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Scleroderma renal crisis

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https://www.readbyqxmd.com/read/29781586/treatment-algorithms-for-systemic-sclerosis-according-to-experts
#1
Andreu Fernández-Codina, Kyle M Walker, Janet E Pope
INTRODUCTION: Treatment for many aspects of systemic sclerosis (SSc) lacks agreement. OBJECTIVES: To generate SSc treatment algorithms endorsed by high percentage of SSc experts. METHODS: Experts from the Scleroderma Clinical Trials Consortium and the Canadian Scleroderma Research group (N=170) were asked whether they agreed with SSc algorithms (from 2012). Two consensus rounds refined agreement; 62 (36%), 54 (32%) and 48 (28%) experts completed surveys...
May 21, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29763902/prognostic-factors-of-renal-involvement-in-systemic-sclerosis
#2
Edoardo Rosato, Antonietta Gigante, Biagio Barbano, Maria Ludovica Gasperini, Rosario Cianci, Maurizio Muscaritoli
BACKGROUND/AIMS: Renal involvement is common in systemic sclerosis (SSc), including asymptomatic reduction of glomerular filtration rate (GFR), increased renal resistance indices, scleroderma renal crisis (SRC) and ANCA-associated vasculitis. The aim was to evaluate type and evolution of renal involvement for a period of five years. METHODS: 121 SSc patients (100 F, 21 M) with mean age of 54.9 ± 13.8, disease duration of 9 ± 6 years, of which 62 had a diffused form and 59 limited form were enrolled...
May 10, 2018: Kidney & Blood Pressure Research
https://www.readbyqxmd.com/read/29738043/complement-activation-in-atypical-hemolytic-uremic-syndrome-and-scleroderma-renal-crisis-a-critical-analysis-of-pathophysiology
#3
Roman Zuckerman, Arif Asif, Eric J Costanzo, Tushar Vachharajani
Scleroderma is an autoimmune disease that affects multiple systems. While pathophysiologic mechanisms governing the development of scleroderma are relatively poorly understood, advances in our understanding of the complement system are clarifying the role of complement pathways in the development of atypical hemolytic uremic syndrome and scleroderma renal crisis. The abundant similarities in their presentation as well as the clinical course are raising the possibility of a common underlying pathogenesis. Recent reports are emphasizing that complement pathways appear to be the unifying link...
May 7, 2018: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29729875/scleroderma-renal-crisis-a-reversible-cause-of-left-ventricular-dysfunction
#4
Juan Martínez-Milla, Hans Paul Gaebelt, Olga Sánchez-Pernaute, Andrea Kallmeyer, José Romero, Jerónimo Farré
We report a case of acute left ventricular dysfunction due to myocarditis, in the setting of a scleroderma renal crisis. The case is particularly intriguing for the favorable outcome of both symptoms and heart function following immunosuppressive therapy. We also highlight the changes observed over time with image techniques as well as in electrocardiograms.
May 2, 2018: Reumatología Clinica
https://www.readbyqxmd.com/read/29686439/clinical-pattern-of-systemic-sclerosis-in-central-ukraine-association-between-clinical-manifestations-of-systemic-sclerosis-and-hypertension
#5
Viktor Semenov, Olexandr Kuryata, Tatiana Lysunets
Objectives: Systemic sclerosis (SSc) is a rare disease of connective tissue, manifestations of which may vary in different geographical areas. We aimed to describe the clinical portrait of patients with SSc in Dnipropetrovsk region and to investigate how initial clinical and laboratory characteristics are connected with the presence of hypertension in SSc onset. Material and methods: Patients were enrolled to this study from the registry of SSc patients, established in the Rheumatology Department, Mechnikov Dnipropetrovsk Regional Clinic, Dnipro...
2018: Reumatologia
https://www.readbyqxmd.com/read/29595279/-systemic-sclerosis-in-2017
#6
Tomáš Soukup, Tomáš Veleta
Systemic sclerosis is classed as a diffuse (systemic) disease of connective tissue. It is a heterogeneous disease significantly shortening life expectancy. Its etiology is unknown. Pathogenetic interplay is assumed to involve a triad of pathological autoimmune inflammation, vasculopathy and fibrosis. Clinical manifestations can be classed based on the preponderant pathogenetic process. Vasculopathy is manifested by secondary Raynauds phenomenon with abnormal findings on the nailfold capillaroscopy, skin telangiectasias, gastric antral vascular ectasia, life threatening scleroderma renal crisis, digital ulcerations and prognostically severe pulmonary arterial hypertension...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29394404/scleroderma-like-renal-crisis-in-a-patient-with-anti-threonyl-trna-synthetase-associated-antisynthetase-syndrome
#7
Maud A W Hermans, Jelle R Miedema, Rob M Verdijk, Paul L A van Daele
No abstract text is available yet for this article.
