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https://www.readbyqxmd.com/read/29765964/a-rare-syndrome-resembling-scleroderma-huriez-syndrome
#1
Nil Su Çelik, Şirin Yaşar, Sema Aytekin, Pembegül Güneş
Huriez syndrome, also referred to as "sclerotylosis," is a rare autosomal dominant genodermatosis characterized by the triad of congenital scleroatrophy of the distal extremities, palmoplantar keratoderma, and hypoplastic nails. The development of aggressive squamous cell carcinoma (SCC) arising in the scleroatrophic area is also a distinctive feature of the syndrome. Early diagnosis is important due to the early onset, mostly in the third to fourth decades of life, and aggressive progress of SCC, which occurs in around 15% of affected individuals...
April 2018: Skin Appendage Disorders
https://www.readbyqxmd.com/read/29686439/clinical-pattern-of-systemic-sclerosis-in-central-ukraine-association-between-clinical-manifestations-of-systemic-sclerosis-and-hypertension
#2
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/29678941/autoantibodies-and-scleroderma-phenotype-define-subgroups-at-high-risk-and-low-risk-for-cancer
#3
Takeru Igusa, Laura K Hummers, Kala Visvanathan, Carrie Richardson, Fredrick M Wigley, Livia Casciola-Rosen, Antony Rosen, Ami A Shah
OBJECTIVES: Recent studies demonstrate autoantibodies are powerful tools to interrogate molecular events linking cancer and the development of autoimmunity in scleroderma. Investigating cancer risk in these biologically relevant subsets may provide an opportunity to develop personalised cancer screening guidelines. In this study, we examined cancer risk in distinct serologic and phenotypic scleroderma subsets and compared estimates with the general population. METHODS: Patients in the Johns Hopkins Scleroderma Center observational cohort were studied...
April 20, 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29666638/from-localized-scleroderma-to-systemic-sclerosis-coexistence-or-possible-evolution
#4
Giuggioli Dilia, Colaci Michele, Cocchiara Emanuele, Spinella Amelia, Lumetti Federica, Ferri Clodoveo
Background: Systemic sclerosis (SSc) and localized scleroderma (LoS) are two different diseases that may share some features. We evaluated the relationship between SSc and LoS in our case series of SSc patients. Methods: We analysed the clinical records of 330 SSc patients, in order to find the eventual occurrence of both the two diseases. Results: Eight (2.4%) female patients presented both the two diagnoses in their clinical histories...
2018: Dermatology Research and Practice
https://www.readbyqxmd.com/read/29655520/comparing-ultraviolet-light-a-photo-chemo-therapy-with-methotrexate-protocol-in-childhood-localized-scleroderma-evidence-from-systematic-review-and-meta-analysis-approach
#5
REVIEW
Edoardo Marrani, Ivan Foeldvari, Jordi Anton Lopez, Rolando Cimaz, Gabriele Simonini
OBJECTIVE: Localized scleroderma is a skin fibrosing disorder that, if untreated, may result in severe disability. The purpose of this systematic review is to compare the present evidence concerning the effectiveness of Methotrexate versus phototherapy, alone or associated with Psoralen, in childhood localized scleroderma. METHOD: A systematic search between January 1996 and May 2017 was performed to identify studies investigating the efficacy of Methotrexate (MTX) or phototherapy (UVA) for treating localized scleroderma with onset ≤18 years...
March 14, 2018: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29650377/articular-surgery-of-the-ischemic-hand-in-systemic-scleroderma-a-vascular-basis-for-arthrodesis-and-arthroplasty
#6
REVIEW
Charles P Melone, Erez Dayan
Hallmark deformities of systemic scleroderma are early onset and progressively disabling flexion contractures of the proximal interphalangeal (PIP) joints often in conjunction with extension or, less frequently, flexion contractures of the metacarpophalangeal (MCP) joints. Although surgical correction is generally recommended, a prevailing reluctance for operative treatment exists owing to the inherent ischemia of the disease with its potentially compromised healing capacity. Nonetheless, with recognition and preservation of the tenuous but well-defined and constant periarticular vascular networks of the PIP and MCP joints, articular reconstruction with uncomplicated wound healing can prove consistently successful for patients with scleroderma...
