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Scleroderma renal crisis

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https://www.readbyqxmd.com/read/28212921/towards-developing-criteria-for-scleroderma-renal-crisis-a-scoping-review
#1
REVIEW
Sabrina Hoa, Edward P Stern, Christopher P Denton, Marie Hudson
OBJECTIVE: The absence of a gold standard for scleroderma renal crisis (SRC) has hindered our understanding of this problem. The objective of this scoping review was to identify the criteria used to define SRC in order to guide the development of a consensus definition for SRC. METHODS: We conducted a search in three databases: Medline, Embase and non-Ovid Pubmed. Papers were eligible for inclusion if they were full-length articles in English whose main topic was SRC or scleroderma renal disease...
February 14, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28120425/kidney-transplantation-in-patients-with-systemic-sclerosis-a-nationwide-multicenter-study
#2
Dominique Bertrand, Julien Dehay, Julien Ott, Rebecca Sberro, Charlotte Brunelle, Nassim Kamar, Charlotte Colosio, Valérie Chatelet, Laetitia Albano, Sophie Girerd, Vincent Audard, Christelle Barbet, Jacques Dantal, Didier Ducloux, Antoine Durrbach, Valérie Garrigue, Marc Hazzan, Anne-Elisabeth Heng, Christophe Mariat, Pierre Merville, Jean-Philippe Rerolle, Bruno Moulin, Dominique Guerrot
Kidney transplantation is one of the therapeutic options for end-stage renal disease (ESRD) in systemic sclerosis (SS). Current evidence demonstrates poorer patient and graft survival after transplantation in SS than in other primary kidney diseases. All the patients presenting ESRD associated with SS who had received a kidney allograft between 1987 and 2013 were systematically included from 20 French kidney transplantation centres. Thirty-four patients received 36 kidney transplants during the study period...
January 25, 2017: Transplant International: Official Journal of the European Society for Organ Transplantation
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
#3
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
#4
REVIEW
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
#5
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/27863511/analysis-of-complement-biomarkers-in-systemic-sclerosis-indicates-a-distinct-pattern-in-scleroderma-renal-crisis
#6
Marcin Okrój, Martin Johansson, Tore Saxne, Anna M Blom, Roger Hesselstrand
BACKGROUND: The complement system has been implicated in pathogenesis of systemic sclerosis (SSc). The goal of the present study was to evaluate improved complement biomarkers in SSc. METHODS: The presence of C4d, reflecting activation of the classical/lectin pathways, C3bBbP corresponding to activation of the alternative pathway, and soluble terminal complement complexes (all complement pathways), was measured in plasma samples by enzyme-linked immunosorbent assay and correlated to clinical parameters...
November 18, 2016: Arthritis Research & Therapy
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/27641135/scleroderma-renal-crisis-and-renal-involvement-in-systemic-sclerosis
#8
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/27578917/scleroderma-renal-crisis-during-intravenous-cyclophosphamide-pulse-therapy-for-complicated-interstitial-lung-disease-was-successfully-treated-with-angiotensin-converting-enzyme-inhibitor-and-plasma-exchange
#9
Norihiro Nagamura, Seikon Kin
Systemic sclerosis (SSc) is a multiorgan disorder involving the skin, heart, lungs, kidneys, and intestines. Progressive interstitial lung disease (ILD) is a serious complication in SSc patients, and cyclophosphamide (CYC) is the only recommended therapy for this condition;(1)) however, its clinical effectiveness is not sufficient. Scleroderma renal crisis (SRC) is a rare complication, characterized by acute renal failure and progressive hypertension. Angiotensin-converting-enzyme inhibitor (ACE-i) is a widely accepted therapy for SRC...
