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    Kidney transplantation for end-stage renal disease in lupus nephritis, a very safe procedure: a single Latin American transplant center experience.
    Lupus 2017 Jan 1:961203317696591. Epub 2017 Jan 1.
    2 ICESI University School of Medicine, Cali, Colombia.
    Background Lupus nephritis (LN) is one of the most frequent complications of SLE and occurs in up to 50% of cases depending on the studied population. Of these, approximately 20% progress to end-stage renal disease (ESRD), with the treatment of choice being a kidney transplant. Objective The objective of this study was to describe the clinical outcome of patients transplanted due to LN, compared with patients transplanted for other causes, in a Latin American population from the Fundación Valle del Lili in Cali, Colombia. Read More

    Serum C-reactive protein concentrations in Nova Scotia Duck Tolling Retrievers with immune-mediated rheumatic disease.
    Acta Vet Scand 2017 Apr 17;59(1):21. Epub 2017 Apr 17.
    Department of Clinical Sciences, Faculty of Veterinary Medicine and Animal Science, Swedish University of Agricultural Sciences, Box 7054, 750 07, Uppsala, Sweden.
    Nova Scotia Duck Tolling Retrievers (NSDTRs) are a dog breed often affected by immune-mediated rheumatic disease (IMRD), a disorder characterised by chronic stiffness and joint pain. Most, but not all, dogs with IMRD, have antinuclear antibodies (ANA), which are also commonly present in the autoimmune disease systemic lupus erythematosus (SLE). The clinical and diagnostic findings of IMRD indicate that it is an SLE-related disorder. Read More

    Use of SLICC criteria in a large, diverse lupus registry enables SLE classification of a subset of ACR-designated subjects with incomplete lupus.
    Lupus Sci Med 2017 17;4(1):e000176. Epub 2017 Mar 17.
    Department of Arthritis and Clinical Immunology, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA.
    Objective: SLE is traditionally classified using the American College of Rheumatology (ACR) criteria. The Systemic Lupus International Collaborating Clinics (SLICC) recently validated an alternative system. This study examined large cohorts of subjects with SLE and incomplete lupus erythematosus (ILE) to compare the impact of ACR and SLICC criteria. Read More

    Accidental hydroxychloroquine overdose resulting in neurotoxic vestibulopathy.
    BMJ Case Rep 2017 Apr 12;2017. Epub 2017 Apr 12.
    Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA
    Hydroxychloroquine is an oral antimalarial medication commonly used off-label for a variety of rheumatological conditions, including systemic lupus erythematosus, rheumatoid arthritis, Sjögren's syndrome and dermatomyositis. We present a case of a 64-year-old woman who presented with acute onset headache, bilateral tinnitus, and left-sided facial numbness and tingling in the setting of accidentally overdosing on hydroxychloroquine. By the next morning, the patient began to experience worsening in the tingling sensation and it eventually spread to her left arm, thigh and distal extremities. Read More

    Systemic lupus erythematosus: A possible cause of non-alcoholic Wernicke's encephalopathy.
    S Afr Med J 2017 Mar 29;107(4):299-301. Epub 2017 Mar 29.
    Division of Rheumatology, Department of Medicine, Faculty of Health Sciences, Sefako Makgatho Health Sciences University, Pretoria, South Africa.
    We report a young woman with systemic lupus erythematosus (SLE) and an acute cerebellar ataxia. A history of poor appetite and vomiting preceded the inco-ordination. Ataxia in SLE has been well described, but is nevertheless uncommon. Read More

    Anti-ribosomal P antibody: a multicenter study in childhood-onset systemic lupus erythematosus patients.
    Lupus 2017 Apr;26(5):484-489
    1 Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo (FMUSP), Brazil.
    Objectives Anti-ribosomal P protein (anti-P) autoantibodies are highly specific for systemic lupus erythematosus (SLE). However, the evaluation of this autoantibody in childhood-onset SLE (cSLE) populations has been limited to a few small series, hampering the interpretation of the clinical and laboratorial associations. Therefore, the objective of this multicenter cohort study was to evaluate demographic, clinical/laboratorial features, and disease damage score in cSLE patients with and without the presence of anti-P antibody. Read More

