Systemic sclerosis: A world wide global analysis

Abstract

The objective of this study was to analyze epidemiological tendencies of systemic sclerosis (SSc) around the world in order to identify possible local variations in the presentation and occurrence of the disease. A systematic review of the literature was performed through electronic databases using the keywords "Systemic Sclerosis" and "Clinical Characteristics." Out of a total of 167 articles, 41 were included in the analysis. Significant differences in the mean age at the time of diagnosis, subsets of SSc, clinical characteristics, and presence of antibodies were found between different regions of the word. Because variations in both additive and nonadditive genetic factors and the environmental variance are specific to the investigated population, ethnicity and geography are important characteristics to be considered in the study of SSc and other autoimmune diseases.

Evaluation of dry eye signs and symptoms in patients with systemic sclerosis

“…SSc has a worldwide distribution, occurring most commonly in females. The peak incidence appears in individuals between 45 and 64 years old [2].…”

“…Systemic sclerosis (SSc) is a rare chronic disease of unknown cause characterized by fibrosis, degenerative changes, and vascular abnormalities in the skin, joints, and internal organs [1,2]. Impairment of internal organs induces a wide spectrum The funding organization had no role in the design or conduct of this research.…”

Autoanticorpos em esclerose sistêmica e sua correlação com as manifestações clínicas da doença em pacientes do Centro-Oeste do Brasil

“…2 A marca registrada da ES é a microvasculopatia, ativação de fibroblastos e excessiva produção de colágeno. 3 Trata-se de enfermidade única ao exibir características de três processos fisiopatológicos distintos: consiste na tríade de injúria vascular, autoimunidade (celular e humoral) e fibrose tecidual e leva ao acometimento cutâneo, além de diversos órgãos internos, como pulmão, coração e trato gastrintestinal, entre outros. 3,4 Acredita-se que o elo entre o envolvimento vascular inicial e a consequência final da doença (fibrose tecidual) poderia ser representado pela autoimunidade.…”

“…3 Trata-se de enfermidade única ao exibir características de três processos fisiopatológicos distintos: consiste na tríade de injúria vascular, autoimunidade (celular e humoral) e fibrose tecidual e leva ao acometimento cutâneo, além de diversos órgãos internos, como pulmão, coração e trato gastrintestinal, entre outros. 3,4 Acredita-se que o elo entre o envolvimento vascular inicial e a consequência final da doença (fibrose tecidual) poderia ser representado pela autoimunidade. Anticorpos circulantes, alteração de mediadores imunológicos e infiltração de células mononucleares em órgãos afetados representam um argumento positivo na hipótese em que a disfunção do sistema imune conduz à enfermidade.…”

Association of C‐reactive protein with high disease activity in systemic sclerosis: Results from the Canadian Scleroderma Research Group

“…Systemic sclerosis (SSc; scleroderma) is an autoimmune connective tissue disease that affects more women than men, often beginning between ages 40 and 55 years (1). There are 2 subtypes of SSc, i.e., diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc), according to the extent of cutaneous involvement (2); dcSSc has more extensive skin involvement, including the trunk or proximal extremities, and is associated with more internal organ involvement such as significant interstitial lung disease (ILD), cardiomyopathy, and scleroderma renal crisis (SRC) and with increased mortality (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18).…”