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scleroderma onset

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https://www.readbyqxmd.com/read/28089973/malignancies-in-patients-with-anti-rna-polymerase-iii-antibodies-and-systemic-sclerosis-analysis-of-the-eular-scleroderma-trials-and-research-cohort-and-possible-recommendations-for-screening
#1
Maria-Grazia Lazzaroni, Ilaria Cavazzana, Enrico Colombo, Rucsandra Dobrota, Jasmin Hernandez, Roger Hesselstrand, Cecilia Varju, Gabriella Nagy, Vanessa Smith, Paola Caramaschi, Valeria Riccieri, Eric Hachulla, Alexandra Balbir-Gurman, Emmanuel Chatelus, Katarzyna Romanowska-Próchnicka, Ana Carolina Araújo, Oliver Distler, Yannick Allanore, Paolo Airò
OBJECTIVE: To analyze the characteristics of anti-RNA polymerase III antibodies (anti-RNAP3)- positive patients with systemic sclerosis (SSc) in the European League Against Rheumatism Scleroderma Trials and Research group (EUSTAR) registry with a focus on the risk of cancer and the characteristics of malignancies, and the aim to provide guidelines about potential cancer screening in these patients. METHODS: (1) Analysis of the EUSTAR database: 4986 patients with information on their anti-RNAP3 status were included...
January 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28033278/delayed-onset-of-posterior-reversible-encephalopathy-syndrome-in-a-case-of-scleroderma-renal-crisis-with-maintenance-hemodialysis-case-report-and-literature-review
#2
Ching-Yang Chen, Shin-Yuan Hung, Yi-Jer Lee, Yi-Chan Lin, Chu-Cheng Pai
INTRODUCTION: In some cases, scleroderma renal crisis (SRC) is not easily distinguishable from other thrombotic microangiopathies such as thrombotic thrombocytopenic purpura, especially when the presentation includes neurological or extra-renal manifestations. Here, we present a case of SRC who developed a rare neurotoxic complication, posterior reversible encephalopathy syndrome (PRES).A 36-year-old man with a history of diffuse cutaneous systemic sclerosis developed SRC and acute-on-chronic renal failure and ultimately required maintenance hemodialysis...
December 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28029745/early-mortality-in-a-multinational-systemic-sclerosis-inception-cohort
#3
Yanjie Hao, Marie Hudson, Murray Baron, Patricia Carreira, Wendy Stevens, Candice Rabusa, Solene Tatibouet, Loreto Carmona, Beatriz E Joven, Molla Huq, Susanna Proudman, Mandana Nikpour
OBJECTIVE: To determine mortality and causes of death in a multinational inception systemic sclerosis (SSc) cohort. METHODS: We quantified mortality as Standardized Mortality Ratio (SMR), Years of Life Lost (YLL) and percentage mortality in the first decade of disease. The inception cohort was comprised of patients recruited within 4 years of disease onset. For comparison, we used a prevalent cohort, which included all patients irrespective of disease duration at recruitment...
December 28, 2016: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/27940590/primary-myocardial-disease-in-scleroderma-a-comprehensive-review-of-the-literature-to-inform-the-uk-systemic-sclerosis-study-group-cardiac-working-group
#4
REVIEW
Lesley-Anne Bissell, Md Yuzaiful Md Yusof, Maya H Buch
Cardiac disease is prevalent in SSc and associated with a poor prognosis. Differentiating primary myocardial disease (SSc-cardiomyopathy) from ischaemic heart disease is difficult and the disease phenotype most at risk is unclear. A comprehensive literature review was performed to inform the UK Systemic Sclerosis Study Group for cardiac disease tasked with producing a best practice pathway for the management of cardiac disease in SSc. This review describes the prevalence of SSc-cardiomyopathy, its associated greater mortality and various manifestations (e...
