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Localized scleroderma (LoS) is a chronic fibrosing disorder of the skin and, less commonly, subcutaneous tissues. As the disease causes subjective symptoms, cosmetic defects, and, at times, functional disability, subjects with LoS experience deterioration of their health-related quality of life (HRQoL). The influence of disease activity/severity and damage status on HRQoL measures in patients with LoS is scarcely known.

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Screening for pulmonary arterial hypertension in patients with scleroderma--a New Zealand perspective.

N Z Med J

August 2014

Department of Medicine (Rheumatology), North Shore Hospital, Private Bag 93503, Takapuna, Auckland 0740, New Zealand.

Background: Pulmonary arterial hypertension (PAH) in scleroderma (SSc) patients is a devastating complication with high mortality if untreated. Early recognition and specific treatment of PAH may improve outcome. Regular interval screening for PAH is generally recommended in scleroderma patients especially with the availability of emerging new therapies.

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Interferons and scleroderma-a new clue to understanding the pathogenesis of scleroderma?

Immunol Lett

June 2008

Laboratório de Microbiologia, Departamento de Parasitologia e Microbiologia, Centro de Ciências da Saúde, Universidade Federal do Piauí, Brazil.

Scleroderma or systemic sclerosis (SSc) is a complex disease characterized by vasculopathy and deregulated immune and fibroblast activation. The resulting excessive production of collagens and other extracellular matrix proteins by fibroblasts as well as the inflammatory response leads to the development of scleroderma. Recently, some emerging data have been showing a possible link between the type I and II interferons (IFNs) and SSc pathogenesis.

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Renal disease remains a major source of morbidity and mortality in patients with scleroderma (systemic sclerosis, SSc). We describe the clinical course of 3 patients with diffuse cutaneous SSc presenting with renal disease subsequently found to have antibodies to myeloperoxidase (anti-MPO) and crescentic glomerulonephritis. The presence of antineutrophil cytoplasmic antibodies (ANCA) and anti-MPO defines a subset of patients with SSc who are susceptible to crescentic glomerulonephritis.

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