Classification of systemic sclerosis

Valentini, Gabriele

doi:10.1016/s0738-081x(94)90324-7

Cited by 17 publications

(2 citation statements)

References 39 publications

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“…30,31 SSc is a collagen vascular disease characterized by fibrosis of the skin (scleroderma), Raynaud's phenomenon, widespread damage of small arteries and microvessels, musculoskeletal manifestations, and other forms of visceral involvement. 32,33 In one series, over 70% of SSc patients demonstrated lung involvement in the form of interstitial fibrosis on postmortem examination. 34 The other less common manifestation of SSc in the lung is pulmonary hypertension.…”

Section: Systemic Sclerosis-associated Pulmonary Fibrosismentioning

confidence: 99%

Genetics of Pulmonary Fibrosis

Wurfel¹,

Raghu²

2002

Seminars in Respiratory and Critical Care Medicine

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Pulmonary fibrosis is a devastating disease that occurs in association with environmental exposures, several inherited diseases, collagen vascular disease, as well as in an idiopathic form. Epithelial cell damage, excessive fibroblast proliferation, upregulation of extracellular matrix production, and attenuated matrix degradation are shared pathophysiological features of these different forms of pulmonary fibrosis. Several observations suggest that genetic predisposition plays an important role in the pathophysiology of pulmonary fibrosis. There is significant inter-individual variation in the degree of pulmonary fibrosis resulting from exposure to similar levels of an inhaled fibrogenic agent. There are several inherited diseases with known genetic mutations that have high rates of pulmonary fibrosis. Idiopathic pulmonary fibrosis can occur in multiple first-degree relatives, including those separated at an early age. Finally, recent studies have identified polymorphisms within genes for cytokines, histocompatability antigens, and extracellular matrix components that are associated with the development of pulmonary fibrosis. Identification of putative pulmonary fibrosis gene/s (PF gene/s) may result in novel diagnostic and therapeutic interventions for those at risk for development of pulmonary fibrosis.

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Section: Systemic Sclerosis-associated Pulmonary Fibrosismentioning

confidence: 99%

Genetics of Pulmonary Fibrosis

Wurfel¹,

Raghu²

2002

Seminars in Respiratory and Critical Care Medicine

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“…In addition, when compared to each other, they show great deal of variability in skin sclerosis, internal organ involvement, disease progression and prognosis. These aspects have prompted the American College of Rheumatology to develop classification criteria able to separate SSc from other CTD; in order to improve the comparability among series recruited from different centers [5] and some clinical investigators to differentiate somewhat mutually exclusive subsets characterized by different course and prognosis [Valentini,6].…”

Section: Introductionmentioning

confidence: 99%

Scleroderma Subsetting

Valentini¹,

Ferri²

2006

CRR

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Patients with Systemic Sclerosis [SSc] present great variability in the extent of skin sclerosis, internal organ involvement and prognosis. These aspects have long prompted clinical investigators to differentiate SSc into subsets. The most widely used subsetting schema was proposed by Carwile LeRoy et al. in 1988. The schema differentiates two main subgroups (i.e., limited cutaneous SSc -lcSSc-and diffuse cutaneous SSc -dcSSc-) characterized by different extent of skin sclerosis, autoantibody profile and pattern of internal organ involvement. This schema, however, is based on a Delphy technique (i.e. a consensus among experts). The other available schema, which differentiates three subsets according to skin sclerosis (i.e. lc-SSc characterized by sclerodactyly alone, intermediate cutaneous SSc-ic-SSc where the limbs are also involved, and dc-SSc with trunk skin sclerosis) has been demonstrated to have a greater prognostic power, but lacks any reference to other prognostically relevant items . A prospective multicenter study devoted to improve the three subsetting schema by adding such items could help the clinical investigator to better differentiate SSc subsets.

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Idiopathic connective tissue disorders

Luzar¹,

Calonje²

2012

McKee's Pathology of the Skin

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Classification of systemic sclerosis

Cited by 17 publications

References 39 publications

Genetics of Pulmonary Fibrosis

Genetics of Pulmonary Fibrosis

Scleroderma Subsetting

Idiopathic connective tissue disorders

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