December 10, 2016: Rheumatology
https://www.readbyqxmd.com/read/27826170/rheumatic-diseases-induced-by-drugs-and-environmental-factors-the-state-of-the-art-part-two
#5
REVIEW
Karolina Niklas, Arkadiusz A Niklas, Dominik Majewski, Mariusz J Puszczewicz
The majority of rheumatic diseases belong to the group of autoimmune diseases and are associated with autoantibody production. Their etiology is not fully understood. Certain medications and environmental factors may have an influence on the occurrence of rheumatic diseases. Establishing a cause-effect relationship between a certain factor and disease induction is not always simple. It is important to administer the drug continuously or monitor exposure to a given factor in the period preceding the onset of symptoms...
2016: Reumatologia
https://www.readbyqxmd.com/read/27815049/therapeutic-effects-of-a-nedd8-activating-enzyme-inhibitor-pevonedistat-on-sclerodermatous-graft-versus-host-disease-in-mice
#6
Chien-Chun Steven Pai, Lam T Khuat, Mingyi Chen, William J Murphy, Mehrdad Abedi
Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is the sole treatment option for highly malignant hematologic disease; however, the major complication-graft-versus-host disease (GVHD)-still hinders its clinical application. In addition, chronic GVHD remains the major cause of long-term morbidity and mortality after allo-HSCT. Previously we showed that bortezomib, a proteasome inhibitor, can ameliorate the sclerodermatous GVHD response while maintaining graft-versus-tumor (GVT) effects. Here we report that pevonedistat (MLN4924), an inhibitor of the Nedd8-activating enzyme, which functions upstream of the proteasome in the ubiquitin-proteasome pathway, can also show similar protective effects...
January 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/27749244/uk-scleroderma-study-group-ukssg-guidelines-on-the-diagnosis-and-management-of-scleroderma-renal-crisis
#7
REVIEW
Bernadette M Lynch, Edward P Stern, Voon Ong, Mark Harber, Aine Burns, Christopher P Denton
The UK Scleroderma Study Group developed guidelines on the diagnosis and management of scleroderma renal crisis (SRC) based on best available evidence and clinical experience. SRC is characterised by the acute onset of severe hypertension and acute kidney injury. Current strategies to reduce the associated morbidity and mortality include identifying at risk patients to aid early diagnosis. ACE inhibitor therapy should be lifelong in all patients, regardless of whether they require renal replacement therapy...
September 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27700194/neurological-abnormalities-in-localized-scleroderma-of-the-face-and-head-a-case-series-study-for-evaluation-of-imaging-findings-and-clinical-course
#8
Anna Lis-Święty, Ligia Brzezińska-Wcisło, Hubert Arasiewicz
INTRODUCTION: Localized scleroderma (LoS) of the face and head is often associated with neurologic manifestations and/or imaging abnormalities in the central nervous system (CNS). CASE SERIES: We present an analysis of 20 cases of LoS affecting the face and head. The CNS symptoms and/or abnormalities in high-resolution computed tomography (HRCT) and/or magnetic resonance imaging (MRI) were observed in 12 patients (60%). In addition to the mild and unspecific disorders (e...
October 4, 2016: International Journal of Neuroscience
https://www.readbyqxmd.com/read/27641135/scleroderma-renal-crisis-and-renal-involvement-in-systemic-sclerosis
#9
REVIEW
Thasia G Woodworth, Yossra A Suliman, Daniel E Furst, Philip Clements
Scleroderma renal crisis (SRC) is a rare, potentially life-threatening complication that affects 2-15% of patients with systemic sclerosis (SSc, also known as scleroderma). SRC typically presents in patients with early, rapidly progressive, diffuse cutaneous SSc within the first 3-5 years after the onset of a non-Raynaud sign or symptom. SRC is characterized by an acute, usually symptomatic increase in blood pressure, a rise in serum creatinine levels, oliguria and thrombotic microangiopathy in about 50% of patients...
