Sclerosis systemic

There is a large body of work on the genetics of systemic sclerosis (68). With the important caveat that autoantibody patterns appear to alter the chance of specific organ involvement, few studies have shown polymorphisms specific for pulmonary fibrosis in systemic sclerosis. Many studies have included too few 

A genetics of antitopoisomerase-1 antibodies (antibody most closely associated with lung fibrosis) 

Japanese patients 28 anti-topoisomerase positive, 34 anti-topoisomerase negative 

Association U.S. patients 850 white, (Taq man) 120 Hispanic, 130 African American Controls  

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Czirjak L, Schlammadinger J, Szegedi G. 1993. Systemic sclerosis and exposure to trichloroethylene. Dermatology 186 236. 

Shindo K, Machida M, Koide K, Fukumura M, Yamazaki R Deconjugation ability of bacteria isolated from the jejunal fluid of patients with progressive systemic sclerosis and its gastric pH. Hepatogastroenterology 1998 45 1643— 1650. 

Rees WDW, Leigh RJ, Christofides ND, Bloom SR, Turnberg LA Interdigestive motor activity in patients with systemic sclerosis. Gastroenterology 1982 83 5 7 5-580. 

Kaye SA, Lim SG, Taylor M, Patel S, Gillespie S, Black CM Small bowel bacterial overgrowth in systemic sclerosis Detection using direct and indirect methods and treatment outcome. BrJ Rheumatol 1995 34 265-269. 

It has been shown that lung macrophages from patients with systemic sclerosis (SS) produced the elevated levels of nitric oxide, superoxide, and peroxynitrite and expressed the enhanced level of iNOS [281], NAC administration reduced peroxynitrite production and might be possibly recommended for the treatment SS patients. Solans et al. [282] found the significant enhancement of lipid peroxidation in erythrocytes from SS patients. Cracowski et al. [283] showed that in vivo lipid peroxidation was enhanced in scleroderma spectrum disorders including SS and undifferentiated connective tissue disease. 

Bovenzi, M. et al., A case-control study of occupational exposures and systemic sclerosis, Int. Arch. Occup. Environ. Health. 77, 10, 2004. 

Englert H. et al., Male systemic sclerosis and occupational silica exposure-a population-based study, Aust. N. Z. J. Med., 30, 215, 2000. 

Diot, E. et al., Systemic sclerosis and occupational risk factors A case-control study, Occup. Environ. Med. 59, 545, 2002. 

Ntoso, K.A., Tomaszewski, J.E., Jimenez, S.A. and Neilson, E.G. (1986). Penicillamine-induced rapidly progressive glomerulonephritis in patients with progressive systemic sclerosis successful treatment of two patients and a review of the literature. Amer. J. Kidney Dis. 8 159-163. 

Czompoly T, Simon D, Czirjak L, Nemeth P. Anti-topoisomerase I autoantibodies in systemic sclerosis. Autoimmun Rev. 2009 Jul 8(8) 692-6. 

Autologous non-myeloablative hematopoietic stem cell transplantation in patients with systemic sclerosis. Bone Marrow Transplant 2007 40 549-555. 

Kalfin, R., Righi, A., Del Rosso, A., Bagchi, D., Gererini, D., Cerinic, M., and Das, D., Activin, a grape seed-derived proanthocyanidin extract, reduces plasma levels of oxidative stress and adhesion molecules (ICAM-1, VCAM-1 and E-selectin) in systemic sclerosis. Free Radical Res., 36, 819,2002. 

Glucocorticoids should be used with caution in progressive systemic sclerosis, and concomitant administration of anticoagulants to prevent ischemic colitis is recommended when administering glucocorticoids in high doses, especially by pulse therapy (SEDA-21, 415 150). 

Yamanishi Y, Yamana S, Ishioka S, Yamakido M. Development of ischemic colitis and scleroderma renal crisis following methylprednisolone pulse therapy for progressive systemic sclerosis. Intern Med 1996 35(7) 583-6. 

Mayes M, Clements PJ, Weiner SR, Porter J, Ellman M, Wise C, Kaufman LD, Williams J, Dole W. Intravenous iloprost infusion in patients with Raynaud phenomenon secondary to systemic sclerosis. A multicenter, placebo-controlled, double-blind study. Ann Intern Med 1994 120(3) 199-206. 

Iloprost is mainly used in patients with chronic critical leg ischemia due to atherosclerosis or Buerger s disease. Episodic digital ischemia in patients with systemic sclerosis or related disorders is another use. The most frequently observed adverse effects, facial flushing and headache, are caused by profound vasodilatation. 

The use of iloprost has been proposed in patients with systemic sclerosis, a disease that is often characterized by pulmonary hypertension and Raynaud s phenomenon. Three patients with systemic sclerosis who were treated with iloprost developed acute thrombotic events (3). In one case, intestinal infarction occurred 1 day after infusion of iloprost. In another patient the left kidney was not perfused 22 days after the last infusion of iloprost because of thrombosis of the left renal artery. The last patient, 9 months after the start of treatment with iloprost, and 5 days after the last infusion, had an anterolateral myocardial infarction. The authors commented that their observations did not allow them to conclude that there is a direct relation between infusion of iloprost and thrombotic events. However, they said that this possibility should be considered, and they suggested that risk factors for thromboembolism should be carefully evaluated in each patient with systemic sclerosis who is receiving iloprost. 

In a randomized, controlled study of cyclic iloprost or nifedipine in 46 patients with systemic sclerosis, the predictable adverse effects of iloprost (headache, nausea and vomiting, and diarrhea) were common but quickly resolved after the end of the infusion (5). They rarely required a temporary dose reduction. Hypotension occurred less often than with nifedipine. 

Four women with CREST syndrome or systemic sclerosis had pain and eventually contracture of the masseter muscles during infusion of iloprost for severe attacks of Raynaud s phenomenon (9). The adverse effect was quickly reversed by reducing the infusion rate. There were no electrocardiographic or cardiac enzyme changes. The mechanism of this effect is obscure. 

An oral formulation has been investigated in patients with Raynaud s phenomenon secondary to systemic sclerosis and in patients with severe ischemia due to Buerger s disease or to atherosclerosis. The first reports were not particularly encouraging in terms of efficacy. Tolerance is acceptable 6% of patients discontinued iloprost compared with 2% with placebo (10,11). 

Tedeschi A, Meroni PL, Del Papa N, Salmaso C, Boschetti C, Miadonna A. Thrombotic events in patients with systemic sclerosis treated with iloprost. Arthritis Rheum 1998 41(3) 559-60. 

Black CM, Halkier-Sorensen L, Belch JJ, Ullman S, Madhok R, Smit AJ, Banga JD, Watson HR. Oral iloprost in Raynaud s phenomenon secondary to systemic sclerosis a multicentre, placebo-controlled, dose-comparison study. Br J Rheumatol 1998 37(9) 952-60. 

Pope J, Fenlon D, Thompson A, Shea B, Furst D, Wells G, Silman A. Cyclofenil for Raynaud s phenomenon in progressive systemic sclerosis. Cochrane Database Syst Rev 2000 (2) CD000955. 

Herranz L, Rovira A, Grande C, Suarez A, Martinez-Ara J, Pallardo LF, Gomez-Pan A. Autoimmune insulin syndrome in a patient with progressive systemic sclerosis receiving penicillamine. Horm Res 1992 37(l-2) 78-80. 

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. 

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