Scleroderma Medical News: January 2007

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January 2007

Six-minute walk test for the evaluation of pulmonary disease severity in scleroderma patients. Hemoglobin desaturation during a 6 minute walk test (MWT) provides additional information regarding severity of disease in scleroderma patients with pulmonary manifestations. PubMed. Chest. 2007 Jan;131(1):217-22. (Also see: Pulmonary Hyptertension and Pulmonary Fibrosis )

Mechanisms of Disease: leukotrienes and lipoxins in scleroderma lung disease--insights and potential therapeutic implications. Pharmacologic correction of a leukotriene-lipoxin imbalance using leukotriene inhibitors or lipoxin analogs might be a new approach to the treatment of SLD. PubMed. Nat Clin Pract Rheumatol. 2007 Jan;3(1):43-51. (Also see: Pulmonary Fibrosis )

A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. This suggests that treatment of pulmonary fibrosis in SSc with low-dose prednisolone and IV CYC followed by AZA stabilizes lung function in a subset of patients with the disease. PubMed. Arthritis Rheum. 2006 Dec;54(12):3962-70. (Also see: Pulmonary Fibrosis )

Rice University breakthrough could prevent multiple fibrotic diseases. A protein, which is called serum amyloid P (or SAP), has proven effective at preventing fibrotic disease from developing in the hearts and lungs of lab animals, and researchers hope it will eventually save thousands of lives once it is developed for human use. EurekAlert! 01/18/07. (Also see: Pulmonary Fibrosis, and Skin Fibrosis )

Predicting 24 Month Pulmonary Function Test, Modified Rodnan Skin Score (mRSS) and Functional Results from Baseline Measurements in the Cyclophosphamide versus Placebo Trial of Systemic Sclerosis (SSc) Alveolitis. This study suggests that 12 months of cyclophosphamide results in continued benefit at 24 months. Daniel E. Furst. 1130/389. ACR 2006. (Also see: Pulmonary Fibrosis, Skin Fibrosis, and Clinical Trials: Cyclophosphamide and Scleroderma Lung Disease )

Coronary Atherosclerosis in Systemic Sclerosis. Coronary calcium, a surrogate marker for atherosclerosis, is found at significantly higher levels in systemic sclerosis (SSc) patients compared to age-, sex-, and race-matched controls. Levels of serum homocysteine are also significantly higher in SSc patients. Vandana Khurma. F9 ACR 2006. (Also see: Scleroderma Cardiac Involvement )

Gastrointestinal Symptoms, Perceived Functioning and Quality of Life Issues Correlate with Gastric Myoelectrical Activity in Systemic Sclerosis (SSc) Patients. GI symptoms, quality of life issues and perceived functioning significantly correlated with gastric myoelectrical activity, which has been shown to reflect gastric dysmotility in SSc patients. Terry A. McNearney. 1137/396 ACR 2006. (Also see: Gastrointestinal Involvement and Scleroderma Quality of Life )

Distinct Genetic Signatures In Systemic Sclerosis Subtypes: Insights From Gene Expression Profiling. Subtle variation in some of these genes which regulate critical pathological pathways in SSc could determine the disease subtype and hence could be potential targets for candidate gene association studies. Sudeep P. Pushpakom. 1835/507 ACR 2006. (Also see: Causes of Scleroderma: Genetics )

B Cell Reconstitution After Rituximab Recapitulates B Cell Ontogeny with a Preponderance of Transitional B cells and a Paucity of Memory B Cells. These results suggest fundamental differences in the B cell depletion and/or reconstitution process experienced by different groups of patients that impact clinical and immunologic outcomes. Jennifer H. Anolik. 1974 ACR 2006. (Also see: Medications and Causes of Scleroderma: B Cells and T Cells )

High Dose Cyclophosphamide (CYC) Without Stem Cell Transplantation in Systemic Sclerosis. High dose CYC without stem cell transplantation leads to rapid improvement of the modified Rodnan skin, HAQ, and PGA scores in the severe early diffuse SSc. Christopher V. Tehlirian. 689 ACR 2006. (Also see: Cytoxan, SCOT Clinical Trial, ASTIS Clinical Trial, and Skin Fibrosis )

Occupational Exposure to Solvents and Gender-Related Risk of Systemic Sclerosis (SSc): a Metaanalysis of Case-Control Studies. Whereas SSc affects women predominantly, among subjects with occupational exposure to solvents, men are at higher risk than women for the disease. J Rheumatol 2007 January;34:97–103. (Also see: Causes of Scleroderma: Solvents )

Disease Subsets, Antinuclear Antibody Profile, and Clinical Features in 127 French and 247 US Adult Patients with Systemic Sclerosis. There are disease classification and SSc-related serum autoantibody differences between French and American patients with SSc. These differences help to explain variations in clinical features reported from different geographic regions. J Rheumatol 2007 January;34:104–9. (Also see: Antibodies and Causes of Scleroderma: Cluster Studies )

Phototherapy: a promising treatment option for skin sclerosis in scleroderma? Phototherapy is able to stop or inhibit the fibrotic processes and to induce softening of sclerotic skin, especially in limited SSc. Rheumatology (Oxford). 2006 Oct;45 Suppl 3:iii52-iii54. (Also see: Skin Fibrosis and Clinical Trials: Phototherapy )

Neuroaxial anesthesia in a patient with progressive systemic sclerosis. Every aspect of anesthetic care may be altered or hindered by the pathogenesis of disease. Although the choice of regional or general anesthesia is unclear, to choose combined spinal epidural anesthesia may be useful. PubMed. BMC Anesthesiol. 2006 Oct 17;6(1):11. (Medications: Anesthesia )

