, early gadolinium enhancement, late gadolinium enhancement, Lake Louise Criteria, T2 mapping, T1 mapping and extracellular volume assessment. We also discuss the use of multiparametric CMR in acute cardiac transplant rejection and a variety of inflammatory conditions such as sarcoidosis, systemic lupus erythrematous, rheumatoid arthritis and systemic sclerosis. Read More

    Effects of N-acetyl-seryl-asparyl-lysyl-proline on blood pressure, renal damage, and mortality in systemic lupus erythematosus.
    Physiol Rep 2017 Jan 26;5(2). Epub 2017 Jan 26.
    Hypertension and Vascular Research Division, Department of Internal Medicine, Henry Ford Hospital, Detroit, Michigan
    Systemic lupus erythematosus (SLE) is an autoimmune disease with a high prevalence of hypertension. NZBWF1 (SLE-Hyp) mice develop hypertension that can be prevented by modulating T cells. The peptide N-acetyl-seryl-aspartyl-lysyl-proline (Ac-SDKP) decreases renal damage and improves renal function in a model of SLE without hypertension (MRL/lpr). Read More

    From Childhood to Adulthood: The Trajectory of Damage in Patients with Childhood-Onset Systemic Lupus Erythematosus.
    Arthritis Care Res (Hoboken) 2017 Jan 24. Epub 2017 Jan 24.
    Objectives 1) To determine the longitudinal damage trajectory of patients with childhood-onset SLE (cSLE). 2) To identify baseline and disease course predictors of damage trajectory. Methods This is a retrospective inception cohort. Read More

    Efficacy and comparison of antimalarials in cutaneous lupus erythematosus subtypes: a systematic review and meta-analysis.
    Br J Dermatol 2017 Jan 23. Epub 2017 Jan 23.
    Service de rhumatologie, Hôpitaux Universitaires de Strasbourg, Laboratoire d'ImmunoRhumatologie Moléculaire, INSERM UMR_S1109, Université de Strasbourg, F-67000, Strasbourg, France.
    Background: The antimalarials (AMs) hydroxychloroquine (HCQ) or chloroquine (CQ) have demonstrated variable cutaneous response rates in Cutaneous Lupus Erythematosus (CLE).

    Objectives: We sought to assess the global cutaneous response rate to HCQ and CQ, with respect to CLE subtypes, based on previously published studies.

    Methods: We performed a systematic review and meta-analysis of studies published in MEDLINE, Embase, Cochrane library between 1965 and December 2015. Read More

    Association between ischemic heart disease and systemic lupus erythematosus-a large case-control study.
    Immunol Res 2017 Jan 17. Epub 2017 Jan 17.
    Department of Medicine 'B', Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel-Hashomer, Israel, Sackler Faculty of Medicine, Tel-Aviv University, 52621, Tel-Hashomer, Israel.
    Ischemic heart disease (IHD) is a well identified cause of mortality in systemic lupus erythematosus (SLE) patients due to an accelerated premature atherosclerosis. We investigated the proportion of comorbid IHD among SLE patients derived from a large, national real-life database. Using data from the largest HMO in Israel, the Clalit Health Services, we selected for patients with SLE. Read More

    The spectrum of rheumatic in-patient diagnoses at a pediatric hospital in Kenya.
    Pediatr Rheumatol Online J 2017 Jan 14;15(1). Epub 2017 Jan 14.
    Division of Pediatric Rheumatology, Department of Pediatrics, McGill University Health Centre, 1001 Decarie Boulevard, Room A04.6306, Montreal, QC, Canada, H4A 3J1.
    Background: Pediatric rheumatic diseases are chronic illnesses that can cause considerable disease burden to children and their families. There is limited epidemiologic data on these diseases in East Africa. The aim of this study was to assess the spectrum of pediatric rheumatic diagnoses in an in-patient setting and determine the accuracy of ICD-10 codes in identifying these conditions. Read More

