Article
Rheumatology
Brian Skaug, Xinjian Guo, Yuanteng Jeff Li, Julio Charles, Kay T. Pham, Jacob Couturier, Dorothy E. Lewis, Claudia Bracaglia, Ivan Caiello, Maureen D. Mayes, Shervin Assassi
Summary: The R206C polymorphism in the coding region of DNASE1L3 gene impairs its ability to digest extracellular DNA, leading to reduced digestion of circulating DNA in plasma from SSc patients.
Article
Rheumatology
Robyn T. Domsic, Thomas A. Medsger, Shiyao Gao, Maureen Laffoon, Suiyuan Huang, Stephen Wisniewski, Cathie Spino, Virginia Steen, Robert Lafyatis, Dinesh Khanna
Summary: By analyzing clinical characteristics and laboratory values, we identified strategies to improve trial design for early diffuse SSc, including skin thickness progression rate, RNA polymerase III antibody positivity, and presence of tendon friction rubs. Applying these strategies to actual trial data can reduce the decline in placebo group skin scores and reveal significant differences in the CRISS score.
Article
Rheumatology
Robyn T. Domsic, Shiyao Gao, Maureen Laffoon, Steven Wisniewski, Yuqing Zhang, Virginia Steen, Robert Lafyatis, Thomas A. Medsger
Summary: Regardless of whether the first SSc manifestation or first non-Raynaud manifestation is used to define disease onset, a disease duration of less than 18 months at enrollment is preferable. A longer disease duration criterion more frequently results in regression to the mean of the mRSS score, which likely contributes to negative trial outcomes.
Review
Cardiac & Cardiovascular Systems
Antonio Totaro, Cosimo Sacra, Gianluca Testa, Antonio Pierro, Vincenzo Ienco, Eugenio Calvo, Rossella Calabrese, Gaetano Castellano, Ennio Lubrano, Fabio Perrotta, Silvia Scriffignano, Antonio Maria Calafiore
Summary: This is a case report of a rare complication of systemic sclerosis, calcific constrictive pericarditis, treated with pericardiectomy, resulting in significant clinical improvement.
CURRENT PROBLEMS IN CARDIOLOGY
(2023)
Article
Immunology
Patrick Coit, Kaila L. Schollaert, Emily M. Mirizio, Kathryn S. Torok, Amr H. Sawalha
Summary: The study assessed DNA methylation differences between juvenile systemic sclerosis (jSSc) and juvenile localized scleroderma (jLS), revealing distinct epigenetic patterns and unique methylated genes in both diseases. Compared to healthy controls, differential methylation sites and genes were identified in jSSc and jLS, indicating potential novel diagnostic biomarkers for localized scleroderma. Pathway analysis showed enrichment of inflammatory pathways in jSSc and the HIPPO signaling pathway in jLS, highlighting different biological processes involved in each condition.
CLINICAL IMMUNOLOGY
(2021)
Review
Rheumatology
Thanika Ketpueak, Wanitcha Chanlounse, Kittiya Na Nan, Chontara Pongsananurak, Nuntana Kasitanon, Worawit Louthrenoo
Summary: This study reported a patient who developed diffuse scleroderma and possible scleroderma renal crisis after paclitaxel therapy for ureter cancer. A literature review identified 27 patients with taxanes-induced scleroderma, and peripheral edema was the most common symptom. Skin lesions gradually progressed to skin fibrosis.
CLINICAL RHEUMATOLOGY
(2022)
Letter
Dermatology
M. Attard, D. O'Kane
Summary: Morphoea is a group of disorders characterized by inflammation and sclerosis of the skin and underlying tissues. While specific licensed treatments are currently lacking, a case study showed rapid response to abatacept.
CLINICAL AND EXPERIMENTAL DERMATOLOGY
(2022)
Article
Biochemistry & Molecular Biology
Maurizio Romagnuolo, Chiara Moltrasio, Angelo Valerio Marzano, Gianluca Nazzaro, Simona Muratori, Sebastiano Recalcati
Summary: This study demonstrates that a 90-day treatment with PDRN can significantly reduce disease activity and damage in localized scleroderma (morphea), for which there are limited therapeutic options.