    A critical analysis of the tools to evaluate neuropsychiatric lupus.
    Lupus 2017 Apr;26(5):504-509
    1 Centre for Rheumatology, Division of Medicine, University College London, London, UK.
    Neuropsychiatric symptoms occur commonly in patients with systemic lupus erythematosus, but they are not always due to active disease. It is crucial to identify cases that are due to active systemic lupus erythematosus so that appropriate treatment can be instituted. There is no single serological or imaging test that distinguishes active neuropsychiatric systemic lupus erythematosus from neuropsychiatric manifestations caused by other factors such as infection. Read More

    Acute-Onset Chest Pain in a 17-Year-Old Female Adolescent With Systemic Lupus Erythematosus.
    Pediatr Emerg Care 2017 Apr 4. Epub 2017 Apr 4.
    From the Department of Pediatrics, Johns Hopkins School of Medicine, Baltimore, MD.
    We report the case of a 17-year-old adolescent girl with systemic lupus erythematosus with disseminated pneumococcal infection leading to purulent pericarditis with cardiac tamponade. Although pericarditis is not an uncommon entity in autoimmune diseases such as systemic lupus erythematosus, purulent pericarditis is a rare cause (<1%) of this presentation. Read More

    Acute acalculous cholecystitis in patients with systemic lupus erythematosus: A unique form of disease flare.
    Lupus 2017 Jan 1:961203317699288. Epub 2017 Jan 1.
    Department of Rheumatology and Clinical Immunology, Clinical Immunology Center, The Ministry of Education Key Laboratory, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
    Objective We aimed to investigate the clinical features of acute acalculous cholecystitis (AAC) in patients with systemic lupus erythematosus (SLE). Methods SLE patients with AAC hospitalized in the Peking Union Medical College Hospital (PUMCH) from January 2001 to September 2015 were retrospectively analyzed. Their medical records were systematically reviewed. Read More

    Mesenteric inflammatory veno-occlusive disease of the spleen metasynchronous with two arterial thrombotic events in systemic lupus erythematosus.
    Lupus 2017 Jan 1:961203317700980. Epub 2017 Jan 1.
    1 Immunology and Allergy, Department of Internal Medicine Specialties, University Hospital and School of Medicine, Geneva, Switzerland.
    Vasculitides, particularly those affecting small vessels, are known to complicate systemic lupus erythematosus (SLE); however, isolated venulitis of the mesenteric bed has rarely been reported. Here we relate the case of a 46-year-old woman with SLE who presented with acute abdominal pain due to artery thrombosis and extended splenic ischemia requiring splenectomy. The histological examination revealed diffuse venulitis in the absence of arterial vasculitis consistent with the definition of mesenteric inflammatory veno-occlusive disease (MIVOD). Read More

    Etiological profile of noncompressive myelopathies in a tertiary care hospital of Northeast India.
    Ann Indian Acad Neurol 2017 Jan-Mar;20(1):41-50
    Department of Neurology, Gauhati Medical College and Hospital, Guwahati, Assam, India.
    Background: The discovery of antibodies against aquaporin-4 and evolving concepts of noncompressive myelopathies in the 21(st) century have made a major impact on the etiological profile of these diseases, with few cases turning out to be idiopathic.

    Objective: To find causes of noncompressive myelopathy in a tertiary care hospital of Northeast India.

    Materials And Methods: An observational study was carried out in the Neurology Department of Gauhati Medical College, Guwahati, from September 2013 to February 2016. Read More

    A study of acute muscle dysfunction with particular reference to dengue myopathy.
    Ann Indian Acad Neurol 2017 Jan-Mar;20(1):13-22
    Department of Neurology, King George Medical University, Lucknow, Uttar Pradesh, India.
    Background: Acute myopathy is a common cause of acute motor quadriparesis which has various etiologies with different courses of illness and prognosis depending on the cause. Understanding this diversity helps us in proper approach toward diagnosis, predicting the prognosis, and possible complications and in improving the treatments that are being provided. This study was planned to study the clinical, electrophysiological, and etiological profile of patients presenting with acute myopathy. Read More

