The term undifferentiated connective tissue diseases is used to define conditions characterized by the presence of signs and symptoms suggestive of a systemic autoimmune disease that do not satisfy the classificative criteria for defined connective tissue diseases (CTD) such as systemic lupus erythematosus (SLE), Sjögren's syndrome (SS), rheumatoid arthritis (RA) and others. A small percentage of patients presenting with an undifferentiated profile will develop during the first year follow up of a full blown CTD, however an average of 75% will maintain an undifferentiated clinical course. These patients may be defined as having a stable undifferentiated connective tissue diseases (UCTD). The most characteristic symptoms of UCTD are represented by arthritis and arthralgias, Raynaud's phenomenon, leukopenia, while neurological and kidney involvement are virtually absent. Eighty percent of these patients have a single autoantibody specificity, more frequently anti-Ro and anti-RNP antibodies. Stable UCTD are considered as distinct clinical entities and therefore it has been proposed to define those conditions as UCTD. Classificative criteria have also been proposed and a work to better define them is still under way.
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http://dx.doi.org/10.1016/j.autrev.2006.03.004 | DOI Listing |
J Neurol Sci
December 2024
Neurological Department, Sheba Medical Center, Tel Hashomer, Ramat Gan 52621, Israel; Center for Neurological Restoration, Neurological Institute, Cleveland Clinic Foundation, Cleveland, USA.
Background: Patients with connective tissue diseases (CTD) can have a wide range of neurological manifestations. Neurological complaints may be the presenting symptom of CTD. Therefore, screening for CTD using anti-nuclear antibodies (ANA) is a common practice.
View Article and Find Full Text PDFStem Cell Res
December 2024
Department of Cell and Developmental Biology, University College London, London WC1E 6DE, UK; Stem Cells and Neuromuscular Regeneration Laboratory, The Francis Crick Institute, 1 Midland Road, London NW1 1AT, UK; Dubowitz Neuromuscular Centre, UCL Great Ormond Street Institute of Child Health and Great Ormond Street Hospital for Children, London, UK. Electronic address:
Ullrich congenital muscular dystrophy (UCMD) represents the most severe subtype of collagen VI-related dystrophies (COL6-RDs), a spectrum of rare extracellular matrix disorders affecting skeletal muscle and connective tissue. Here, we generated an induced pluripotent stem cell (iPSC) line (CRICKi021-A) from a UCMD patient with de novo dominant-negative mutation in COL6A1 gene by reprogramming dermal fibroblasts using a non-integrating mRNA-based protocol. The resulting human iPSCs displayed normal morphology, expressed pluripotency-associated markers and differentiated into the three germ layers.
View Article and Find Full Text PDFJ Cosmet Dermatol
January 2025
Clinical Research Center of the Carolinas, Charleston, South Carolina, USA.
Background: Exosomes are nanoscale vesicles derived from various cell types and tissues that have many potential applications, generating great interest from researchers. One particularly intriguing application of exosomes is their use as a direct therapeutic for aesthetic indications. Several studies and case reports have explored the impact of exosomes for numerous cosmetic concerns but a consensus on the outcomes of these studies has not been established.
View Article and Find Full Text PDFJ Biomed Mater Res A
January 2025
Faculty of Materials Science and Engineering, Warsaw University of Technology, Warsaw, Poland.
Bone tissue regeneration can be affected by various architectonical features of 3D porous scaffold, for example, pore size and shape, strut size, curvature, or porosity. However, the design of additively manufactured structures studied so far was based on uniform geometrical figures and unit cell structures, which often do not resemble the natural architecture of cancellous bone. Therefore, the aim of this study was to investigate the effect of architectonical features of additively manufactured (aka 3D printed) titanium scaffolds designed based on microtomographic scans of fragments of human femurs of individuals of different ages on in vitro response of human bone-derived mesenchymal stem cells (hMSC).
View Article and Find Full Text PDFFront Genet
December 2024
Department of Pediatrics, West China Second University Hospital, Chengdu, Sichuan, China.
Background: Autosomal recessive cutis laxa type 1B (ARCL1B) is an extremely rare disease characterized by severe systemic connective tissue abnormalities, including cutis laxa, aneurysm and fragility of blood vessels, birth fractures and emphysema. The severity of this disease ranges from perinatal death to manifestations compatible with survival. To date, no cases have been reported in the Chinese population.
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