New Treatment Hope For Systemic Sclerosis (SSc). On experimental fibroblast cultures from SSc patients, imatinib mesylate (a small growth factor inhibiting molecule) strongly reduced the synthesis of EMC proteins, the number of myofibroblasts, and the thickness of skin, almost back to levels observed in the healthy control groups. Science Daily. 12/29/06. (Also see: Treatments, Causes of Scleroderma: Cytokines, and Causes of Scleroderma: Molecular Defect )

Urticarial vasculitis appearing in the progression of systemic sclerosis (SSc). We demonstrate that, in the present case, mast cells might be involved in both courses of urticarial vasculitis and SSc as a common factor. PubMed. J Dermatol. 2006 Nov;33(11):792-7. (Also see: Vasculitis, Skin Fibrosis, Causes of Scleroderma: Molecular Defect, and Limited Scleroderma )

Risk factors for lung cancer in patients with scleroderma: A nested case-control study. Patients with scleroderma who smoke are 7 times more likely to develop lung cancer than non- smokers. PubMed. Ann Rheum Dis. 2006 Sep 19. (Also see: Scleroderma and Cancer and Pulmonary Involvement: Preventive Care )

Unique characteristics of systemic sclerosis sine scleroderma-associated interstitial lung disease. In the presentation of an idiopathic interstitial pneumonia, the presence of a nucleolar-staining ANA, telangiectasia, Raynaud phenomenon with abnormal capillaroscopy findings, gastroesophageal reflux, or pericardial disease suggests underlying systemic sclerosis.. PubMed. Chest. 2006 Oct;130(4):976-81. (Also see: Diffuse Without Skin Involvement, Pulmonary Fibrosis, and Pulmonary Involvement )

Long Term Follow Up of a Cohort of Patients With Positive Antinuclear Antibodies (ANA) and Raynaud’s Phenomenon (RP) in the Absence of Connective Tissue Disease at Initial Evaluation. Initial evaluation indicates that there is a subset of patients who remain with only positive ANA and RP and do not develop a defined CTD. Marie S. O'Brien. 1881/553 ACR 2006. (Also see: Antibodies and Raynaud's )

Cognitive Impairment in Systemic Sclerosis (SSc). Cognitive impairments are significantly more frequently encountered in SSc than in Rheumatoid Arthritis patients. The cause of such cognitive impairment remains to be investigated. Christine Moroni. 1873/545 ACR 2006. (Also see: Brain Involvement )

Outcomes at 24-months are Better for Patients Who Received One-year of Cyclophosphamide (CYC) Therapy for SSc Alveolitis Than in Those Who Received One-year of Placebo in the Scleroderma Lung Study. Differences between groups with respect to breathlessness, activities of daily living and general well-being were larger and favored CYC. Philip J. Clements. 1252 ACR 2006. (Also see: Pulmonary Fibrosis and Clinical Trials: Cyclophosphamide and Scleroderma Lung Disease )

High-Dose Immune Suppressive Therapy And Autologous Peripheral Blood Stem Cell Transplantation (PBSCT) For Systemic Sclerosis : Analysis of Immune Reconstitution. These observations suggest that high-dose immune suppressive therapy with auto-PBSCT improves the disease activity of patients with SSc. Hiroshi Tsukamoto. 690 ACR 2006. (Also see: Stem Cell Transplant and Causes of Scleroderma: Cytokines )

Disease Features, Disease Type, HLA Types, and Autoantibody Profile in 17 Multicase SSc Families. These findings suggest that the concordance for disease type and SSc specific autoantibodies is more common among SSc family members and the ACA positive limited SSc has a stronger genetic basis, and that the familial SSc does not represent a unique disease subset. Shervin Assassi. 1153/412 ACR 2006. (Also see: Causes of Scleroderma: Genetics, and Scleroderma Family Registry and DNA Repository )

Reduced Aerobic Capacity in Systemic Sclerosis (SSc) Patients Without Pulmonary Involvement. Abnormal vascular response to exercise may account for this finding since the vascular system is one of the major target organs in this pathologic condition. Natalia C. Oliveira. 1866/538 ACR 2006. (Also see: Causes of Scleroderma: Vascular )

Outcome of patients with scleroderma admitted to intensive care unit. A report of nine cases. The outcome of scleroderma patients admitted to the ICU was extremely poor. Infectious complication was the most common cause of death in our patients. PubMed. Clin Exp Rheumatol. 2006 Jul-Aug;24(4):380-6. (Also see: SSc: Prognosis and Mortality and Pulmonary Fibrosis )

Morbidity and mortality of patients diagnosed with systemic sclerosis after the age of 75: a nested case-control study. We conclude that a diagnosis of SSc at an older age appears to be a poor prognostic indicator related to both disease severity and comorbidities. A higher clinical suspicion will lead to an earlier diagnosis and a potential decrease in morbidity and mortality. PubMed. Clin Rheumatol. 2006 Nov;25(6):831-4. (Also see: Types of Scleroderma: Prognosis and Mortality )

Prognostic markers for systemic sclerosis. The prognosis of systemic sclerosis depends chiefly on the extent of the skin lesions, which correlates with the severity of the cardiovascular, pulmonary, and renal manifestations. PubMed. Joint Bone Spine. 2006 Oct;73(5):490-4. (Also see: Types of Scleroderma: Prognosis and Mortality )

Epidemiology of connective tissue disorders. The reported prevalence and incidence of connective tissue disorders are quite variable, depending on differences in study methodology. The prevalence of systemic sclerosis is lower, however, varying significantly between different studies and countries. PubMed. Rheumatology (Oxford). 2006 Oct;45 Suppl 3:iii3-iii4. (Also see: Causes of Scleroderma )