Systemic sclerosisSclerodermaGuidelinesRecommendationsTreatmentManagementEvidence basedBest practicesPAHILDSystemic sclerosis (SSc) is a rare heterogeneous connective tissue disease. Recommendations addressing
Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease with multi-organ involvement, fibrosis and vasculopathy. Treatment in SSc, including early diffuse cutaneous SSc (dcSSc) and the use of organ-specific therapies, has improved, as evident from randomized clinical trials. Treatment...
Hein R, Behr J, Hündgen M, Hunzelmann N, Meurer M, Braun-Falco O, Urbanski A, Krieg T. Treatment of systemic sclerosis with gamma-interferon. Br J Dermatol. 1992 May; 126 (5):496–501.Hein R, Behr J, Hundgen M, et al. Treatment of systemic sclerosis with ␥ interferon. Br...
Recent European guidelines have introduced the concept of exercise pulmonary hypertension (ex-PH). However, the clinical characteristics of ex-PH in systemic sclerosis (SSc) remains unknown. We aimed to investigate the characteristics of exercise pulmonary hypertension (ex-PH) in patients with systemic...
A multicenter, randomized, double-blind, placebo-controlled trial of oral type I collagen treatment in patients with diffuse cutaneous systemic sclerosis: I. oral type I collagen does not improve skin in all patients, but may improve skin in late-phase disease Arthritis Rheum, 58 (2008), pp....
Balaban EP, Zashin SJ, Geppert TD, Lipsky PE (1991) Treatment of systemic sclerosis with antithymocyte globulin. Arthritis Rheum 34: 244–245Balaban, EP, Zashin, SJ, Geppert, TD, Lipsky, PE (1991) Treatment of systemic sclerosis with antithymocyte globulin. Arthritis Rheum 34: pp. 244-245...
Gogulska Z, Smolenska Z, Turyn J, Mika A, Zdrojewski Z (2021) Lipid alterations in systemic sclerosis. Front Mol Biosc 8:761721. https://doi.org/10.3389/fmolb.2021.761721 30. Kowal-Bielecka O, Fransen J, Avouac J et al (2017) Update of EULAR recommendations for the treatment of ...
Systemic sclerosis (scleroderma; SSc) is an orphan disease with the highest case-specific mortality of any connective-tissue disease. Excessive collagen deposit in affected tissues is a key for the disease's pathogenesis and comprises most of the clinical manifestations. Lidocaine seems to be an alt...
Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease with multi-organ involvement, fibrosis and vasculopathy. Treatment in SSc, including early diffuse cutaneous SSc (dcSSc) and the use of organ-specific therapies, has improved, as evi
1. 1.1. Juvenile-onset systemic sclerosis (jSSc) is defined as youth, children and young adults meeting the SSc ACR/EULAR 2013 diagnostic criteria with the onset of Raynaud’s or non-Raynaud’s manif...