April 9, 2018: Journal of Hand Surgery
https://www.readbyqxmd.com/read/29536565/chronic-cutaneous-graft-versus-host-disease-in-children-a-report-of-14-patients-from-a-tertiary-care-pediatric-dermatology-clinic
#7
Arti Nanda, Maitham A A Husain, Waleed Al-Herz, Adla Almekaimi, Humoud Al-Sabah, Mohammad Al-Otaibi
BACKGROUND/OBJECTIVES: Allogeneic hematopoietic stem cell transplantation (HSCT) is a treatment option for many life-threatening disorders in children. Chronic graft-versus-host disease (cGVHD) is a significant complication of HSCT, and its treatment is challenging. Skin is the most common organ affected in cGVHD, with protean manifestations posing a challenge in diagnosis and management. The objective was to have a better understanding of the spectrum of chronic cutaneous GVHD (cc-GVHD) in children...
May 2018: Pediatric Dermatology
https://www.readbyqxmd.com/read/29425538/-etiological-profile-of-secondary-raynaud-s-phenomenon-in-an-internal-medicine-department-about-121-patients
#8
T Ben Salem, M Tougorti, S Bziouech, M Lamloum, M Khanfir, I Ben Ghorbel, M H Houman
INTRODUCTION: Raynaud's phenomenon is a reversible episodic vasospastic disorder triggered by cold or emotion. Two types of Raynaud's phenomenon were distinguished: Raynaud's disease and secondary Raynaud's phenomenon. The purpose of this study was to determine the etiologic profile of secondary Raynaud's phenomenon in an internal medicine department. METHODS: A descriptive retrospective study including patients with secondary Raynaud's phenomenon followed in a tertiary internal medicine department between 2000 and 2013...
February 2018: Journal de Médecine Vasculaire
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
#9
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
#10
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/29339444/light-hvem-signaling-in-keratinocytes-controls-development-of-dermatitis
#11
Rana Herro, Jr-Wen Shui, Sonja Zahner, Daniel Sidler, Yuko Kawakami, Toshiaki Kawakami, Koji Tamada, Mitchell Kronenberg, Michael Croft
Dermatitis is often associated with an allergic reaction characterized by excessive type 2 responses leading to epidermal acanthosis, hyperkeratosis, and dermal inflammation. Although factors like IL-4, IL-13, and thymic stromal lymphopoietin (TSLP) are thought to be instrumental for the development of this type of skin disorder, other cytokines may be critical. Here, we show that the tumor necrosis factor (TNF) superfamily protein LIGHT (homologous to lymphotoxin, exhibits inducible expression, and competes with HSV glycoprotein D for binding to HVEM, a receptor expressed on T lymphocytes) is required for experimental atopic dermatitis, and LIGHT directly controls keratinocyte hyperplasia, and production of periostin, a matricellular protein that contributes to the clinical features of atopic dermatitis as well as other skin diseases such as scleroderma...
February 5, 2018: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/29333701/the-childhood-arthritis-and-rheumatology-research-alliance-consensus-treatment-plans-toward-comparative-effectiveness-in-the-pediatric-rheumatic-diseases
#12
Sarah Ringold, Peter A Nigrovic, Brian M Feldman, George A Tomlinson, Emily von Scheven, Carol A Wallace, Adam M Huber, Laura E Schanberg, Suzanne C Li, Pamela F Weiss, Robert C Fuhlbrigge, Esi M Morgan, Yukiko Kimura
The pediatric rheumatic diseases are a heterogeneous group of rare diseases, posing a number of challenges for the use of traditional clinical and translational research methods. Innovative comparative effectiveness approaches are needed to efficiently study treatment strategies and disease outcomes. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed the consensus treatment plan (CTP) approach as a comparative effectiveness tool for research in pediatric rheumatology. CTPs are treatment strategies, developed by consensus methods among CARRA members, intended to reduce variation in treatment approaches, standardize outcome measurements, and allow for comparison of the effectiveness of different approaches with the goal of improving disease outcomes...