    Acute necrotizing retinal vasculitis as onset of systemic lupus erythematosus: A case report.
    Medicine (Baltimore) 2017 Jan;96(2):e5754
    aClinic of Rheumatology, University Hospital "St. Iv. Rilski," Department of Internal Medicine bClinic of Ophthalmology, University Hospital "TsaritsaYoanna," cOphthalmology Outpatient Clinic, University Hospital "St. Iv. Rilski," dClinic of ophthalmology, University Hospital "Alexandrovska," eDepartment of Internal Medicine, Medical Institute-MID, Medical University, Sofia, Bulgaria.
    Rationale: Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by autoantibody production, complement activation, and deposition of immune complexes in tissues and organs. SLE can involve any region of the visual system. Although ocular manifestations are not part of the classification criteria for SLE, they can be observed in up to one-third of the patients with SLE. Read More

    Successful treatment of donor-derived hepatitis C viral infection in three transplant recipients from a donor at increased risk for bloodborne pathogens.
    Transpl Infect Dis 2017 Jan 6. Epub 2017 Jan 6.
    Division of Gastroenterology and Hepatology, Thomas Jefferson University, Philadelphia, PA, USA.
    We report here the successful treatment of hepatitis C virus (HCV) transmitted from a nucleic acid testing (NAT)-negative donor to 3 HCV-negative recipients - 2 renal transplants and 1 liver. Both renal recipients underwent standard deceased-donor renal transplantation with immediate graft function. The liver recipient underwent standard orthotopic liver transplantation and recovered uneventfully. Read More

    Acute lupus choroidopathy: multimodal imaging and differential diagnosis from central serous chorioretinopathy.
    Int Ophthalmol 2017 Jan 3. Epub 2017 Jan 3.
    Department of Ophthalmology, School of Medicine, Gazi University, Ankara, Turkey.
    Importance: Systemic lupus erythematosus (SLE) is a thoroughly examined multi-organ disease whose common ocular complications are also well documented. However, SLE choroidopathy, being a rare ocular complication, is needed to be better understood, in order to make the differential diagnosis from clinical conditions such as central serous chorioretinopathy and provide adequate prompt treatment.

    Objective: To provide a clear understanding for the clinical course of SLE choroidopathy by the help of enhanced depth imaging optical coherence tomography, fluorescein angiography, and indocyanine angiography. Read More

    Brain histopathology in patients with systemic lupus erythematosus: identification of lesions associated with clinical neuropsychiatric lupus syndromes and the role of complement.
    Rheumatology (Oxford) 2017 Jan 25;56(1):77-86. Epub 2016 Oct 25.
    Department of Pathology.
    Objectives: Neuropsychiatric (NP) involvement is a poorly understood manifestation of SLE. We studied post-mortem histopathology in relation to clinical NPSLE syndromes and complement deposition in brains of NPSLE and SLE patients and controls. Furthermore, we investigated the correlation between cerebral post-mortem histopathology and ex vivo 7 T MRI findings in SLE and NPSLE. Read More

    Hydroxychloroquine for the prevention of recurrent cardiovascular events in myocardial infarction patients: rationale and design of the OXI trial.
    Eur Heart J Cardiovasc Pharmacother 2016 Nov 8. Epub 2016 Nov 8.
    Heart and Lung Center, University of Helsinki and Helsinki University Hospital, PL 340, 00029 HUS, Finland
    Background: Inflammation of the arterial wall plays a central role in the pathogenesis of atherosclerosis. Among patients with rheumatic diseases, anti-rheumatic medication reduces the incidence of cardiovascular (CV) diseases, but only few studies have addressed their cardioprotective effects on patients with no rheumatic diseases. Hydroxychloroquine (HCQ) is an anti-rheumatic drug commonly used in the treatment of rheumatoid arthritis and systemic lupus erythematosus. Read More

    Comorbid Conditions are Associated With Emergency Department Visits, Hospitalizations, and Medical Charges of Patients With Systemic Lupus Erythematosus.
    J Clin Rheumatol 2017 Jan;23(1):19-25
    From the *Wuxi Medical School, Jiangnan University, Wuxi, Jiangsu, PR China; †Nebraska Department of Health and Human Services, Lincoln, NE; and ‡Department of Epidemiology, University of Nebraska Medical Center, Omaha, NE.
    Background/objectives: In addition to increase mortality, comorbidities can increase medical costs for systemic lupus erythematosus (SLE). Healthcare utilization can dramatically increase medical costs. It is essential to better understand the comorbidities that can lead to healthcare utilization, such as emergency department visit and/or hospitalization, for SLE patients. Read More