Editorial Material
Dermatology
An-kang Gu, Jing Li, Li-Tao Zhang
Summary: The teenager likely has platysma contracture, a rare skin condition that presents with corrugated, elevated, and indurated short cords in the front of the neck surrounded by sclerotic skin.
Article
Rheumatology
Kristina Elizabeth Neergaard Clark, Shiwen Xu, Moustafa Attah, Voon H. Ong, Christopher Dominic Buckley, Christopher P. Denton
Summary: This study investigated cellular differences in diffuse cutaneous systemic sclerosis (dcSSc) patients compared with healthy controls (HCs), and found differences in cell clusters between the ARA+ and ATA+ subsets, highlighting the importance of considering antibodies and disease stage in the management and trial design of dcSSc.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Article
Medicine, General & Internal
Paulina Szczepanik-Kulak, Anna Michalak-Stoma, Dorota Krasowska
Summary: Morphea is a chronic autoimmune disease with characteristic clinical features of active and inactive skin lesions. This study demonstrates the valuable use of dermoscopy in assessing the activity or inactivity of lesions in morphea skin disease, aiding in therapeutic decisions and monitoring for relapse.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Rheumatology
Mugdha Sawant, Fang Wang, Janis Koester, Anja Niehoff, Michele M. Nava, Evy Lundgren-Akerlund, Donald Gullberg, Birgit Leitinger, Sara Wickstroem, Beate Eckes, Thomas Krieg
Summary: This study investigates the role of collagen-fibroblast interactions in regulating fibroblast functions and fibrosis. The results show that fibroblasts lacking collagen-binding integrins exhibit defects in cytoskeletal architecture, traction stress generation, and matrix production and organization. Mice lacking collagen-binding integrins also display an attenuated fibrotic response with impaired mechanotransduction and reduced collagen production. These findings suggest that targeting fibroblast-collagen interactions could be a promising therapeutic approach for fibrotic diseases.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Article
Multidisciplinary Sciences
Giuliana Cerro-Chiang, Matthew Ayres, Alejandro Rivas, Tahmineh Romero, Sarah J. Parker, Mitra Mastali, David Elashoff, Peter Chen, Jennifer E. Van Eyk, Paul J. Wolters, Francesco Boin, Tanzira Zaman
Summary: This study aimed to identify blood protein biomarkers associated with the progression of SSc-ILD. Serum proteins were profiled and analyzed for their association with disease progression. Results showed 29 proteins associated with progression, but these associations did not remain significant after adjusting for multiple testing. Unsupervised clustering analysis also did not reveal significant heterogeneity between the subjects.
SCIENTIFIC REPORTS
(2023)
Article
Medicine, Research & Experimental
Jeonghyeon Moon, Seon-yeong Lee, Jeong Won Choi, A. Ram Lee, Jin Hee Yoo, Su-Jin Moon, Sung-Hwan Park, Mi-La Cho
Summary: Scleroderma is an autoimmune disease characterized by dermal fibrosis, and metformin has been shown to have protective effects against scleroderma by reducing pro-inflammatory factors and inhibiting cytokines related to Th17 differentiation. Additionally, metformin modulates the inflammatory process in skin fibroblasts by decreasing mTOR-STAT3 signaling, suggesting its potential as a treatment for scleroderma by inhibiting pro-inflammatory cytokines and promoting anti-inflammatory activity.
JOURNAL OF TRANSLATIONAL MEDICINE
(2021)
Article
Rheumatology
Sophie Blaise, Carine Boulon, Marion Mangin, Patricia Senet, Isabelle Lazareth, Bernard Imbert, Francois-Xavier Lapebie, Philippe Lacroix, Joel Constans, Patrick Carpentier
Summary: This study evaluates the prevalence and clinical correlates of peripheral arterial disease of the upper limbs in patients with systemic sclerosis (SSc) using finger brachial pressure index (FBPI) measurements. The study finds a high prevalence of arterial disease in SSc patients and suggests that FBPI is a strong and independent predictor of digital ulcers. The study also indicates that both macro- and microangiopathy contribute to the ischemic damage of the fingertips.
ARTHRITIS CARE & RESEARCH
(2022)