April 1, 2018: Rheumatology
https://www.readbyqxmd.com/read/29390428/clinical-and-serological-features-of-systemic-sclerosis-in-a-multicenter-african-american-cohort-analysis-of-the-genome-research-in-african-american-scleroderma-patients-clinical-database
#8
MULTICENTER STUDY
Nadia D Morgan, Ami A Shah, Maureen D Mayes, Robyn T Domsic, Thomas A Medsger, Virginia D Steen, John Varga, Mary Carns, Paula S Ramos, Richard M Silver, Elena Schiopu, Dinesh Khanna, Vivien Hsu, Jessica K Gordon, Heather Gladue, Lesley A Saketkoo, Lindsey A Criswell, Chris T Derk, Marcin A Trojanowski, Victoria K Shanmugam, Lorinda Chung, Antonia Valenzuela, Reem Jan, Avram Goldberg, Elaine F Remmers, Daniel L Kastner, Fredrick M Wigley, Pravitt Gourh, Francesco Boin
Racial differences exist in the severity of systemic sclerosis (SSc). To enhance our knowledge about SSc in African Americans, we established a comprehensive clinical database from the largest multicenter cohort of African American SSc patients assembled to date (the Genome Research in African American Scleroderma Patients (GRASP) cohort).African American SSc patients were enrolled retrospectively and prospectively over a 30-year period (1987-2016), from 18 academic centers throughout the United States. The cross-sectional prevalence of sociodemographic, clinical, and serological features was evaluated...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29359289/-renal-involvement-in-connective-tissue-diseases
#9
Stefan Markus Weiner
Renal involvement is common and heterogenous in connective tissue diseases and has a main influence on prognosis and mortality. In systemic lupus erythematosus proliferative glomerulonephritis is the most common manifestation, while in primary Sjogren's syndrome interstitial nephritis with tubular dysfunction is the predominant pathological feature. In systemic sclerosis the most serious renal manifestation is scleroderma renal crisis characterized by abrupt onset of hypertension and acute kidney injury associated with an increase in plasma renin activity...
January 2018: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/29346637/scleroderma-renal-crisis-and-cancer
#10
Colombe Crosnier, Aurélie Achille, Marie Lecouffe-Desprets, Antoine Néel, Cécile Durant, Christian Agard
We report two cases of elderly patients with diffuse systemic sclerosis (SSc) and inaugural scleroderma renal crisis (SRC). Initial exhaustive search for an underlying cancer was negative, but aggressive metastatic neoplasia was diagnosed after 1 year of follow-up. We emphasize the role of 18-FDG PET-scan to detect neoplasia in the following months after inaugural SRC in elderly.
January 15, 2018: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/29214548/first-clinical-symptom-as-a-prognostic-factor-in-systemic-sclerosis-results-of-a-retrospective-nationwide-cohort-study
#11
Manuel Rubio-Rivas, Xavier Corbella, Melany Pestaña-Fernández, Carles Tolosa-Vilella, Alfredo Guillen-Del Castillo, Dolores Colunga-Argüelles, Luis Trapiella-Martínez, Nerea Iniesta-Arandia, María Jesús Castillo-Palma, Luis Sáez-Comet, María Victoria Egurbide-Arberas, Norberto Ortego-Centeno, Mayka Freire, Jose Antonio Vargas-Hitos, Juan José Ríos-Blanco, Jose Antonio Todolí-Parra, Mónica Rodríguez-Carballeira, Adela Marín-Ballvé, Pablo Segovia-Alonso, Xavier Pla-Salas, Ana Belén Madroñero-Vuelta, Manuel Ruiz-Muñoz, Vicent Fonollosa-Pla, Carmen Pilar Simeón-Aznar, E Callejas Moraga, E Calvo, C Carbonell, M J Castillo, A J Chamorro, D Colunga, X Corbella, M V Egurbide, G Espinosa, V Fonollosa, M Freire, F J García Hernández, R González León, A Guillén Del Castillo, N Iniesta, R Lorenzo, A B Madroñero, B Marí, A Marín, N Ortego-Centeno, M Pérez Conesa, M Pestaña, X Pla, J J Ríos Blanco, M Rodríguez Carballeira, M Rubio Rivas, M Ruiz Muñoz, L Sáez Comet, P Segovia, C P Simeón, A Soto, E Tarí, J A Todolí, C Tolosa, L Trapiella, J A Vargas Hitos, G Verdejo
The objective of the study is to determine the importance of the mode of onset as prognostic factor in systemic sclerosis (SSc). Data were collected from the Spanish Scleroderma Registry (RESCLE), a nationwide retrospective multicenter database created in 2006. As first symptom, we included Raynaud's phenomenon (RP), cutaneous sclerosis, arthralgia/arthritis, puffy hands, interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), and digestive hypomotility. A total of 1625 patients were recruited...