    Alveolar hemorrhage as the initial presentation of systemic lupus erythematosus.
    Reumatologia 2016 28;54(5):264-266. Epub 2016 Nov 28.
    Universidade Federal de Pelotas, Pelotas, Brazil.
    Alveolar hemorrhage (AH) is a rare syndrome that can often occur in autoimmune diseases, blood clotting disorders, infection or by acute inhalation injury, presenting rapid evolution and high mortality, especially with late diagnosis and treatment. Among the autoimmune diseases, there are reported cases in patients with primary antiphospholipid syndrome (PAPS), vasculitis and systemic lupus erythematosus (SLE). An early diagnosis is an essential tool in the successful management of this complication, requiring aggressive treatment based on vigorous immunosuppression and broad-spectrum antibiotic. Read More

    Longitudinal Patterns of Response to Standard of Care Therapy for Systemic Lupus Erythematosus: Implications for Clinical Trial Design.
    Arthritis Rheumatol 2016 Dec 19. Epub 2016 Dec 19.
    New York University School of Med, USA.
    Objective: To evaluate longitudinal patterns of response to standard of care (SOC) therapy in clinical trials for systemic lupus erythematosus (SLE) and identify characteristics that differentiate non-responders from persistent responders.

    Methods: Data were obtained from the Collective Data Analysis Initiative of the Lupus Foundation of America on 147 patients treated with placebo plus SOC in two 52-week Phase II/III trials in moderately-to-severely active SLE patients without acute nephritis. Cross-sectional and longitudinal analyses were performed of BILAG-based responses (improvement in all baseline A/B without new flare). Read More

    The role of clinically significant antiphospholipid antibodies in systemic lupus erythematosus.
    Reumatismo 2016 Dec 16;68(3):137-143. Epub 2016 Dec 16.
    Rheumatology and Clinical Immunology, Spedali Civili of Brescia.
    The objective is to investigate the role of clinically significant antiphospholipid antibodies (aPL) in a cohort of systemic lupus erythematosus (SLE) patients. All SLE patients followed for at least 5 years and with available aPL profile at the beginning of the follow-up in our center were studied. Clinically significant aPL were defined as: positive lupus anticoagulant test, anti-cardiolipin and/or anti- β2Glycoprotein I IgG/IgM >99th percentile on two or more occasions at least 12 weeks apart. Read More

    Cord Blood Transplantation Following Reduced-Intensity Conditioning for Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis during Systemic Lupus Erythematosus Treatment.
    J Clin Exp Hematop 2016 ;56(2):126-129
    Department of Internal Medicine, Miyazaki Prefectural Miyazaki Hospital.
    Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a serious disorder in which monoclonal growth of T cells infected with EBV and macrophage activation cause pancytopenia, high fever and acute liver failure. Patients with chemotherapy- or immunosuppression-resistant EBV-HLH require allogeneic hematopoietic stem cell transplantation (allo-HSCT), but patients who have no sibling donors may not have time to wait for an unrelated donor. In pediatric patients, there are some reports on allogeneic cord blood transplantation (allo-CBT) for the treatment of EBV-HLH; however, in adult patients, reports of allo-CBT for EBV-HLH are quite limited. Read More

    Effect of donor STAT4 polymorphism rs7574865 on clinical outcomes of pediatric acute leukemia patients after hematopoietic stem cell transplant.
    Int Immunopharmacol 2017 Feb 10;43:62-69. Epub 2016 Dec 10.
    Department of Pediatrics, Seoul National University College of Medicine, Seoul, Republic of Korea; Cancer Research Institute, Seoul National University College of Medicine, Seoul, Republic of Korea. Electronic address:
    STAT4 polymorphism, rs7574865 is linked to various autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis. Its T minor allele is associated with higher STAT4 mRNA and protein expression, indicating a stronger skewed immune response than the norm. Although widely studied in autoimmune disease patients and the general population, its effect on immunocompromised subjects is still unknown. Read More

    Abnormal hematopoiesis and autoimmunity in human subjects with germline IKZF1 mutations.
    J Allergy Clin Immunol 2016 Dec 1. Epub 2016 Dec 1.
    Department of Pediatrics and Developmental Biology, Perinatal and Maternal Medicine, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), Tokyo, Japan. Electronic address:
    Background: Ikaros, which is encoded by IKZF1, is a transcriptional factor that play a critical role in hematopoiesis. Somatic IKZF1 alterations are known to be involved in the pathogenesis of leukemia in human subjects. Recently, immunodeficiency caused by germline IKZF1 mutation has been described. Read More