    Different genetic alteration of A20 in a Sézary syndrome case with Vα2-Jα22 T cell clone.
    Asia Pac J Clin Oncol 2017 Mar 14. Epub 2017 Mar 14.
    Key Laboratory for Regenerative Medicine of Ministry of Education, Jinan University, Guangzhou, China.
    Background: The comprehensive genetic alterations underlying the pathogenesis of Sézary syndrome (SS) remains largely unknown. Previous studies showed that alterations of tumor necrosis factor-α-induced protein 3 gene (TNFAIP3; A20) are frequent in SS. In this study, we characterized the mutation and polymorphisms of A20 in a case with SS and compared with the genetic feature of A20 in T-cell acute lymphoblastic leukemia (T-ALL). Read More

    Neurological Manifestations in Systemic Lupus Erythematosus: A Single Centre Study from North East India.
    J Clin Diagn Res 2017 Jan 1;11(1):OC05-OC09. Epub 2017 Jan 1.
    Assistant Professor, Department of Neurology, NEIGRIHMS , Shillong, Meghalaya, India .
    Introduction: Neurological manifestations although common in Systemic Lupus Erythematosus (SLE), are often not recognized due to their diversed and varied presentation. Therefore, the study was planned to highlight the pattern of neurological involvement in SLE to help in early recognition.

    Aim: To study the pattern of neurological involvement in SLE and its correlation with disease activity and different investigation. Read More

    Collapsing glomerulopathy in systemic lupus erythematosus.
    BMJ Case Rep 2017 Feb 27;2017. Epub 2017 Feb 27.
    Department of Nephrology and Hypertension, Mayo Clinic, Jacksonville, Florida, USA.
    Collapsing glomerulopathy (CG) is a rare disease that can be associated with multiple other disorders. It usually leads to poor prognosis with a high percentage of patients progressing to end-stage renal disease. In this article, we illustrate a clinical case of CG associated with systemic lupus erythematosus that had a prompt response to mycophenolate and prednisone. Read More

    Toxic epidermal necrolysis in a child with lupus-associated pancreatitis.
    Rheumatol Int 2017 Feb 26. Epub 2017 Feb 26.
    Department of Anesthesia, Faculty of Medicine, University of Belgrade, Tirsova 10, Belgrade, Serbia.
    Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening dermatological conditions that are characterized by mucosal erosions, epidermal detachments and erosions. The most common causes of SJS and TEN are drugs; other causes such as systemic lupus erythematosus (SLE), vaccinations and infections have been rarely implicated. We present the case of a 14-year-old female patient with acute pancreatitis as an initial manifestation of systemic lupus erythematosus, complicated by the toxic epidermal necrolysis with a fatal outcome. Read More

    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 Jun 1;236:151-156. 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 Mar 2;78:70-78. 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

    Transfusion-related acute lung injury in a patient with systemic lupus erythematosus.
    Clin Exp Rheumatol 2017 Mar-Apr;35(2):353. Epub 2017 Jan 27.
    Rheumatology Clinic, Department of Internal Medicine, Medical School, University of Ioannina, Greece.
    ****************************************************************************. 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 Apr 22;19(2). Epub 2017 Feb 22.
    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 three HCV-negative recipients-two renal transplants and one 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 2017 Apr;3(2):92-97
    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

    Brief Report: Longitudinal Patterns of Response to Standard of Care Therapy for Systemic Lupus Erythematosus: Implications for Clinical Trial Design.
    Arthritis Rheumatol 2017 Apr;69(4):785-790
    New York University School of Medicine, New York, New York.
    Objective: To evaluate longitudinal patterns of response to standard of care for systemic lupus erythematosus (SLE) in clinical trials and to identify characteristics that differentiate nonresponders from persistent responders.

    Methods: Data on 147 patients with moderately to severely active SLE without acute nephritis who were treated with placebo plus standard of care in two 52-week phase II/III trials were obtained from the Collective Data Analysis Initiative of the Lupus Foundation of America. Cross-sectional and longitudinal analyses of British Isles Lupus Assessment Group (BILAG)-based responses (improvement in all baseline A or B scores without new flare) were performed. 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 2017 Apr 5;188(1):63-78. Epub 2017 Jan 5.
    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

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