December 7, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29145709/review-defining-a-unified-vascular-phenotype-in-systemic-sclerosis
#12
REVIEW
Yannick Allanore, Oliver Distler, Marco Matucci-Cerinic, Christopher P Denton
Microcirculation impairment and related vasculopathy are hallmarks of systemic sclerosis (SSc). Digital ulceration is second only to Raynaud's phenomenon as a vascular complication occurring in patients with SSc. Digital ulcers are painful and generate disability. Furthermore, patients may develop recurrent digital ulcers, and it is reasonable to question whether the outcomes of such patients might be different from those of patients who are not affected. Recently, several registries have provided relevant information about digital ulcers...
February 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29051891/scleroderma-renal-crisis-in-mixed-connective-tissue-disease-with-full-renal-recovery-within-3-months-a-case-report-with-expanding-treatment-modalities-to-treat-each-clinical-sign-as-an-independent-entity
#13
Jordana Cheta, Suresh Rijhwani, Harlan Rust
Mixed connective tissue disease (MCTD) is a rheumatologic overlap syndrome that can present with symptoms of systemic lupus erythematous, scleroderma, and polymyositis. A severe but rare complication that can occur in MCTD is scleroderma renal crisis. With multiple poor prognostic indicators, the renal outcome is usually poor. The clinical and histological picture is one of a thrombotic microangiopathy. Clinical suspicion has to be high for additional thrombotic or autoimmune processes coexisting due to associated morbidity...
October 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28992172/points-to-consider-in-renal-involvement-in-systemic-sclerosis
#14
REVIEW
Felice Galluccio, Ulf Müller-Ladner, Daniel E Furst, Dinesh Khanna, Marco Matucci-Cerinic
This article discusses points to consider when undertaking a clinical trial to test therapy for renal involvement in SSc, not including scleroderma renal crisis. Double-blind, randomized controlled trials vs placebo or standard background therapy should be strongly considered. Inclusion criteria should consider a pre-specified range of renal functions or stratification of renal function. Gender and age limitations are probably not necessary. Concomitant medications including vasodilators, immunosuppressants and endothelin receptor antagonists and confounding illnesses such as diabetes, kidney stones, hypertension and heart failure need to be considered...
September 1, 2017: Rheumatology
https://www.readbyqxmd.com/read/28980912/influence-of-antibody-profile-in-clinical-features-and-prognosis-in-a-cohort-of-spanish-patients-with-systemic-sclerosis
#15
Nerea Iniesta Arandia, Carmen Pilar Simeón-Aznar, Alfredo Guillén Del Castillo, Dolores Colunga Argüelles, Manuel Rubio-Rivas, Luis Trapiella Martínez, Francisco José García Hernández, Luis Sáez Comet, María Victoria Egurbide Arberas, Norberto Ortego-Centeno, Mayka Freire, Begoña Marí Alfonso, José Antonio Vargas Hitos, Juan José Ríos Blanco, José Antonio Todolí Parra, Monica Rodríguez-Carballeira, Adela Marín Ballvé, Antonio Javier Chamorro Fernández, Xavier Pla Salas, Ana Belen Madroñero Vuelta, Manuel Ruiz Muñoz, Vicent Fonollosa Pla, Gerard Espinosa
OBJECTIVES: To assess the clinical manifestations and prognosis of Spanish patients with systemic sclerosis (SSc) according to their immunological profile. METHODS: From the Spanish Scleroderma Study Group or RESCLE (Registro de ESCLErodermia as Spanish nomenclature) Registry we selected those patients in which anti-centromere (ACA), anti-topoisomerase I (ATA), and anti-RNA polymerase III (ARA) antibodies had been determined, and a single positivity for each SSc specific antibody was detected...
