Systemic sclerosis is a chronic autoimmune disease of still not fully understood pathogenesis. Fibrosis, vascular wall damage, and disturbances of innate and acquired immune responses with autoantibody production are prominent features. Systemic sclerosis has specific subsets with different autoantibodies, and differences in the affected skin areas. The suspicion of systemic sclerosis and establishing the diagnosis will be facilitated by the criteria created by EULAR/ACR experts. The treatment of this autoimmune disease remains a challenge for clinicians and new therapeutic options are constantly sought. The occurrence of various symptoms and the involvement of many organs and systems make systemic sclerosis a multidisciplinary disease and require a holistic approach. The present article summarizes different clinical features of systemic sclerosis and the profile of autoantibodies and discusses recent rules and future perspectives in disease management.
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http://dx.doi.org/10.5114/reum.2019.87619 | DOI Listing |
Ann Rheum Dis
January 2025
Meinig School of Biomedical Engineering, Cornell University, Ithaca, New York, USA. Electronic address:
Objectives: This study aims to elucidate the microbial signatures associated with autoimmune diseases, particularly systemic lupus erythematosus (SLE) and inflammatory bowel disease (IBD), compared with colorectal cancer (CRC), to identify unique biomarkers and shared microbial mechanisms that could inform specific treatment protocols.
Methods: We analysed metagenomic datasets from patient cohorts with six autoimmune conditions-SLE, IBD, multiple sclerosis, myasthenia gravis, Graves' disease and ankylosing spondylitis-contrasting these with CRC metagenomes to delineate disease-specific microbial profiles. The study focused on identifying predictive biomarkers from species profiles and functional genes, integrating protein-protein interaction analyses to explore effector-like proteins and their targets in key signalling pathways.
Ann Rheum Dis
January 2025
School of Medicine, Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK; LTHT, NIHR Leeds Biomedical Research Centre, Leeds, UK. Electronic address:
Background: The EULAR recommendations for the treatment of systemic sclerosis (SSc) were updated in 2017, informed by a systematic literature review (SLR) completed in 2014.
Objectives: The aim of this new SLR was to provide the most up-to-date literature to underpin contemporary EULAR recommendations for the management of SSc.
Methods: 30 searches for 30 interventions (including several outcomes/clinical questions), and 1 dedicated search (with several interventions) for calcinosis were prioritised by the task force.
Ann Rheum Dis
January 2025
Department of Rheumatology, Université Paris Cité UFR de Médecine, Paris, France.
Objectives: To update the 2017 European Alliance of Associations for Rheumatology (EULAR) recommendations for treatment of systemic sclerosis (SSc), incorporating new evidence and therapies.
Methods: An international task force was convened in line with EULAR standard operating procedures. A nominal group technique exercise was performed in two rounds to define questions underpinning a subsequent systematic literature review.
Clin Cosmet Investig Dermatol
January 2025
Department of Dermatology, Guangzhou Dermatology Hospital, Guangzhou, Guangdong, People's Republic of China.
Scleromyxedema (SM) is a rare primary cutaneous mucinosis characterized by systemic papules and scleroderma-like manifestations, often associated with monoclonal gammopathy. We present the case of a 37-year-old male with SM who developed yellowish plaques on the neck and back over three years. Histopathological examination revealed mucin deposition, fibroblast proliferation, and fibrosis, supporting the diagnosis.
View Article and Find Full Text PDFMed J Armed Forces India
July 2024
Air Officer Commanding, 5 Air Force Hospital, Jorhat, India.
A 65-year-old male patient presented to eye outpatient department of a zonal hospital in North Eastern India with complaints of diminution of vision for 1-year duration. On ocular examination, his unaided visual acuity was 6/36 right eye and 6/12 left eye. He was diagnosed as a case of immature senile cataract with nuclear sclerosis grade 2+ in the right eye and immature senile cataract with nuclear sclerosis grade 1+ in the left eye, with no other ocular or systemic findings.
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