January 15, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29322341/interstitial-lung-disease-in-systemic-sclerosis-data-from-the-spanish-scleroderma-study-group
#13
D Sánchez-Cano, N Ortego-Centeno, J L Callejas, V Fonollosa Plá, R Ríos-Fernández, C Tolosa-Vilella, G Espinosa-Garriga, D Colunga-Argüelles, M V Egurbide-Arberas, M Rubio-Rivas, M Freire, J J Ríos-Blanco, L Trapiella-Martínez, M Rodríguez-Carballeira, A Marín-Ballvé, X Pla-Salas, C P Simeón-Aznar
OBJECTIVES: To evaluate the clinical characteristics of patients with interstitial lung disease (ILD) in the setting of a large cohort of systemic sclerosis (SSc) patients, and to analyse the differences according to the SSc subtype (following the modification of classification criteria of the American College of Rheumatology for SSc proposed by LeRoy and Medsger), factors are associated with moderate-to-severe impairment of lung function, as well as mortality and causes of death. METHODS: A descriptive study was performed, using the available data from the Spanish Scleroderma Study Group...
March 2018: Rheumatology International
https://www.readbyqxmd.com/read/29246416/hepatobiliary-involvement-in-systemic-sclerosis-and-the-cutaneous-subsets-characteristics-and-survival-of-patients-from-the-spanish-rescle-registry
#14
Begoña Marí-Alfonso, Carmen Pilar Simeón-Aznar, Alfredo Guillén-Del Castillo, Manuel Rubio-Rivas, Luis Trapiella-Martínez, José Antonio Todolí-Parra, Mónica Rodríguez Carballeira, Adela Marín-Ballvé, Nerea Iniesta-Arandia, Dolores Colunga-Argüelles, María Jesús Castillo-Palma, Luis Sáez-Comet, María Victoria Egurbide-Arberas, Norberto Ortego-Centeno, Mayka Freire, José Antonio Vargas Hitos, Antonio-J Chamorro, Ana Belen Madroñero-Vuelta, Isabel Perales-Fraile, Xavier Pla-Salas, Rafael A Fernández-De-La-Puebla, Vicent Fonollosa-Pla, Carles Tolosa-Vilella
OBJECTIVE: To assess the prevalence and causes of hepatobiliary involvement (HBI) in systemic sclerosis (SSc), to investigate the clinical characteristics and prognosis of SSc patients with HBI (SSc-HBI) and without HBI (SSc-non-HBI), and to compare both groups according to the cutaneous SSc subsets. METHODS: In all, 1572 SSc patients were collected in the RESCLE registry up to January 2015, and all hepatobiliary disturbances were recorded. We investigated the HBI-related characteristics and survival from the entire SSc cohort and according to the following cutaneous subsets: diffuse cutaneous SSc (dcSSc), limited cutaneous SSc (lcSSc), and SSc sine scleroderma (ssSSc)...
October 6, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29214548/first-clinical-symptom-as-a-prognostic-factor-in-systemic-sclerosis-results-of-a-retrospective-nationwide-cohort-study
#15
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/29196241/scleroderma-skin-ulcers-definition-classification-and-treatment-strategies-our-experience-and-review-of-the-literature
#16
REVIEW
Dilia Giuggioli, Andreina Manfredi, Federica Lumetti, Michele Colaci, Clodoveo Ferri
BACKGROUND: Skin ulcers (SU) are one of the most frequent manifestations of systemic sclerosis (SSc). SSc-SU are very painful, often persistent and recurrent; they may lead to marked impairment of patient's activities and quality of life. Despite their severe impact on the whole SSc patient's management, the proposed definition, classification criteria, and therapeutic strategies of SSc-SU are still controversial. OBJECTIVE: The present study aimed to elaborate a comprehensive proposal of definition, classification, and therapeutic strategy of SSc-SU on the basis of our long-term single center experience along with a careful revision of the world literature on the same topic...
