Scleroderma Medical News: November 2007

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November 2007

Anticentromere Antibodies (ACA) Identify Patients with Sjögren's Syndrome (SS) and Autoimmune Overlap Syndrome. The presence of ACA among patients with SS allows identification of a subset of patients with "SS overlap syndrome," who show a wide diversity of autoimmunity, encompassing but not limited to limited cutaneous sclerosis (SSc). J Rheumatol 2007;34:2253-8 (Also see: Sjogren's Syndrome, Antibodies, and Scleroderma in Overlap )

Scleroderma in a Population-based Sample: Evidence Suggests Increasing Incidence. In this large population-based sample, there was evidence of increasing incidence for generalized scleroderma over the interval 1994 to 2003. Part of the observed trend in incidence may reflect an increasing recognition of the disease in the population by physicians. S. Bernatsky. 1193/425. ACR 2007. (Also see: About Scleroderma and Difficult Diagnosis )

Long-term Effects of Bosentan Treatment on Survival in Patients with Pulmonary Arterial Hypertension (PAH) Related to Connective Tissue Disease (CTD). The 2-year survival of patients treated with bosetan far exceeds the natural history of survival in untreated PAH-CTD, and supports a long-term benefit of bosentan treatment in patients with PAH-CTD. Janet E. Pope. 1199/431. ACR 2007. (Also see: Bosentan )

Risk Factors For Mortality In Patients With Systemic Sclerosis (SSc) And Interstitial lung disease (ILD). Mortality in patients with SSc and ILD is increased in those with an early and severe impairment of pulmonary function, concomitant cardiac involvement and elevated ESR (Sed rate), but not in Scl-70 positive patients. Beatriz E. Joven. 6/6. ACR 2007. (Also see: Mortality and Prognosis, and Pulmonary Fibrosis )

Intraspinal (ISC) and Paraspinal Calcinosis (PSC) Associated with Systemic Sclerosis. PSC and ISC are closely associated with vascular complications in SSc, and may cause neurological manifestations crucially. Takehiko Ogawa. 8/8. ACR 2007. (Also see: Calcinosis )

High dose cyclophosphamide without stem cell rescue in scleroderma. High dose cyclophosphamide without stem rescue can lead to clinically significant improvement in skin score and measures of disease severity in patients with diffuse cutaneous scleroderma. PubMed. Ann Rheum Dis. 2007 Nov 1. (Also see: Diffuse Scleroderma, Clinical Trials, and Medications )

Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. One year of CYC improved lung function, skin scores, dyspnea, and health status/disability, effects which either persisted or increased further for several months after stopping therapy. However, except for a sustained impact on dyspnea, all of these effects waned and were no longer apparent at 24 months. PubMed. Am J Respir Crit Care Med. 2007 Nov 15;176(10):1026-34. (Also see: Medications, Cytoxan and Pulmonary Involvement )

Ultraviolet A1 phototherapy for treatment of acrosclerosis in systemic sclerosis: controlled study with half-side comparison analysis. These results suggest that UVA1 phototherapy does not improve cutaneous thickness in acrosclerosis (sclerodactyly) even if few functional improvements, and some ulcerations healings can be occasionally observed. Photodermatology Photoimmunology & Photomedicine, Vol 23, No 6, December 2007, pp. 215-221(7). (Also see: Sclerodactyly )

Selective endothelin A receptor antagonism with sitaxsentan for pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD). Sitaxsentan appears to be efficacious in patients with PAH associated with CTD. Annals of the Rheumatic Diseases 2007;66:1467-1472. (Also see: Pulmonary Hypertension )

Morphoea: a manifestation of infection with Borrelia species? The frequent detection of this microorganism in morphoea points to a specific involvement of B. burgdorferi or other similar strains in the development of or as a trigger of this disease. PubMed. Br J Dermatol. 2007 Oct 17. (Also see: Morphea )

Pulmonary Hypertension (PH) and Thyroid Disease. Patients with PH have a higher prevalence of thyroid disease than other pulmonary patients. Evaluation of thyroid function in PH patients may be warranted to detect and assess coexisting thyroid disease. Chest, doi:10.1378/chest.07-0366. (Also see: Pulmonary Hypertension and Thyroid Disease )

Celiac disease (CD) associated with systemic sclerosis (SS). Diagnosis of CD in patients with SS may be difficult but is essential to achieve optimal treatment response in patients with poor quality of life. PubMed. Gastroenterol Hepatol. 2007 Nov;30(9):538-41. (Also see: Celiac disease )

Comparison of surgical approaches to recalcitrant gastroesophageal reflux disease in the patient with scleroderma. This finding suggests that laparoscopic Roux-en-Y gastric bypass (RYGBP) may be an option for the primary management of scleroderma-associated gastroesophageal reflux. PubMed. Ann Thorac Surg. 2007 Nov;84(5):1710-5. (Also see: GERD)

FDA Alert for CellCept (mycophenolate mofetil). Roche and FDA notified healthcare providers that use of CellCept (mycophenolate mofetil) is associated with increased risk of first trimester pregnancy loss and increased risk of congenital malformations. In addition, CellCept reduces blood levels of the hormones in an oral contraceptive pill and could theoretically reduce its effectiveness. FDA Medwatch. October 2007. (Also see: Medications and FDA Warnings )

Severe fibrosis and increased expression of fibrogenic cytokines in the gastric wall of systemic sclerosis patients. A pronounced deposition of collagen, the presence of myofibroblasts, and increased expression of several profibrotic factors are important hallmarks in the stomach of patients with SSc. Wiley InterScience. Arthritis and Rheumatism. Volume 56, Issue 10, Pages 3442 - 3447. (Also see: Causes of Scleroderma: Cytokines and GI Involvement )