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082 - Autoimmunity laboratory investigations in systemic sclerosis diagnosis and monitopring – The question of the clinician

Autor(s): E. Bartoloni Bocci, A. Giordano, O. Bistoni, R. Gerli

Issue: RIMeL - IJLaM, Vol. 3, N. 3-S1, 2007 (MAF Servizi srl ed.)

Page(s): 82-85

Systemic sclerosis (SSc) or scleroderma is a multisystem disease that affects the skin and internal organs. It is a very complex connective tissue disease from both a pathogenic and clinical point of view, since its spectrum spans simple Raynaud’s phenomenon, localized forms of skin fibrosis and the clinically most important forms of SSc sustained by inflammatory, vascular and fibrotic pathological events. A closer relationship between these disparate conditions is now appreciated, and skin sclerosis is no longer regarded as mandatory for the diagnosis of SSc. There have been substantial changes in disease classification and appreciation of its natural history associated with the recent introduction of novel investigations and treatments of organ-based complications. Although SSc still has a high disease-related mortality and effective diseasespecific systemic treatments are lacking, there have been major improvements, indeed, in the management of some organ-based complications, including renal and pulmonary involvement. Angiotensin-converting enzyme (ACE)-inhibitors for scleroderma renal crisis and advanced therapies for classes III and IV pulmonary arterial hypertension exemplify progress in the treatment of SSc. Laboratory investigations and, in particular, disease-specific autoantibody detection play a key role in the classification and in the prognostic assignment of scleroderma patient at disease onset with fundamental effects for a correct clinical and therapeutic approach of the disease.

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