February 2018: Autoimmunity Reviews
https://www.readbyqxmd.com/read/29164837/serum-surfactant-protein-d-and-exhaled-nitric-oxide-as-biomarkers-of-early-lung-damage-in-systemic-sclerosis
#17
Alida Benfante, Riccardo Messina, Alessandra Paternò, Nicola Scichilone
BACKGROUND: Interstitial lung disease (ILD) complicates the course of systemic sclerosis (SSc), representing the main cause of death in these patients. The identification of parameters that can predict the early onset and progression of ILD in SSc represents an unmet need in clinical practice. The study was designed to explore whether the surfactant proteins (SP) A and D may be used as noninvasive tools for the early identification of ILD in SSc. Alveolar exhaled nitric oxide (NO) was investigated as a surrogate marker of distal inflammation...
April 2018: Minerva Medica
https://www.readbyqxmd.com/read/29155994/work-productivity-in-systemic-sclerosis-its-economic-burden-and-association-with-health-related-quality-of-life
#18
Kathleen Morrisroe, Vijaya Sudararajan, Wendy Stevens, Joanne Sahhar, Jane Zochling, Janet Roddy, Susanna Proudman, Mandana Nikpour
Objective: To evaluate work productivity and its economic burden in SSc patients. Methods: Consecutive SSc patients enrolled in the Australian Scleroderma Cohort Study were mailed questionnaires assessing employment (Workers' Productivity and Activity Impairment Questionnaire and a custom-made questionnaire) and health-related quality of life (HRQoL) (36-item Short Form Health Survey and Patient-Reported Outcomes Measurement Information System 29). Linear regression methods were used to determine factors associated with work productivity...
January 1, 2018: Rheumatology
https://www.readbyqxmd.com/read/28990485/there-is-a-need-for-new-systemic-sclerosis-subset-criteria-a-content-analytic-approach
#19
S R Johnson, M L Soowamber, J Fransen, D Khanna, F Van Den Hoogen, M Baron, M Matucci-Cerinic, C P Denton, T A Medsger, P E Carreira, G Riemekasten, J Distler, A Gabrielli, V Steen, L Chung, R Silver, J Varga, U Müller-Ladner, M C Vonk, U A Walker, F A Wollheim, A Herrick, D E Furst, L Czirjak, O Kowal-Bielecka, F Del Galdo, M Cutolo, N Hunzelmann, C D Murray, I Foeldvari, L Mouthon, N Damjanov, B Kahaleh, T Frech, S Assassi, L A Saketkoo, J E Pope
OBJECTIVES: Systemic sclerosis (SSc) is heterogenous. The objectives of this study were to evaluate the purpose, strengths and limitations of existing SSc subset criteria, and identify ideas among experts about subsets. METHODS: We conducted semi-structured interviews with randomly sampled international SSc experts. The interview transcripts underwent an iterative process with text deconstructed to single thought units until a saturated conceptual framework with coding was achieved and respondent occurrence tabulated...
October 9, 2017: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/28951708/morphea-in-middle-anatolia-turkey-a-5-year-single-center-experience
#20
Isil Bulur, Hilal Kaya Erdoğan, Tekden Karapınar, Zeynep Nurhan Saracoglu
INTRODUCTION: Morphea, also referred to as localized scleroderma, is a rare fibrosing skin disorder of undetermined cause. AIM: We report our single-center experience with morphea. MATERIAL AND METHODS: The study included 53 patients who were diagnosed with morphea by histopathology in our department between 2010 and 2015. Study data were collected retrospectively from the records of morphea patients. RESULTS: The study included 53 patients (38 women, 15 men), and median age at onset was 39...
August 2017: Postȩpy Dermatologii i Alergologii
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