August 2016: Nagoya Journal of Medical Science
https://www.readbyqxmd.com/read/27559487/a-unique-presentation-of-anti-rna-polymerase-iii-positive-systemic-sclerosis-sine-scleroderma
#10
Cody M Lee, Diana Girnita, Arundhati Sharma, Surabhi Khanna, Jean M Elwing
Systemic sclerosis is a rare autoimmune disorder with a wide spectrum of clinical manifestations and a multitude of autoantibodies that are associated with it. In the past several years, advances in serologic testing have led to research indicating important prognostic and phenotypic associations with certain subsets of autoantibodies. In particular, anti-RNA polymerase III (anti-RNAP III) has been associated with diffuse cutaneous disease, scleroderma renal crisis, a temporal relationship with malignancy, myositis, synovitis, joint contractures, and gastric antral vascular ectasia...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27472742/complement-activation-and-effect-of-eculizumab-in-scleroderma-renal-crisis
#11
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/27312381/digital-ulcers-and-cutaneous-subsets-of-systemic-sclerosis-clinical-immunological-nailfold-capillaroscopy-and-survival-differences-in-the-spanish-rescle-registry
#12
Carles Tolosa-Vilella, Maria Lluisa Morera-Morales, Carmen Pilar Simeón-Aznar, Begoña Marí-Alfonso, Dolores Colunga-Arguelles, José Luis Callejas Rubio, Manuel Rubio-Rivas, Maika Freire-Dapena, Alfredo Guillén-Del Castillo, Nerea Iniesta-Arandia, Maria Jesús Castillo-Palma, Marivi Egurbide-Arberas, Luis Trapiellla-Martínez, José A Vargas-Hitos, José Antonio Todolí-Parra, Mónica Rodriguez-Carballeira, Adela Marin-Ballvé, Xavier Pla-Salas, Juan José Rios-Blanco, Vicent Fonollosa-Pla
OBJECTIVE: Digital ulcers (DU) are the most common vascular complication of systemic sclerosis (SSc). We compared the characteristics between patients with prior or current DU with those never affected and evaluated whether a history of DU may be a predictor of vascular, organ involvement, and/or death in patients with SSc. METHODS: Data from SSc patients with or without prior or current DU were collected by 19 referral centers in an ongoing registry of Spanish SSc patients, named Registro de ESCLErodermia (RESCLE)...
October 2016: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/27139167/thrombotic-complications-after-radial-arterial-line-placement-in-systemic-sclerosis-a-case-series
#13
Julie J Paik, Ram Hirpara, Jennifer A Heller, Laura K Hummers, Fredrick M Wigley, Ami A Shah
OBJECTIVE: To demonstrate potential thrombotic complications after radial arterial line placement in patients with scleroderma. METHODS: This is a retrospective case series of 4 patients with scleroderma who were hospitalized in the intensive care unit (ICU) requiring invasive hemodynamic monitoring and developed severe complications after radial arterial line placement. We reviewed their medical records to assess their laboratory findings and clinical presentations...
October 2016: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/27134252/the-frequency-of-scleroderma-renal-crisis-over-time-a-metaanalysis
#14
Matthew Turk, Janet E Pope
OBJECTIVE: Systemic sclerosis (SSc) leads to a high mortality from internal organ involvement. Scleroderma renal crisis (SRC) usually occurs in the diffuse cutaneous SSc (dcSSc) subset early in the disease, often with acute severe hypertension and renal failure. Prevalence of SRC since its classification in the early 1970s was determined in publications to assess whether the prevalence of SRC has changed over time because the proportion with the dcSSc subset is smaller in contemporary cohorts...
July 2016: Journal of Rheumatology
https://www.readbyqxmd.com/read/27125627/evaluation-of-estimated-glomerular-filtration-rate-and-clinical-variables-in-systemic-sclerosis-patients
#15
Antonietta Gigante, Biagio Barbano, Guido Granata, Silvia Quarta, Antonio Amoroso, Felice Salsano, Rosario Cianci, Edoardo Rosato
OBJECTIVES: The most important renal complication of systemic sclerosis (SSc) is scleroderma renal crisis (SRC). Many patients demonstrate less severe renal complications, most likely associated with reduced renal blood flow and a consequent reduction in glomerular filtration rate (GFR). The mechanism of this slowly progressive form of chronic renal disease is unclear. The aim of this study was to evaluate GFR by the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) and the 7-variable Modification of Diet and Renal Disease (MDRD) equations in SSc patients and to correlate estimated GFR (eGFR) with clinical variables of the disease...
