Scleroderma Medical News: June 2007

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June 2007

Ethnic Disparities Among Patients with Pulmonary Hypertension Associated with Systemic Sclerosis. In this cohort of patients, African Americans were more likely to have dcSSc. Among patients with PH, African Americans presented at a younger age than their Caucasian counterparts. Incidence of diastolic dysfunction was higher in the PH population. J Rheumatol 2007 June;34:1277-82. (Also see: Pulmonary Hypertension and Causes of Scleroderma: Race )

FDA Approves New Orphan Drug for Treatment of Pulmonary Arterial Hypertension (PAH). The U.S. Food and Drug Administration today approved Letairis (ambrisentan) for the treatment of PAH. Letairis significantly improved physical activity capacity compared with a placebo, as shown by a six-minute walk, a standard test. Letairis also delayed the worsening of the pulmonary hypertension. FDA News. 06/15/07. (Also see: Pulmonary Hypertension )

Plaque-type scleroderma associated with linear and oesophageal features and facial and extra-facial hemiatrophy. The combination of several sub-types of scleroderma and facial and extra-facial hemiatrophy in the same patient may indicate that these entities actually represent different spectra of the same disease. PubMed. Ann Dermatol Venereol. 2007 Jan;134(1):68-71. (Also see: Morphea and Linear Scleroderma)

Familial linear scleroderma (en coup de sabre) responsive to antimalarials and narrowband ultraviolet B therapy. A 32-year-old woman and her 35-year-old sister presented with plaques of scleroderma en coup de sabre. Treatment of the younger sister with antimalarials and narrow-band ultraviolet B (NB-UVB) phototherapy slowed disease progression and reversed hair loss. PubMed. Dermatol Online J. 2007 Jan 27;13(1):11. (Also see: Linear Scleroderma: En Coup de Sabre )

Capillaroscopy is a dynamic process in mixed connective tissue disease (MCTD). Nailfold capillaroscopy in MCTD is a dynamic process and analysis of each scleroderma-pattern parameter seems to be a good indicator of lung involvement and disease severity. Lupus, Vol. 16, No. 4, 254-258 (2007). (Also see: MCTD and Common Medical Tests )

Capillaroscopic patterns in rheumatic diseases. Microvascular damage is a typical feature of Systemic Sclerosis (SSc) and more than 95% of the patients present architectural disorganization, giant capillaries, haemorrhages, loss of capillaries, avascular areas and neovascularization, as main microvascular abnormalities. These sequential capillaroscopic changes characterize the "scleroderma pattern" and reflect the SSc microangiopathy. PubMed. Acta Reumatol Port. 2007 Jan-Mar;32(1):29-36. (Also see: What is Raynaud's? and Common Medical Tests )

Prevalence of renal involvement in Indian patients with systemic sclerosis. SRC (scleroderma renal crisis) is very rare in Indian patients with SSc. However, non-renal crisis abnormalities appear to be as common in Indian patients as compared to the western literature. PubMed. Indian J Med Sci. 2007 Feb;61(2):91-6. (Also see: Renal Involvement, and Causes of Scleroderma: Ethnicity, Race, and Geographical Regions )

Mortality and risk factors of scleroderma renal crisis: a French retrospective study in 50 patients. SRC remains associated with severe morbidity and mortality. CS (corticosteroid exposure) might increase the risk of developing SRC. Further studies are needed to confirm these results. Ann Rheum Dis. Published Online First: 8 June 2007. doi:10.1136/ard.2006.066985. (Also see: Renal Involvement )

What should be used as tools in women at risk of autoimmune disease? Because of the multifactorial nature of most autoimmune disorders, women seeking breast implants should be screened for the known triggers for developing CTD. Until we have better diagnostic tools, the women at risk of autoimmune diseases should not be considered for implantation of silicone devices for cosmetic purposes. PubMed. Acta Dermatovenerol Croat. 2007;15(1):45. (Also see: Causes of Scleroderma: Breast Implants )

Systemic sclerosis after interferon alphacon-1 therapy for hepatitis C. IFN has multiple effects on the immune system and is known to trigger the development of autoantibodies, as well as the onset or exacerbation of autoimmune disease. We suspect that the immunomodulatory effects of IFNalphacon-1 triggered the clinical manifestations of SSc in this patient. PubMed. Intern Med. 2007;46(8):473-6. (Also see: Liver Involvement: Hepatitis C and Causes of Scleroderma: Drugs and Medications )

Nifedipine effect on red cell rheological properties in patients with systemic scleroderma. In the present study we were able to demonstrate that erythrocyte deformability and two other related variables such as membrane fluidity and osmotic fragility improve significantly with nifedipine therapy. It is likely that nifedipine inhibiting cytoplasmic calcium accumulation could restore some red blood cell membrane properties. PubMed. Clin Hemorheol Microcirc. 2007;36(2):105-10. (Also see: Medications )

Infliximab therapy in pulmonary fibrosis associated with collagen vascular disease. This study suggests that inhibition of TNF-alpha with infliximab may stabilize the progression of pulmonary fibrosis associated with CVD. Prospective, controlled trials are necessary to determine the efficacy of infliximab in pulmonary fibrosis associated CVD. PubMed. Clin Exp Rheumatol. 2007 Jan-Feb;25(1):23-8. (Also see: Pulmonary Fibrosis and Medications )

Research may yield improved treatment for diseased lungs. The research team hopes to create improved diagnostic tools that enable them to track stiffening of large and small arteries and link these measurements with impaired ventricular function. In a limited series of studies with scleroderma patients, a researcher also will investigate whether excess collagen negatively affects heart health. University of Wisconsin-Madison. 05/24/07. (Also see: Pulmonary Hypertension )

Prevalence and Characteristics of Moderate to Severe Pulmonary Hypertension in Systemic Sclerosis with and without Interstitial Lung Disease. Prevalence of moderate to severe PH was similar in SSc patients with and those without ILD. In patients with ILD, a lower PaO2 was the unique independent indicator associated with PH. In some patients with severe ILD, PH was out of proportion to the degree of fibrosis. J Rheumatol 2007May;34:1005-11. (Also see: Pulmonary Hypertension, and Pulmonary Fibrosis )

Imatinib mesylate reduces production of extracellular matrix and prevents development of experimental dermal fibrosis. Considering its favorable pharmacokinetics and clinical experience with its use in other diseases, imatinib mesylate is a promising candidate for the treatment of fibrotic diseases such as SSc. PubMed. Arthritis Rheum. 2007 Jan;56(1):9-12. (Also see: Skin Fibrosis and Scleroderma Treatments )

Cutaneous gene expression by DNA microarray in murine sclerodermatous graft-versus-host disease, a model for human scleroderma. These constellations of immunologic changes provide a "fingerprint" for fibrosing autoimmune disease. They are useful to understand the pathogenesis of Scl GVHD, to identify markers for early diagnosis of disease, and to devise more effective strategies for intervention in early scleroderma and Scl GVHD. PubMed. J Invest Dermatol. 2007 Feb;127(2):281-92. (Also see: Skin Fibrosis, and Graft-versus-Host-Disease )