Results 51 to 60 of about 18,247 (232)

A case of Myhre syndrome mimicking juvenile scleroderma

Pediatric Rheumatology Online Journal, 2020
Background Myhre syndrome is a genetic disorder caused by gain of function mutations in the SMAD Family Member 4 (SMAD4) gene, resulting in progressive, proliferative skin and organ fibrosis.
Barbara Jensen, Rebecca James, Ying Hong, Ebun Omoyinmi, Clarissa Pilkington, Neil J. Sebire, Kevin J. Howell, Paul A. Brogan, Despina Eleftheriou   +8 more
doaj   +1 more source

Molecular subsets in the gene expression signatures of scleroderma skin. [PDF]

PLoS ONE, 2008
Scleroderma is a clinically heterogeneous disease with a complex phenotype. The disease is characterized by vascular dysfunction, tissue fibrosis, internal organ dysfunction, and immune dysfunction resulting in autoantibody production.We analyzed the ...
Ausra Milano, Sarah A Pendergrass, Jennifer L Sargent, Lacy K George, Timothy H McCalmont, M Kari Connolly, Michael L Whitfield   +6 more
doaj   +1 more source

Real‐World Safety and Effectiveness of JAK Inhibitors in Systemic Sclerosis: A Propensity‐Matched Study From the EUSTAR Cohort

Arthritis Care &Research, EarlyView.
Objective JAK inhibitors (JAKi) have shown promising effects in early‐phase studies of systemic sclerosis (SSc). We aimed to assess the safety and explore the effectiveness of JAKi compared to conventional immunosuppressants in SSc. Methods A longitudinal retrospective study of the European Scleroderma Trials and Research Group (EUSTAR) cohort was ...
Stefano Di Donato, Marie‐Elise Truchetet, Marco Minerba, Oliver Distler, Juan José Alegre Sancho, Yolanda Braun‐Moscovici, Christina Bergmann, Petros Sfikakis, Jeska K. de Vries‐Bouwstra, Murray Baron, Silvia Bellando‐Randone, Lorenzo Dagna, Christopher P. Denton, Madelon C. Vonk, Vanessa Smith, Ivan Castellvi, Gabriela Riemekasten, Andra Balanescu, Masataka Kuwana, Maria De Santis, Kamal Solanki, Anastas Batalov, Vahan Mukuchyan, Marco Matucci‐Cerinic, Yannick Allanore, Francesco Del Galdo, Michael Hughes, EUSTAR Collaborators   +27 more
wiley   +1 more source

Comparison between clinical and immunological features of scleroderma with and without interstitial lung disease

Journal of the Pakistan Medical Association
The study was conducted at the Fauji Foundation Hospital, Rawalpindi, Pakistan, from January 1, 2023, to December 31, 2023, and comprised 20 female patients ages 18-55 years having scleroderma. Overall, 14(70%) patients had diffuse scleroderma and 6(30%)
Muhammad Salman Mushtaq, Haris Gul, Muhammad Umer Daraz, Muhammad khan, Saba Samreen   +4 more
doaj   +1 more source

Correlation Between Skin and Affected Organs in 52 Sclerodermic Patients Followed in a Diseases Management Team: Development of a Risk Prediction Model of Organ-Specific Complications

Frontiers in Immunology, 2021
ObjectiveTo identify the existence of a correlation among the various organs affected, focusing primarily on immuno-dermatological aspects, and to create a risk prediction model of organ-specific complications.Material and MethodsFifty-two patients with ...
Emanuele Cozzani, Andrea Muracchioli, Giuseppe Murdaca, Mirko Beccalli, Simone Caprioli, Patrizia Zentilin, Pietro Ameri, Marco Grosso, Rodolfo Russo, Luca Carmisciano, Aurora Parodi   +10 more
doaj   +1 more source

Engagement Patterns with an AI Health Coach for Systemic Sclerosis Self‐Management: A Mixed Methods Study

Arthritis Care &Research, Accepted Article.
Objective To evaluate utility of an artificial intelligence (AI) health coach for systemic sclerosis (SSc) self‐management and identify patterns associated with participant engagement. Methods We conducted a mixed‐methods study in which an AI health coach, powered by a large language model (LLM), was used to support self‐management for SSc.
Nirali Shah, Melanie Morris, Cristina Daraban, Sophia Johnson, Susan L. Murphy   +4 more
wiley   +1 more source

Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study

, 2018
Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational ...
Ong VH, Buch MH, Carreira PE, Farge D, Balbir-Gurman A, Fligelstone K, Moinzadeh P, Distler O, Brown E, Hall FC, Damjanov N, Vonk M, Sheeran T, McLaren JS, Sahhar J, van Laar JM, Mouthon L, Czirják L, Proudman S, Matucci-Cerinic M, Riemekasten G, Trad GS, Denton CP, Ahmad Y, Distler JHW, Hamilton J, Stevens W, Becker M, Chakravarty K, Serratrice C, Coppere B, Lanyon P, Grange C, Lunt M, Midtvedt Ø, Herrick AL., Gunawardena H, MacGregor AJ, Anderson ME, Madhok R, Gregory WJ, Hachulla E, Akil M, Peytrignet S, Hesselstrand R, Pan X, Jordan AC, Chung L, Pathare S, Diot E, Silman A, Hudson M, McHugh N, Inanç M, Agard C, Müller-Ladner U, Ochiel R, Veale DJ, Fauchais AL, Ancuta C, Jacobsen S, Roddy J, Rudin A   +62 more
core   +1 more source

A rare cutaneous manifestation of systemic sclerosis

European Journal of Case Reports in Internal Medicine
A 43-year-old male with a history of intravenous drug use and alcohol consumption presented to the emergency department with three-month history of failure to thrive.
Joelle Sleiman, Taimur Aslam, Anastasia Slobodnick, Alina G. Bridges   +3 more
doaj   +1 more source

Perivascular Matrix Densification Dysregulates Angiogenesis and Activates Pro‐Inflammatory Endothelial Cells

Advanced Science, EarlyView.
Perivascular matrix densification promotes the emergence of aberrant endothelial tip cells (ATECs) that invade and persist within fibrotic microenvironments. Using in vivo lineage tracing and a human microvessel model, this study shows that fibrous matrix cues destabilize VE‐cadherin–mediated junctions to gate TGF‐β signaling, inducing a pro ...
Jingyi Xia, William Y. Wang, Kyle A. Jacobs, Kairav Maniar, Daphne Lin, Evan H. Jarman, Daniel L. Matera, Kristen Loesel, Christopher D. Davidson, Harrison L. Hiraki, Xiaotian Tan, Eve H. Shikanov, Robert N. Kent, Carole Parent, Xudong Fan, Ariella Shikanov, Matthew L. Kutys, Brendon M. Baker   +17 more
wiley   +1 more source

Evaluating the cutaneous involvement in scleroderma : Torsional stiffness revisited

, 2001
Objective: The pace, progression and extent of the skin lesions in scleroderma may parallel the risk of new internal organ involvement and the progression of existing internal lesions.
Helliwell, Phillip S., Isdale, Amanda H., Smeathers, James, Knight, Linda R.   +3 more
core   +1 more source