June 2016: Clinical Nephrology
https://www.readbyqxmd.com/read/27056603/n-terminal-pro-brain-natriuretic-peptide-as-predictor-of-outcome-in-scleroderma-renal-crisis
#16
Cecilia Beatrice Chighizola, Francesca Pregnolato, Pier Luigi Meroni, Christopher Paul Denton, Voon H Ong
OBJECTIVES: Although in scleroderma renal crisis (SRC) outcome has improved to a great extent with the introduction of ACE inhibitors, there remains significant mortality and morbidity with frequent requirement for renal replacement therapy. Therefore, novel biomarkers to identify patients at high risk of poor outcome would be valuable. The aim of this study was to assess the role of the N terminal fragment of pro Brain Natriuretic Peptide (N-TproBNP) as predictor of outcome in SRC. METHODS: 20 subjects with confirmed SRC were retrospectively enrolled...
September 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/26942857/anti-rna-polymerase-iii-antibody-associated-scleroderma-renal-crisis-in-a-patient-with-limited-cutaneous-systemic-sclerosis-a-case-report
#17
Daisuke Takada, Junichi Hoshino, Koichi Kikuchi, Junko Yabuuchi, Yuta Kogure, Toshiharu Ueno, Akinari Sekine, Masayuki Yamanouchi, Keiichi Sumida, Koki Mise, Tatsuya Suwabe, Noriko Hayami, Naoki Sawa, Kenmei Takaichi, Nobukazu Hayasi, Takeshi Fujii, Kenichi Ohashi, Yoshifumi Ubara
A 69-year-old Japanese man was presented with hypertensive crisis. Renal histology revealed malignant nephrosclerosis, including an onion skin pattern with fibrinoid necrosis of the small arteries from arterioles up to interlobular arteries. Immunological investigation clarified positive anti-RNA polymerase (RNAP) III antibody, and limited cutaneous systemic sclerosis (Lc SSc) was diagnosed by skin biopsy as the underlying disease causing scleroderma renal crisis (SRC). Angiotensin covering enzyme (ACE) inhibitor therapy and calcium antagonist were effective for his renal condition...
March 4, 2016: Modern Rheumatology
https://www.readbyqxmd.com/read/26868300/emergency-situations-in-rheumatology-with-a-focus-on-systemic-autoimmune-diseases
#18
REVIEW
Jiri Vymetal, Martina Skacelova, Andrea Smrzova, Anna Klicova, Marketa Schubertova, Pavel Horak, Josef Zadrazil
BACKGROUND AND AIM: Rheumatic diseases are commonly considered chronic conditions. However, acute manifestations can be very severe and represent a diagnostic problem. Examples are systemic lupus erythematosus with acute flare, glomerulonephritis, CNS disorders and catastrophic antiphospholipid syndrome, scleroderma with interstitial lung disease, pulmonary hypertension and renal crisis and polyangiitis with alveolar haemorhage and acute respiratory failure. This aim of this paper is to overview emergency situations which can be encountered in the care of patients with autoimmune systemic diseases and vasculitides...
March 2016: Biomedical Papers of the Medical Faculty of the University Palacký, Olomouc, Czechoslovakia
https://www.readbyqxmd.com/read/26711696/recent-developments-in-the-classification-evaluation-pathophysiology-and-management-of-scleroderma-renal-crisis
#19
REVIEW
Cybele Ghossein, John Varga, Andrew Z Fenves
Scleroderma renal crisis (SRC) is an uncommon complication of systemic sclerosis. Despite the advent of angiotensin-converting inhibitor therapy, SRC remains a life-threatening complication. Recent studies have contributed to a better understanding of SRC, but much remains unknown regarding its pathophysiology, risk factors, and optimal management. Genetic studies provide evidence that immune dysregulation might be a contributing factor, providing hope that further research in this direction might illuminate pathogenesis and provide novel predictors for this complication...
January 2016: Current Rheumatology Reports
https://www.readbyqxmd.com/read/26664221/acute-kidney-injury-in-a-patient-with-systemic-sclerosis-looking-beyond-scleroderma-renal-crisis
#20
R P Goswami, S Mondal, K Basu, S Das, P Ghosh, A Ghosh
No abstract text is available yet for this article.
November 2015: Indian Journal of Nephrology
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