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lupus erythematosus diagnosis

Shuaihantian Luo, Yunuo Wang, Ming Zhao, Qianjin Lu
Systemic lupus erythematosus (SLE) is a severe autoimmune disease that causes multiple-organ dysfunction mainly affecting women in their childbearing years. Type I IFN synthesis is usually triggered by viruses, and its production is tightly regulated and limited in time in health individuals. However, many patients with systemic autoimmune diseases including SLE have signs of aberrant production of type I interferon (IFN) and display an increased expression of IFN-inducible genes. Continuous type I IFNs derived from activated plasmacytoid dendritic cells (pDCs) by interferogenic immune complexes (ICs) and migration of these cells to tissues both break immune tolerance and promote an on-going autoimmune reaction in human body...
October 18, 2016: International Immunopharmacology
Yu-Min Shen
Atypical hemolytic uremic syndrome (aHUS) is a rare genetic disorder caused by defective complement regulation resulting in thrombotic microangiopathy (TMA). Patients can present as children or adults. The syndrome consists of hemolytic anemia with schistocytosis, thrombocytopenia, significant renal damage, and/or other organ system dysfunction(s). Patients with aHUS may succumb to the complications of the disease with the very first manifestation; surviving patients often suffer from progressive organ dysfunction with significant morbidity and mortality despite plasma infusion or plasma exchange...
2016: Thrombosis Journal
Abhinav Anand, Kruthi Malur, Juhi Kawale, Milind Y Nadkar
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. However, she had relapse which subsequently responded to pulse cyclophosphamide and steroids...
July 2016: Journal of the Association of Physicians of India
Arsenio Spinillo, Fausta Beneventi, Elena Locatelli, Vèronique Ramoni, Roberto Caporali, Claudia Alpini, Giulia Albonico, Chiara Cavagnoli, Carlomaurizio Montecucco
BACKGROUND: The burden of pregnancy complications associated with well defined, already established systemic rheumatic diseases preexisting pregnancy such as rheumatoid arthritis, systemic lupus erythematosus or scleroderma is well known. Systemic rheumatic diseases are characterized by a long natural history with few symptoms, an undifferentiated picture or a remitting course making difficult a timely diagnosis. It has been suggested that screening measures for these diseases could be useful but the impact of unrecognized systemic rheumatic disorders on pregnancy outcome is unknown...
October 18, 2016: BMC Pregnancy and Childbirth
Jessica H Knight, Penelope P Howards, Jessica B Spencer, Katina C Tsagaris, Sam S Lim
OBJECTIVE: Systemic lupus erythematosus (SLE) disproportionately affects women and often develops during their reproductive years. Research suggests that some women who receive cyclophosphamide as treatment for SLE experience earlier decline in menstrual function, but reproductive health among women with SLE who have not taken this drug is less well understood. This study aims to better understand the relation between SLE and reproduction by assessing early secondary amenorrhoea and pregnancy in women treated with and without cyclophosphamide from a population-based cohort with large numbers of African-Americans...
2016: Lupus Science & Medicine
Chao-Yi Wu, Huang-Yu Yang, Tsung-Chieh Yao, Su-Hsun Liu, Jing-Long Huang
An urge of biomarker identification is needed to better monitor lupus nephritis (LN) disease activity, guide clinical treatment, and predict patient's long-term outcome. With the proinflammatory effect and its association with inflammasomes, the significance of interleukin-18 (IL-18) among pediatric-onset systemic lupus erythematous (pSLE) patient, especially, its importance in predicting long-term renal outcome was investigated.In a pSLE cohort of 96 patients with an average follow-up period of 10.39 ± 3...
October 2016: Medicine (Baltimore)
Julie A Birt, YingMeei Tan, Neelufar Mozaffarian
OBJECTIVES: To better understand the real-world characteristics and costs of Sjögren's syndrome (SS). METHODS: Analysing the MarketScan Commercial Claims database from Jan. 1, 2006 to Dec. 31, 2011, we identified 10,414 patients ≥18 years old newly diagnosed with SS. Patient characteristics, drugs (commonly used for SS), resource utilisation, and medical costs were evaluated for 12 months pre- and post-diagnosis. RESULTS: Mean age was 55 years; 90% were female...