    What is the role of hydroxychloroquine in reducing thrombotic risk in patients with antiphospholipid antibodies?
    Hematology Am Soc Hematol Educ Program 2016 Dec;2016(1):714-716
    Department of Medicine, McMaster University, Hamilton, ON, Canada.
    A 35-year-old man presents with an acute unprovoked deep vein thrombosis of the left lower extremity. He is treated with anticoagulation and elects to discontinue treatment after 6 months. He subsequently develops polyarthralgias, fatigue, and a malar rash, and a diagnosis of systemic lupus erythematosus is made based on laboratory and clinical findings. Read More

    Progression of noncalcified and calcified coronary plaque by CT angiography in SLE.
    Rheumatol Int 2017 Jan 23;37(1):59-65. Epub 2016 Nov 23.
    Division of Rheumatology, Johns Hopkins University School of Medicine, 1830 E. Monument Street Suite 7500, Baltimore, MD, 21205, USA.
    Premature coronary artery disease remains the major cause of late death in systemic lupus erythematosus (SLE). Coronary artery calcium (CAC) represents an advanced stage of atherosclerosis, whereas noncalcified coronary atherosclerotic plaque (NCP) typically is more prone to trigger acute coronary events. The aim of this study was to assess the stability of NCP over time and identify factors associated with changes in NCP in patients with SLE. Read More

    RORγt expression in Tregs promotes systemic lupus erythematosus via IL-17 secretion, alteration of Treg phenotype and suppression of Th2 responses.
    Clin Exp Immunol 2016 Nov 23. Epub 2016 Nov 23.
    III Medizinische Klinik, Universitätsklinikum Eppendorf, Hamburg, Germany.
    Systemic lupus erythematosus (SLE) is a common autoimmune disorder with a complex and poorly understood immunopathogenesis. However, a pathogenic role for the T helper type 17 (Th17) axis was demonstrated by many studies, while regulatory T cells (Tregs ) were shown to mediate protection. Recently, we and others characterized a novel and independent T cell population expressing both the Treg characteristic transcription factor forkhead box protein 3 (FoxP3) and the Th17-defining retinoic acid receptor-related orphan nuclear receptor γt (RORγt). Read More

    Lupus nephritis in children - 10 years' experience.
    Cent Eur J Immunol 2016 25;41(3):248-254. Epub 2016 Oct 25.
    Department of Pediatrics and Nephrology, Medical University of Warsaw, Poland.
    Systemic lupus erythematosus (SLE) in children is usually more severe than it is in adults and there is a higher incidence of renal involvement. We described 18 children (16 girls, 2 boys) with lupus nephritis (LN), whose average age was 14.4 ±1. Read More

    Fulminant Hepatic Failure Secondary to Herpes Hepatitis in a Patient With Myasthenia Crisis: An Elusive Diagnosis.
    J Clin Med Res 2016 Dec 26;8(12):921-924. Epub 2016 Oct 26.
    Department of Medicine, Bronx Lebanon Hospital Center, 1650 Selwyn Ave, Suite #10C, Bronx, NY 10457, USA.
    Herpes hepatitis is a rare cause of fulminant hepatic failure contributing to less than 1% of all cases. It is most often seen in persons who are immunosuppressed and in pregnant women. The presentation is usually non-specific and rapidly progressive, thus making antemortem diagnosis of this condition rare. Read More

    Sudden death in infective endocarditis.
    Autops Case Rep 2016 Jul-Sep;6(3):17-22. Epub 2016 Sep 30.
    Infectious Disease Control Department - Instituto do Coração - Faculty of Medicine - Universidade de São Paulo, São Paulo/SP - Brazil .
    The case fatality rate of infective endocarditis (IE) is high and is associated with varying causes. Among them, acute myocardial infarction due to an embolism in a coronary artery is rare; the incidence of this complication in the setting of IE is reported to be up to 1.5%. Read More