November 2016: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/27635465/prevalence-and-risk-factors-for-left-ventricular-diastolic-dysfunction-in-a-scleroderma-cohort
#10
S Vemulapalli, L Cohen, V Hsu
OBJECTIVES: Left ventricular diastolic dysfunction (LVDD) is more common in systemic sclerosis (SSc) compared to the general population. Focal myocardial ischaemia and fibrosis may be important in its pathogenesis. LVDD is associated with increased mortality and little is known about the risk factors. Advanced SSc lung complications may accompany LVDD. METHOD: We conducted a cross-sectional study of 300 SSc outpatients with and without LVDD, seen between May 2012 and May 2014, and performed univariate and multivariate regression analyses to determine clinical factors associated with LVDD...
September 16, 2016: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/27548651/myopathy-in-scleroderma-and-in-other-connective-tissue-diseases
#11
Julie J Paik
PURPOSE OF REVIEW: This review discusses the most updated literature of myopathy in scleroderma and other connective tissue diseases. RECENT FINDINGS: In the past year, studies have demonstrated that myopathy in scleroderma is associated with poor outcomes such as disability and mortality. In addition, muscle histopathology in scleroderma continues to reveal that it is a heterogeneous entity, and that necrosis and acute neurogenic atrophy may be a more prevalent histopathologic feature in muscle biopsies than previously reported...
November 2016: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/27504022/rheumatic-diseases-induced-by-drugs-and-environmental-factors-the-state-of-the-art-part-one
#12
REVIEW
Karolina Niklas, Arkadiusz A Niklas, Dominik Majewski, Mariusz Puszczewicz
The majority of rheumatic diseases belong to the group of autoimmune diseases and are associated with autoantibody production. Their etiology is not fully understood. Certain medications and environmental factors may have an influence on the occurrence of rheumatic diseases. Establishing a cause-effect relationship between a certain factor and disease induction is not always simple. It is important to administer the drug continuously or monitor exposure to a given factor in the period preceding the onset of symptoms...
2016: Reumatologia
https://www.readbyqxmd.com/read/27477178/trichotillomania-bizzare-patern-of-hair-loss-at-11-year-old-girl
#13
Jana Zímová, Pavlína Zímová
Trichotillomania (TTM) is defined by the Diagnostics and Statistic Manual of Mental Disorders, 4th edition (DMS-IV) as hair loss from a patient`s repetitive self-pulling of hair. The disorder is included under anxiety disorders because it shares some obsessive-compulsive features. Patients have the tendency towards feelings of unattractiveness, body dissatisfaction, and low self-esteem (1,2). It is a major psychiatric problem, but many patients with this disorder first present to a dermatologist. An 11-year-old girl came to our department with a 2-month history of diffuse hair loss on the frontoparietal and parietotemporal area (Figure 1)...
June 2016: Acta Dermatovenerologica Croatica: ADC
https://www.readbyqxmd.com/read/27472742/complement-activation-and-effect-of-eculizumab-in-scleroderma-renal-crisis
#14
Arnaud Devresse, Selda Aydin, Moglie Le Quintrec, Nathalie Demoulin, Patrick Stordeur, Catherine Lambert, Sara Gastoldi, Yves Pirson, Michel Jadoul, Johann Morelle
BACKGROUND: Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis characterized by abrupt onset of hypertension, thrombotic microangiopathy, and kidney injury. The mechanisms of the disease remain ill-defined, but a growing body of evidence suggests that activation of the complement system may be involved. METHODS: Here, we report the case of a patient presenting with severe SRC and strong evidence of complement activation, both in serum and in the kidney, in the absence of genetic defect of the complement system...
July 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27407276/eosinophilic-fasciitis-after-parasite-infection
#15
Marta Oliveira, Fabia Patinha, Antonio Marinho
Eosinophilic fasciitis is a systemic inflammatory disease characterized by symmetrical swelling and skin induration of the distal portions of the arms and/or legs, evolving into a scleroderma-like appearance, accompanied by peripheral blood eosinophilia. It is a rare disease with a poorly understood etiology. Corticosteroid treatment remains the standard therapy, either taken alone or in association with an immunosuppressive drug. This paper presents a case of a male patient with palpebral edema and marked eosinophilia, diagnosed with intestinal parasitic infection in October 2006...
