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Clinical Applications of Immunoassays
Published in Richard O’Kennedy, Caroline Murphy, Immunoassays, 2017
Systemic sclerosis is a multisystem connective tissue disorder and is characterised by thickening and fibrosis of the skin (scleroderma). There are two disease patterns comprising diffuse cutaneous systemic sclerosis (dcSS) and limited cutaneous systemic sclerosis (lcSS) based on the extent of skin involvement. In dcSS, sclerotic skin is seen on the trunk and proximal limbs as well as face and distal limbs and is associated with a worse prognosis. In lcSS, scleroderma is restricted to the face, neck and limbs distal to the elbow and knee and generally has a better prognosis when compared to dcSS. Many patients with lcSS have the CREST syndrome, which is an acronym for calcinosis, Raynaud’s disease, esophageal dysmotility, sclerodactyly and telangiectasia.
Allogeneic Hematopoietic Stem Cell Transplantation for Autoimmune Diseases
Published in Richard K. Burt, Alberto M. Marmont, Stem Cell Therapy for Autoimmune Disease, 2019
Shimon Slavin, Alberto Marmont, Richard K. Burt
Scleroderma with diffuse skin involvement and/or visceral involvement has a 5 year mortality of 60% (12% mortality per year). This mortality is higher than chronic myelogenous leukemia (CML) or low grade lymphomas for which allogeneic HSCT is an accepted treatment. For a carefully designed regimen, the high mortality of scleroderma justifies the risk of allogeneic HSCT from an HLA matched sibling using unmanipulated bone marrow or peripheral blood stem cells. High risk patients eligible for this therapy may be defined as: An established diagnosis of scleroderma and two or more of the following high risk features: a) Diffuse cutaneous scleroderma with involvement proximal to the elbow or knee and a Rodnan score (refer to Chapter 46) of >14.85b) DLCO <80% of predicted or decrease in lung function (TLC, DLCO or FEV1) of 10% or more over 12 months.c) Active alveolitis on bronchoalveolar lavage.d) Pulmonary fibrosis or alveolitis on CT scan or CXR.e) Elevated ESR ≥25 mm/hour.f) Proteinuria (greater than trace on dipstick).g) Urine blood on dipstick or sediment.h) Abnormal EKG (non-specific ST-T wave abonormalities, low QRS voltage, or ventricular hypertrophy).
Genetic variants affecting chemical mediated skin immunotoxicity
Published in Journal of Toxicology and Environmental Health, Part B, 2022
Isisdoris Rodrigues de Souza, Patrícia Savio de Araujo-Souza, Daniela Morais Leme
Scleroderma is a fibrotic condition characterized by vascular injury and increased accumulation of extracellular matrix proteins in the skin (Yamamoto 2008). Although the underlying mechanisms behind development of scleroderma are yet not completely understood, evidence suggests that overproduction of extracellular matrix by fibroblasts results from complex interactions among endothelial cells, lymphocytes, macrophages and fibroblasts with involvement of several mediators including cytokines, chemokines, and growth factors (Yamamoto 2008). Scleroderma has been associated with human exposure to xenobiotics, such as silica, organic solvents, formaldehyde, epoxy resins and vinyl chloride (Bovenzi et al. 2001).