    C1q and HMGB1 reciprocally regulate human macrophage polarization.
    Blood 2016 Nov 28;128(18):2218-2228. Epub 2016 Sep 28.
    Center for Autoimmune and Musculoskeletal Diseases, The Feinstein Institute for Medical Research, Manhasset, NY.
    A healthy immune system results from a balance of stimulatory and inhibitory pathways that allow effective responses to acute insults, without descending into chronic inflammation. Failed homeostasis is characteristic of autoimmune diseases such as systemic lupus erythematosus. Although HMGB1 induces proinflammatory M1-like macrophage differentiation, we describe a mechanism by which C1q modulates this activity and collaborates with HMGB1 to induce the differentiation of monocytes to anti-inflammatory M2-like macrophages. Read More

    Systemic lupus erythematous revealed by cytomegalovirus infection.
    Pan Afr Med J 2016 15;24:241. Epub 2016 Jul 15.
    Internal Medicine Department, CHU Sahloul, Tunisia.
    Cytomegalovirus (CMV) infection have been described as exacerbing systemic lupus erythematous (SLE). The role of CMV in starting off SLE remains object of debate. We report a severe presentation of SLE revealed by CMV infection with hemophogocytic syndrome. Read More

    mTOR has distinct functions in generating versus sustaining humoral immunity.
    J Clin Invest 2016 Nov 17;126(11):4250-4261. Epub 2016 Oct 17.
    Little is known about the role of mTOR signaling in plasma cell differentiation and function. Furthermore, for reasons not understood, mTOR inhibition reverses antibody-associated disease in a murine model of systemic lupus erythematosus. Here, we have demonstrated that induced B lineage-specific deletion of the gene encoding RAPTOR, an essential signaling adaptor for rapamycin-sensitive mTOR complex 1 (mTORC1), abrogated the generation of antibody-secreting plasma cells in mice. Read More

    Autoimmune gastrointestinal complications in patients with systemic lupus erythematosus: case series and literature review.
    Lupus 2016 Dec 20;25(14):1509-1519. Epub 2016 Jun 20.
    Centre for Rheumatology, Department of Medicine, University College London, London, UK
    The association of systemic lupus erythematosus (SLE) with gastrointestinal autoimmune diseases is rare, but has been described in the literature, mostly as case reports. However, some of these diseases may be very severe, thus a correct and early diagnosis with appropriate management are fundamental. We have analysed our data from the SLE patient cohort at University College Hospital London, established in 1978, identifying those patients with an associated autoimmune gastrointestinal disease. Read More

    Progressive outer retinal necrosis syndrome in the course of systemic lupus erythematosus.
    Lupus 2016 Dec 13;25(14):1610-1614. Epub 2016 May 13.
    Department of General and Interventional Radiology and Neuroradiology, Wrocław Medical University, Wroclaw, Poland.
    Progressive outer retinal necrosis syndrome (PORN) is a severe clinical variant of necrotizing herpetic chorioretinitis, which occurs almost exclusively in patients with advanced acquired immunodeficiency syndrome (AIDS). To date, only a few cases of PORN have been reported in patients, mostly among those who were immunocompromised. To our knowledge, only one case of PORN in a patient with systemic lupus erythematosus (SLE) has been described. Read More

    Brief Report: Trends in Hospitalizations Due to Acute Coronary Syndromes and Stroke in Patients With Systemic Lupus Erythematosus, 1996 to 2012.
    Arthritis Rheumatol 2016 Nov;68(11):2680-2685
    NIH, Bethesda, Maryland.
    Objective: Cardiovascular disease (CVD) has been recognized as a major cause of morbidity in patients with systemic lupus erythematosus (SLE), but it is not clear whether increased awareness of these risks has translated into improvements in CVD morbidity at the population level. The aim of this study was to examine trends in hospitalization rates for CVD events in a representative sample of adult patients with SLE in the US from 1996 to 2012.

    Methods: We used the Nationwide Inpatient Sample to estimate the rates of hospitalization for acute myocardial infarction (MI), unstable angina, and ischemic stroke from 1996 to 2012 in patients with SLE. Read More

    Features of 847 Childhood-Onset Systemic Lupus Erythematosus Patients in Three Age Groups at Diagnosis: A Brazilian Multicenter Study.
    Arthritis Care Res (Hoboken) 2016 Nov 6;68(11):1736-1741. Epub 2016 Oct 6.
    Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Brazil.
    Objective: To evaluate demographic data and clinical and laboratory features at disease diagnosis in 3 different age groups of childhood-onset systemic lupus erythematosus (SLE): group A, early-onset (<6 years); group B, school age (≥6 to <12 years); and group C, adolescent (≥12 to <18 years).