October 7, 2016: Clinical and Experimental Rheumatology
Dam Kim, Jeongim Choi, Soo-Kyung Cho, Chan-Bum Choi, Tae-Hwan Kim, Jae-Bum Jun, Dae-Hyun Yoo, Sang-Cheol Bae, Yoon-Kyoung Sung
OBJECTIVE: To identify the clinical characteristics of diffuse alveolar hemorrhage (DAH) compared to other types of acute diffuse lung infiltration in SLE patients, and the factors associated with mortality in these patients. METHODS: We studied a retrospective cohort including SLE patients with acute diffuse lung infiltration on thoracic CT between January 2004 and August 2014. We divided them into 2 groups, a DAH and a non-DAH group, and compared the clinical characteristics and outcomes in the 2 groups...
September 14, 2016: Seminars in Arthritis and Rheumatism
Wais Afzal, Talal Arab, Tofura Ullah, Katerina Teller, Kaushik J Doshi
Lymphadenopathy could represent a vast spectrum of etiologies including infectious and non-infectious diseases. Besides proper history taking, physical examination, and laboratory investigations, a tissue diagnosis is often necessary to unmask the cause of generalized lymphadenopathy. Here we present a 23-year-old woman who was admitted for diffuse generalized lymphadenopathy, fatigue, malaise, weight loss, nausea, and bilateral lower extremity edema. She reported a history of seizures as well as stroke 2 years prior with no other medical conditions present...
November 2016: Journal of Clinical Medicine Research
Mehmet Taşdemir, Chiar Hasan, Ayşe Ağbaş, Özgür Kasapçopur, Nur Canpolat, Lale Sever, Salim Çalışkan
Systemic lupus erythematosus and Sjögren's syndrome are chronic auto- inflammatory disorders which can lead to serious organ damage. Although systemic lupus erythematosus and Sjögren's syndrome were previously considered two forms of the same disease because of presence of clinical coexistence of these two conditions, the view that they are two different conditions with mutual characteristics has become prominent in recent years. In this paper, we reported a 16 year-old girl who was followed up with a diagnosis of Sjögren's syndrome for six years and then was observed to have overlap of systemic lupus erythematosus...
September 2016: Türk Pediatri Arşivi
Devika Gupta, Supreet Mohanty, Deepshi Thakral, Arvind Bagga, Naveet Wig, Dipendra Kumar Mitra
BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) in the background of systemic lupus erythematosus (SLE) is rare. Inability to discriminate between these two entities may be fatal for the patient. Here we report two cases of SLE with secondary HLH, one of which manifested HLH as the initial presentation, and the significance of HLH's timely diagnosis. CASE REPORT We describe two cases of SLE secondarily affected by HLH, which were diagnosed by various laboratory parameters and detection of profoundly reduced NK cell activity by using flow cytometry...
October 13, 2016: American Journal of Case Reports
Bérangère S Joly, Alain Stepanian, Thierry Leblanc, David Hajage, Hervé Chambost, Jérôme Harambat, Fanny Fouyssac, Vincent Guigonis, Guy Leverger, Tim Ulinski, Thérésa Kwon, Chantal Loirat, Paul Coppo, Agnès Veyradier
BACKGROUND: Thrombotic thrombocytopenic purpura is a rare thrombotic microangiopathy, related to a severe ADAMTS13 deficiency (a disintegrin and metalloprotease with thromboSpondin type 1 repeats, member 13; activity <10% of normal). Childhood-onset thrombotic thrombocytopenic purpura is very rare and initially often misdiagnosed, especially when ADAMTS13 deficiency is acquired (ie, not linked to inherited mutations of the ADAMTS13 gene). We aimed to investigate initial presentation, management, and outcome of acquired thrombotic thrombocytopenic purpura in children...
October 3, 2016: Lancet Haematology
Filomena Panza, Maria Claudia Alcaro, Fiorella Petrelli, Francesca Angelotti, Federico Pratesi, Paolo Rovero, Paola Migliorini
BACKGROUND: The detection of anti-dsDNA antibodies is critical for the diagnosis and follow-up of systemic lupus erythematosus (SLE) patients. The presently available assays are characterized by a non-optimal specificity (solid phase assays) or sensitivity (Crithidia Luciliae immunofluorescence test (CLIFT)). To overcome the limits of CLIFT and solid phase chromatin assays, we explored the diagnostic potential of an assay based on plasmid DNA containing a highly bent fragment of 211 bp from Crithidia Luciliae minicircles, complexed with histone peptides...
October 4, 2016: Arthritis Research & Therapy
Takuma Tsuzuki Wada, Kojiro Sato, Toshihide Mimura
We encountered a case of a middle-aged woman with systemic lupus erythematosus. As the patient had progressive peripheral neuropathy including foot drop, we intended to treat her with intensive immunosuppressive therapy as soon as possible. Pretreatment assessment, however, revealed multiple nodular lesions in the lungs and bones, suggesting disseminated tumor metastasis or miliary tuberculosis. To our surprise, gallium and bone scintigraphy as well as cytodiagnosis revealed no sign of malignancy or infection, leading us to suspect the presence of another multisystem disorder...
