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scleroderma onset

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...
March 14, 2018: Pediatric Dermatology
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
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)
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
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
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 approaches. Innovative comparative effectiveness approaches are needed to efficiently study treatment approaches 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
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
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
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
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
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...
November 21, 2017: Minerva Medica
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...
October 9, 2017: Rheumatology
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
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
Kenji Izuhara, Satoshi Nunomura, Yasuhiro Nanri, Masahiro Ogawa, Junya Ono, Yasutaka Mitamura, Tomohito Yoshihara
We found for the first time that IL-4 and IL-13, signature type 2 cytokines, are able to induce periostin expression. We and others have subsequently shown that periostin is highly expressed in chronic inflammatory diseases-asthma, atopic dermatitis, eosinophilc chronic sinusitis/chronic rhinosinusitis with nasal polyp, and allergic conjunctivitis-and that periostin plays important roles in the pathogenesis of these diseases. The epithelial/mesenchymal interaction via periostin is important for the onset of allergic inflammation, in which periostin derived from fibroblasts acts on epithelial cells or fibroblasts, activating their NF-κB...
December 2017: Cellular and Molecular Life Sciences: CMLS
Zoltan Barth, Birgit N Witczak, Berit Flatø, Akos Koller, Ivar Sjaastad, Helga Sanner
OBJECTIVE: In juvenile dermatomyositis (JDM), microvascular abnormalities measured by nailfold capillaroscopy (NFC) are common early in disease course. We aimed to compare the presence of NFC abnormalities in patients with medium- to longstanding JDM with that of controls, and to explore associations between NFC abnormalities and disease activity and other disease characteristics. METHODS: Fifty-eight JDM patients clinically examined median 16.8 (2-38) years after disease onset were compared with matched controls...
August 13, 2017: Arthritis Care & Research
Patricia E Carreira, Loreto Carmona, Beatriz E Joven, Estibaliz Loza, Jose Luis Andreu, Gabriela Riemekasten, Serena Vettori, Yannick Allanore, Alexandra Balbir-Gurman, Paolo Airò, Ulrich A Walker, Nemanja Damjanov, Lidia P Ananieva, Simona Rednic, László Czirják, Oliver Distler, Dominique Farge, Roger Hesselstrand, Ada Corrado, Paola Caramaschi, Mohammed Tikly, Marco Matucci-Cerinic
OBJECTIVES: To explore the prevalence and clinical associations of elevated systolic pulmonary artery pressure (sPAP), measured by Transthoracic Doppler-echocardiography (TTE) in patients with early systemic sclerosis (SSc). METHODS: A cross-sectional analysis of the prospective EULAR Scleroderma Trial and Research (EUSTAR) database was performed. SSc patients with <3 years from the first non-Raynaud's phenomenon (RP) symptom at baseline EUSTAR visit, were selected...
September 2017: Clinical and Experimental Rheumatology
Ami A Shah, Antony Rosen, Laura K Hummers, Betty J May, Alpana Kaushiva, Richard B S Roden, Deborah K Armstrong, Fredrick M Wigley, Livia Casciola-Rosen, Kala Visvanathan
OBJECTIVES: Systemic sclerosis (scleroderma) and dermatomyositis are two prototypic autoimmune diseases that are strongly associated with malignancy. While specific autoantibodies in these diseases are markers of an increased risk of cancer at scleroderma and dermatomyositis onset, it is not known whether these autoantibodies are biomarkers of cancer risk in patients without rheumatic disease. METHODS: In a matched case-control study of women without rheumatic disease, identified from a familial breast cancer cohort, 50 breast cancer cases and 50 controls were assayed for 3 autoantibodies that are known markers of cancer-associated scleroderma and dermatomyositis: anti-RNA polymerase III, anti-NXP2, and anti-TIF1γ...
September 2017: Clinical and Experimental Rheumatology
Luis Trapiella-Martínez, José Bernardino Díaz-López, Luis Caminal-Montero, Carles Tolosa-Vilella, Alfredo Guillén-Del Castillo, Dolores Colunga-Argüelles, Manuel Rubio-Rivas, 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, Antonio Javier Chamorro-Fernández, Xavier Pla-Salas, Ana Belén Madroñero-Vuelta, Manuel Ruiz-Muñóz, Vicent Fonollosa-Pla, Carmen Pilar Simeón-Aznar
OBJECTIVES: According to the existence of subclinical organ involvement pre-scleroderma should be divided into two subsets: very early and early disease. Pre-scleroderma patients included in the Spanish Scleroderma Registry (RESCLE) Cohort were reclassified into subsets. Differences were evaluated and the risk of progression to definite systemic sclerosis was estimated. METHODS: The characteristics of very early and early SSc patients were compared. A logistic regression model was used to determine the risk factors of progression...
August 2017: Autoimmunity Reviews
Masataka Kuwana
The presence of circulating anti-nuclear antibodies (ANAs) is a hallmark of immune dysregulation in patients with systemic sclerosis (SSc). Currently, a variety of SSc-specific ANAs, including anticentromere, anti-topoisomerase I, and anti-RNA polymerase III antibodies, have been well characterized, and their commercial kits are available worldwide. Since these autoantibodies are specifically detected in SSc patients and are associated with unique sets of disease manifestations, they are widely used in routine clinical practice for diagnosis, clinical subgrouping, and prediction of future organ involvements and prognosis...
2017: Journal of Nippon Medical School, Nippon Ika Daigaku Zasshi
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