Systemic sclerosis clinical features

Volpe A, Biasi D, Caramaschi P, Mantovani W, Bambara LM, Canestrini S, Ferrari M, Poli G, Degan M, Carletto A, Pieropan S, Minuz P. 2006. Levels of F2-isoprostanes in systemic sclerosis Correlation with clinical features. Rheumatology (Oxford) 45 314-320. 

A fairly strong and consistent association between exposure, primarily in occupational settings, to solvents (e.g. trichloroethylene) and scleroderma has been reported in numerous epidemiological studies (see chapter 8). Scleroderma-like diseases can also be induced by other chemical compounds, such as drugs (D Cruz, 2000) and silica ( Erasmus syndrome ). Workers exposed to vinyl chloride monomers exhibit clinical features that resemble systemic sclerosis, such as fibrotic skin lesions, pulmonary fibrosis, and skin capillary abnormalities. However, vinyl chloride disease also harbours several features that are clearly distinct from systemic sclerosis. After exposure is discontinued, skin lesions, capillary abnormalities, and acroosteolytic lesions revert to nearly normal (Haustein Ziegler, 1985). 

Table 3. Clinical features and laboratory findings of systemic sclerosis patients associated with silicosis n = 37), limited dif-fuse form = 22 15...

Regulation of homocysteine metabolism appears to be especially important in the central nervous system, presumably because of the critical role of methyl transfer reactions in the production of neurotransmitters and other methylated products. It has been known for decades that mental retardation is a feature of the genetic diseases, such as CBS deficiency, that cause severe hyperhomocysteinemia and ho-mocystinuria. Impaired cognitive function is also seen in pernicious anemia, which causes hyperhomocysteinemia due to deficiency of cobalamin (see Chapter 28). Hyperhomocysteinemia also may be linked to depression, schizophrenia, multiple sclerosis, and Alzheimer s disease. The molecular mechanisms underlying these clinical associations have not yet been delineated. 

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