    Methods: This was a Brazilian multicenter cohort retrospective study in 10 pediatric rheumatology centers, including 847 childhood-onset SLE patients.

    Results: Patients were divided into 3 groups: group A with 39 patients (4%), group B with 395 patients (47%), and group C with 413 patients (49%). Read More

    Acute Limb-Threatening Ischemia Associated With Antiphospholipid Syndrome: A Report of Two Cases.
    J Foot Ankle Surg 2016 Nov - Dec;55(6):1318-1322. Epub 2016 Feb 19.
    Professor, Department of Plastic and Reconstructive Surgery, Saitama Medical University, Saitama, Japan.
    Acute limb ischemia results from sudden deterioration in the arterial supply to the limb, occasionally leading to limb loss or fatality. Antiphospholipid syndrome (APS) is known to induce acute limb ischemia among the various etiologies responsible for arterial obstruction. APS is a systemic autoimmune disorder characterized by a combination of arterial and/or venous thrombosis and limb loss. Read More

    Role of galectin-3 in autoimmune and non-autoimmune nephropathies.
    Autoimmun Rev 2017 Jan 23;16(1):34-47. Epub 2016 Sep 23.
    Division of Rheumatology, Department of Medicine (DIMED), University of Padova, Italy. Electronic address:
    Galectins are evolutionary conserved β-galactoside binding proteins with a carbohydrate-recognition domain (CRD) of approximately 130 amino acids. In mammals, 15 members of the galectin family have been identified and classified into three subtypes according to CRD organization: prototype, tandem repeat-type and chimera-type galectins. Galectin-3 (gal-3) is the only chimera type galectin in vertebrates containing one CRD linked to an unusual long N-terminal domain which displays non-lectin dependent activities. Read More

    Mesenteric Vasculitis in a Case of Systemic Lupus Erythematosus.
    J Assoc Physicians India 2016 Jul;64(7):70-73
    Professor and Chief of Rheumatology, Department of Medicine, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra.
    We present a case of mesentric vasculitis with systemic lupus erythematosus who relapsed after high dose steroids but achieved subsequent remission after starting pulse cyclophosphamide therapy. 38 years old female who had earlier polyserositis and cerebral venous thrombosis was admitted with provisional diagnosis of SLE and developed acute abdominal pain during hospital stay. She was diagnosed as mesenteric vasculitis and initially responded to pulse methylprednisolone. Read More

    Application of SLICC classification criteria in undifferentiated connective tissue disease and evolution in systemic lupus erythematosus: analysis of a large monocentric cohort with a long-term follow-up.
    Lupus 2016 Oct 4. Epub 2016 Oct 4.
    Department of Medical Sciences, Rheumatology Unit, University of Ferrara and Azienda Ospedaliero-Universitaria Sant'Anna, Cona (Ferrara), Italy.
    Objectives: The objectives of this study were to analyse the performance of the Systemic Lupus International Collaborating Clinics (SLICC) 2012 classification criteria for systemic lupus erythematosus (SLE) in a large cohort of undifferentiated connective tissue disease (UCTD) population at onset of the disease and during a long-term follow-up of 15 years (1999-2013) and to evaluate the transition from UCTD to SLE, according to American College of Rheumatology (ACR) 1997 and SLICC 2012 classification criteria.

    Methods: A cohort of patients who met the classification criteria proposed by Mosca et al. for UCTD, were analysed. Read More

    Mapping urinary chemokines in human lupus nephritis: Potentially redundant pathways recruit CD4(+) and CD8(+) T cells and macrophages.
    Eur J Immunol 2017 Jan 21;47(1):180-192. Epub 2016 Nov 21.
    Department of Nephrology and Intensive Care Medicine, Charité Universitätsmedizin Berlin, Berlin, Germany.
    Renal infiltration of inflammatory cells contributes to the pathogenesis of lupus nephritis (LN). Current knowledge on the recruitment mechanisms relies mainly on findings in rodent models. Here, we assess various chemokine pathways in human LN by comparing urinary chemokine concentrations (in 25 patients with acute LN and in 78 lupus patients without active LN) with the expression of corresponding chemokine receptors on urinary leukocytes (in ten acute LN patients). Read More

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