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March 2010

Incidence of childhood linear scleroderma and systemic sclerosis (SSc) in the UK and Ireland. We received notification of 185 potential cases, and 94 valid cases were confirmed: 87 (93%) with localized scleroderma and 7 (7%) with SSc. These data provide additional estimates of the incidence of this rare disorder and its subforms. Herrick AL. Arthritis Care Res (Hoboken). 2010 Feb;62(2):213-8. (Also see: Juvenile Scleroderma)

Age-related survival and clinical features in systemic sclerosis (SSc) patients older or younger than 65 at diagnosis. In elderly patients, the lSSc subset is more prevalent than the diffuse. Pulmonary and cardiac involvement are more prevalent in aged patients and appears sooner after the disease diagnosis. SSc is clearly related to increased mortality, although it is not significant in the elderly group. Carmen Pérez-Bocanegra. Rheumatology, March 11, 2010. (Also see: Prognosis and Mortality)

L-Arginine in pregnant scleroderma patients. Systemic sclerosis (SSc) pregnant women show a high frequency of premature births and occurrence of renal crisis. Some evidences showed the role of L-arginine in the prevention and treatment of preeclampsia. Dilia Giuggioli. (SpringerLink) Clinical Rheumatology. March 10, 2010. (Also see: Pregnancy and Scleroderma)

Innovative therapies for systemic sclerosis. Although there is still no treatment that is unequivocally effective for scleroderma, there have been some promising developments over the past number of years with identification of novel candidate targets and innovative strategies, including targeted immunomodulatory therapies, tyrosine kinase inhibitors and agents that may promote vascular repair. Voon H. Ong. Current Opinion in Rheumatology. 25 February 2010. (Also see: Scleroderma Treatments)

Successful treatment of steroid and cyclophosphamide-resistant diffuse scleroderma-associated interstitial lung disease with rituximab. We report herein a case of 47-year-old female with diffuse type of SSc with steroid and cyclophosphamide-resistant ILD that was successfully treated with RTX. Thus, we suggested that RTX could be an efficacious therapeutic modality for severe, conventional treatment-resistant SSc-associated ILD. (Springerlink) Wan-Hee Yoo. Rheumatology International. 01/07/10. (Also see: Medications)

Assessment of tissue fibrosis in skin biopsies from patients with systemic sclerosis (SSc) employing confocal laser scanning microscopy (CLSM): an objective outcome measure for clinical trials? CLSM followed by quantitative image analysis provides an objective and unbiased assessment of skin fibrosis in SSc and could be a useful end-point for clinical trials with disease-modifying agents to monitor the response or progression of the disease. Joanna Busquets. Rheumatology. March 4, 2010. (Also see: Skin Fibrosis)

Localized scleroderma and zinc: a pilot study. We conclude that high-dose zinc gluconate can therefore be a valuable alternative treatment for localized scleroderma, with good tolerability, although placebo-controlled studies are necessary to confirm our results. Brocard A. Eur J Dermatol. 2010 Feb 2. (PubMed). (Also see: Alternative Therapies and Localized Scleroderma)

Similarities and Differences Between Primary and Secondary Sjögren’s Syndrome (SS). A higher frequency of oral symptoms and parotid enlargement and stronger B cell activity (autoantibody production and lymphocyte infiltration) were observed in primary SS. Whether these results reflect a true difference between the 2 disease entities or derive from underlying variables remains uncertain. Gabriela Hernández-Molina. JRheum. March 1, 2010. (Also see: Sjögren’s Syndrome)

Thumb Involvement in Raynaud’s Phenomenon as an Indicator of Underlying Connective Tissue Disease. In a retrospective study to assess whether the degree of thumb involvement differs between primary Raynaud’s phenomenon (PRP) and secondary Raynaud’s phenomenon (SRP), it was found that although the median temperature gradient along the thumb was not significantly different between SRP and PRP, the thumb is more likely to be involved in SRP than in PRP. Thumb involvement is one of a number of clinical indicators that should alert the clinician to the possibility of an underlying connective tissue disease/disorder. Batsi Chikura. JRheum. March 1, 2010. (Also see: Raynaud’s Phenomenon)

Therapeutic angiogenesis in patients with systemic sclerosis by autologous transplantation of bone-marrow-derived cells. Autologous transplantation of bone-marrow-derived cells was shown to be a novel and useful approach to intractable ulcers in systemic sclerosis. Yoshiaki Ishigatsubo. Modern Rheumatology. February 18, 2010 (Also see: Digital Ulcer Treatments)

Lack of effect of 8 weeks atorvastatin on microvascular endothelial function in patients with systemic sclerosis. Atorvastatin 20 mg/day, given for 8 weeks, was not associated with changes in microvascular function or structure. The large variation in outcome scores means that it is not possible to rule out an effect on the basis of this trial. Future studies should be of longer duration and include patients with early disease who are unlikely to have irreversible structural vascular disease. Hala Y. Sadik. Rheumatology. February 16, 2010. (Also see: Statins)

Diagnosis and Classification of Systemic Sclerosis (SSc). Patients with limited SSc (lSSc) must have Raynaud's phenomenon and SSc specific nail fold capillary changes and/or SSc specific autoantibodies. Some lSSc patients who have no cutaneous involvement but common SSc nail fold capillaroscopy abnormalities, specific antinuclear antibodies, and visceral involvement are sometimes called SSc sine scleroderma. Eric Hachulla. Clinical Reviews in Allergy and Immunology. February 09, 2010. (Also see: Types of Scleroderma and Diagnosis)

Renal disease in systemic sclerosis with normal serum Creatinine. Patients with systemic sclerosis should be screened for renal involvement irrespective of disease severity or duration. Reem H. A. Mohamed Clinical Rheumatology. February 22, 2010. (Also see: Renal Involvement)

Diffuse systemic sclerosis and autoimmune hepatitis (AIH): a unique association. The authors describe herein the first report of a patient with diffuse systemic sclerosis who was diagnosed with AIH with positive antimitochondrial antibody and had an excellent response to immunosuppressive drugs. (SpringerLink) C. Ewerton Maia Rodriguez. Clinical Rheumatology. February 7 2010. (Also see: Hepatitis, Autoimmune)

Lymphatic and blood vessels in scleroderma skin, a morphometric analysis. Vascular involvement is frequent in systemic sclerosis, but the role of the lymphatic vasculature is poorly known. Our data show that skin lesions in systemic sclerosis are characterized by a selective rarefaction of lymphatic vasculature that spares periglandular vessels and that might have a pathogenic role in the evolution and in the clinical manifestations of the disease. Antonella Rossi, PhD Human Pathology. 11 December 2009. (Also see: Vascular Involvement)