2016: Reumatologia
https://www.readbyqxmd.com/read/27401109/a-systematic-review-of-tools-for-determining-activity-of-localized-scleroderma-in-paediatric-and-adult-patients
#16
REVIEW
A Lis-Święty, I Janicka, A Skrzypek-Salamon, L Brzezińska-Wcisło
Localized scleroderma (LoS) is a rare inflammatory skin disorder that affects the dermis and sometimes subcutaneous tissues. LoS can have very long periods of quiescence followed by reactivation, but the progression or activity of the disease is difficult to measure. To review the measuring tools used for the evaluation of LoS activity, to choose the most appropriate technique to facilitate progress towards properly assessing the disease, a systematic review of the literature was carried out using the PubMed MEDLINE...
January 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/27382493/mutation-in-lemd3-man1-associated-with-osteopoikilosis-and-late-onset-generalized-morphea-a-new-buschke-ollendorf-syndrome-variant
#17
Benjamin Korman, Jun Wei, Anne Laumann, Polly Ferguson, John Varga
Introduction. Buschke-Ollendorf syndrome (BOS) is an uncommon syndrome characterized by osteopoikilosis and other bone abnormalities, accompanied by skin lesions, most frequently connective tissue nevi. BOS is caused by mutations in the LEMD3 gene, which encodes the inner nuclear membrane protein Man1. We describe a unique case of osteopoikilosis associated with late-onset localized scleroderma and familial LEMD3 mutations. Case Report. A 72-year-old woman presented with adult-onset diffuse morphea and bullous skin lesions...
2016: Case Reports in Dermatological Medicine
https://www.readbyqxmd.com/read/27277422/a-novel-dnmt1-mutation-associated-with-early-onset-hereditary-sensory-and-autonomic-neuropathy-cataplexy-cerebellar-atrophy-scleroderma-endocrinopathy-and-common-variable-immune-deficiency
#18
Robin Fox, John Ealing, Helen Murphy, David P Gow, David Gosal
DNA methyltransferase 1 (DNMT1) is an enzyme which has a role in methylation of DNA, gene regulation, and chromatin stability. Missense mutations in the DNMT1 gene have been previously associated with two neurological syndromes: hereditary sensory and autonomic neuropathy type 1 with dementia and deafness (HSAN1E) and autosomal dominant cerebellar ataxia, deafness, and narcolepsy (ADCA-DN). We report a case showing overlap of both of these syndromes plus associated clinical features of common variable immune deficiency, scleroderma, and endocrinopathy that could also be mutation associated...
September 2016: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/27239931/-pulmonary-fibrosis-as-the-first-manifestation-of-scleroderma-systematica-overlap-syndrome
#19
N A Morova, T V Kropotina, E S Shelyagina, A V Pavlov
The paper describes a clinical case of a female patient with signs of visceral scleroderma systematica and rheumatoid arthritis (RA). Interstitial pulmonary fibrosis was the first manifestation of overlap syndrome. The articular and cutaneous of the syndrome were moderate and cast doubts upon whether the systemic rheumatic disease was present. Postmortem examination confirmed the presence of pulmonary fibrosis, scleroderma, and RA. The case is of interest from the point of view of the atypical onset of the disease...
2016: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/27149136/paraneoplastic-scleroderma-are-there-any-clues
#20
Hana Jedlickova, Veronika Durčanská, Vladimír Vašků
Dear Editor, Scleroderma associated with neoplasia is rare, with only a small number of cases reported. We describe 4 patients with paraneoplastic scleroderma who were treated at the I. Department of Dermatovenereology, St. Anna Hospital, during the period between 2004 and 2014. The patients were diagnosed with cholangiogenic carcinoma, endometrial carcinoma, prostatic adenocarcinoma, and adenoma of the suprarenal gland. In the case of concurrent scleroderma and tumor, four situations may occur: they can develop independently of each other; scleroderma may be induced by the tumor; the tumor can develop in the scleroderma; or the tumor can be induced by immunosuppressive therapy...
April 2016: Acta Dermatovenerologica Croatica: ADC
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