September 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28942404/successful-treatment-using-rituximab-in-a-patient-with-refractory-polymyositis-complicated-by-scleroderma-renal-crisis
#16
Kyoko Innami, Tomoyuki Mukai, Shoko Kodama, Yoshitaka Morita
Corticosteroids are the first-line treatment for patients with inflammatory myopathies. Myositis can be a clinical feature of scleroderma (polymyositis-scleroderma overlap syndrome), and treatment of this syndrome is a challenge for clinicians because moderate to high doses of corticosteroids are considered a risk factor for development of acute kidney injury in affected patients. We report here the case of a 56-year-old woman with scleroderma who developed polymyositis and was successfully treated with rituximab...
September 23, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28869411/the-presence-of-small-joint-contractures-is-a-risk-factor-for-survival-in-439-patients-with-systemic-sclerosis
#17
Gabriella Nagy, Tünde Minier, Cecília Varjú, Réka Faludi, Katalin T Kovács, Veronika Lóránd, Veronika Hermann, László Czirják, Gábor Kumánovics
OBJECTIVES: Analysis of risk factors and mortality of 439 patients with systemic sclerosis (SSc) in a tertiary care centre. METHODS: The mean follow up time was 8.4±5.6 years. Lost to follow up rate was 6.4%. Female to male ratio was 366 to 73. Two hundred sixty patients had limited and 179 diffuse cutaneous SSc (dcSSc). A standard protocol including musculoskeletal examinations was used for the assessment of patients. RESULTS: By Kaplan-Meier analysis the overall 5-, 10- and 15 year survival were 88...
September 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28752678/risk-factors-and-outcome-of-thai-patients-with-scleroderma-renal-crisis-a-disease-duration-matched-case-control-study
#18
Suparaporn Wangkaew, Supawita Lertthanaphok, Saowanee Puntana, Kajohnsak Noppakun
INTRODUCTION: Data regarding the prevalence, risk factors and outcome of scleroderma renal crisis (SRC) in Asian patients with systemic sclerosis (SSc) are limited. OBJECTIVE: To determine the prevalence, risk factors and outcomes of SRC in Thai SSc patients. METHOD: Medical records of all SSc patients seen at the Division of Rheumatology, Chiang Mai University, Thailand, from January 1990 to December 2015 were retrospectively reviewed. For each SRC case, a disease duration (±1 year) matched control (four SSc patient without SRC for each SRC patient) was identified...
July 27, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28580628/mortality-in-the-waikato-hospital-systemic-sclerosis-cohort
#19
Clement Ooi, Kamal Solanki, Chunhuan Lao, Chris Frampton, Douglas White
OBJECTIVE: To characterize the causes of mortality and standardised mortality ratio in a cohort of patients with systemic sclerosis (SSc). METHODS: A cohort of 132 patients enrolled at the Waikato Systemic Sclerosis Clinic was prospectively followed from 2005 to 2016. Patient demographics, diagnoses and laboratory reports were used to assess risk of mortality and generate standardised mortality ratios (SMR). Survival was analyzed using Kaplan-Meier methods. RESULTS: Of the cohort of 132 patients, 20 (15%) were deceased by the end of the study period...
January 2018: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28413064/systemic-sclerosis
#20
REVIEW
Christopher P Denton, Dinesh Khanna
Systemic sclerosis, also called scleroderma, is an immune-mediated rheumatic disease that is characterised by fibrosis of the skin and internal organs and vasculopathy. Although systemic sclerosis is uncommon, it has a high morbidity and mortality. Improved understanding of systemic sclerosis has allowed better management of the disease, including improved classification and more systematic assessment and follow-up. Additionally, treatments for specific complications have emerged and a growing evidence base supports the use of immune suppression for the treatment of skin and lung fibrosis...
October 7, 2017: Lancet
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