March 2016: Eur J Rheumatol
Avital Avriel, Stela Fleischer, Michael Friger, Ora Shovman, Gal Neuman, Yehuda Shoenfeld, Mahmoud Abu-Shakra
A significantly high correlation between reduced activity of Annexin A5 by the flow cytometric assay (FCA) and the diagnosis of antiphospholipid syndrome (APS) has been reported. The aim of this study was to assess the clinical and laboratory significance of the Annexin A5 competition assay among patients with systemic lupus erythematosus (SLE). The FCA competition assay was performed on blood samples from 57 consecutive SLE patients. The FCA was performed according to a previously validated method. Forty-seven patients (82...
October 4, 2016: Clinical Rheumatology
A Bortoluzzi, F Furini, F Campanaro, M Govoni
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...
October 4, 2016: Lupus
Bruna Burko Rocha Chu, Giorgina Falcão Brandão Côrtes Gobbo, Rafaela Copês, Glênio Gutjahr, Erick Cavalcanti Cossa, Eduardo Dos Santos Paiva
Leprosy is a chronic infectious disease with a wide spectrum of clinical manifestations. Musculoskeletal impairment is the third most frequent clinical manifestation, the most common being arthritis. Cases misdiagnosed as lupus erythematosus, vasculitis, rheumatoid arthritis, and systemic sclerosis have already been described. We report a case of leprosy with skin thickening, distal phalanx resorption and facial telangiectasia, simulating systemic sclerosis. Due to a considerable prevalence in Brazil, and also considering the deformities that can be caused by a delay in diagnosis and treatment, leprosy should be put forward by the rheumatologist as a diagnostic hypothesis in patients with musculoskeletal involvement...
September 17, 2016: Revista Brasileira de Reumatologia
Marco Ardigo, Marina Agozzino, Chiara Franceschini, Francesco Lacarrubba
Reflectance confocal microscopy (RCM) allows real-time, noninvasive microscopic view of the skin at nearly histologic resolution serially over time. RCM increases the sensibility and sensitivity of the diagnosis of skin tumours. RCM evaluates descriptive features of psoriasis, lupus erythematosus, contact dermatitis, and others. Three groups of optical histology have been described: psoriasiform, spongiotic, and interface dermatitis. In a multicenter study, RCM patterns of spongiotic, hyperkeratotic, and interface dermatitis have been analyzed and an algorithmic method of analysis for fast application in the clinical setting based on a multivariate analysis has been proposed...
October 2016: Dermatologic Clinics
Aline Lauria Pires Abrão, Caroline Menezes Santana, Ana Cristina Barreto Bezerra, Rivadávio Fernandes Batista de Amorim, Mariana Branco da Silva, Licia Maria Henrique da Mota, Denise Pinheiro Falcão
Orofacial manifestations occur frequently in rheumatic diseases and usually represent early signs of disease or of its activity that are still neglected in clinical practice. Among the autoimmune rheumatic diseases with potential for oral manifestations, rheumatoid arthritis (RA), inflammatory myopathies (IM), systemic sclerosis (SSc), systemic lupus erythematosus (SLE), relapsing polychondritis (RP) and Sjögren's syndrome (SS) can be cited. Signs and symptoms such as oral hyposalivation, xerostomia, temporomandibular joint disorders, lesions of the oral mucosa, periodontal disease, dysphagia, and dysphonia may be the first expression of these rheumatic diseases...
September 2016: Revista Brasileira de Reumatologia
Ye Shu, Qinghua Hu, Hai Long, Christopher Chang, Qianjin Lu, Rong Xiao
Autoimmune diseases are characterized by aberrant immune responses against healthy cells and tissues. However, the exact mechanisms underlying the development of these conditions remain unknown. CD4+CD25+ regulatory T cells (Tregs) are a subset of mature T cells which have an important role in maintaining immune homeostasis and preventing autoimmune diseases. Forkhead box p3 (Foxp3), a member of the fork head transcription factor family, is recognized as a marker of CD4+CD25+ Tregs. The decreased number and/or function of CD4+CD25+ Tregs in peripheral blood and related tissues has been demonstrated in systemic lupus erythematosus, systemic sclerosis, and other autoimmune diseases, which are at least partially regulated by epigenetic mechanisms...
September 29, 2016: Clinical Reviews in Allergy & Immunology
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