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Scleroderma
Published in Charles Theisler, Adjuvant Medical Care, 2023
Scleroderma is a chronic autoimmune disease with two main types. (1) Localized scleroderma has two subtypes and only affects the skin. It often appears as thick, hard waxy patches (morphea type) or streaks (linear type) on the skin. Symptoms tend to come and go with flare-ups and periods of relatively stability. It is not uncommon for the condition to go away or to stop progressing without treatment.1 Tightening of the skin can cause fingers to curl and lose their mobility. (2) Systemic scleroderma has three subtypes and can result in changes in the skin and muscles, circulation of blood, and extend to internal organs, especially the kidneys, lungs, heart, or digestive tract.
Connective Tissue Disorders
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Laura Atzori, Caterina Ferreli, Franco Rongioletti
Overview: The majority of patients are women. Young African American patients tend to have an earlier onset of disease and a more severe course. SSc can be divided into limited cutaneous systemic scleroderma (lcSSc), formerly known as the CREST syndrome (Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia), and a diffuse cutaneous systemic scleroderma (dcSSc). There are different autoantibody profiles, patterns of organ involvement, and prognosis. In systemic scleroderma, there is the involvement of the internal organs, such as the digestive tract, heart, lungs, and kidneys.
Animal Models Of Connective Tissue Diseases
Published in Marcos Rojkind, Connective Tissue in Health and Disease, 2017
Gerald Α. Hegreberg, Lynetta J. Freeman
The underlying cause of the fibrosis and vascular degeneration is not known. There are three popular theories regarding the primary cause of systemic scleroderma.176-178 One theory proposes that the vascular change is the primary cause, while another theory is that the collagen deposition is primary and precedes the vascular damage. Still another theory proposes that the connective tissue changes are caused by an immunologic mechanism.
“I'm still dad”: The Impact of Scleroderma on being a Father
Published in Occupational Therapy In Health Care, 2018
Janet L. Poole, Donna Haygood, Cindy Mendelson
Scleroderma is an autoimmune collagen disease that is manifested by hardening and fibrosis of the skin and internal organs. While the prevalence is 4.6 times greater in females than males (Meier et al., 2012), the disease is more severe in men resulting in a poorer prognosis and increased occurrence of lung and heart involvement (Elhai et al., 2016). The mean age of onset, between 30–50 years of age, is during the peak years when males are becoming or being fathers. Systemic scleroderma has two main subtypes: limited cutaneous scleroderma and diffuse cutaneous scleroderma. Limited systemic scleroderma (lSSc) has fibrotic skin involvement limited to the hands, fingers and face and later involvement of the internal organs (Connolly & Tuffanelli, 2004). In diffuse systemic scleroderma (dSSc), the more severe subtype, skin involvement occurs more proximally with early internal organ involvement.
The association between hand disease severity and fatigue in individuals with systemic sclerosis: a scoping review
Published in Disability and Rehabilitation, 2022
Donnamarie Krause, Janet L. Poole, Dinesh Khanna, Susan L. Murphy
Systemic scleroderma, including skin hardening, skin thickening, skin ulcers, and complications. We also included hand, contracture, function, and fatigue. These terms were expanded to include matching MeSH in PubMed, CINAHL, PsycINFO between January and June 2021. The International Classification of Functioning, Disability and Health (ICF) as a classification of health and health-related domains includes scleroderma as a systemic musculoskeletal and connective tissue disorder, which also involves the skin and subcutaneous tissue and was used to validate the search process [19].
Clinical characteristics and outcome in patients with antisynthetase syndrome associated interstitial lung disease: a retrospective cohort study
Published in European Clinical Respiratory Journal, 2019
Mads Lynge Jensen, Anders Løkke, Ole Hilberg, Charlotte Hyldgaard, Elisabeth Bendstrup, Dan Tran
Antibody distribution, demographics and clinical features are shown in Table 2. Coexistence of anti-SSA/Ro5 was found in all 11 patients tested, and positive ANA in five patients. One patient with anti-SSA/Ro5 had sicca symptoms. Three patients were diagnosed with an overlap syndrome of systemic scleroderma and AS. Creatinine kinase was elevated in seven patients (Table 1).