Scleroderma Medical News: April 2007

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April 2007

Nail changes in connective tissue diseases: do nail changes provide clues for the diagnosis? Proximal nailfold is the most important site of affection in CTDs. These nail changes can be used in combination with highly sensitive diagnostic modalities to establish an accurate diagnosis. PubMed. J Eur Acad Dermatol Venereol. 2007 Apr;21(4):497-503. (Also see: Raynaud's Diagnosis and Common Medical Tests )

Medical Signs and Symptoms Associated with Disability, Pain, and Psychosocial Adjustment in Systemic Sclerosis. Skin score is strongly associated with disability and pain. Dependent edema has negative implications across quality-of-life (QOL) outcomes. Assessment (including self-report of patient symptoms) of specific medical signs and symptoms may indicate SSc patients experiencing diminished QOL. J Rheumatol 2007 February;34:359–67. (Also see: Emotional Adjustment )

Clinical features and personality traits associated with psychological distress in systemic sclerosis patients. SSc patients experience elevated symptoms of psychological distress. Several clinical parameters are associated with distress, but the role of various personality traits could not be disregarded. Early psychiatric assessment and intervention could prevent psychological distress in SSc patients. PubMed. J Psychosom Res. 2007 Jan;62(1):47-56. (Also see: Emotional Adjustment )

Appearance self-esteem (ASE) in systemic sclerosis--subjective experience of skin deformity and its relationship with physician-assessed skin involvement, disease status and psychological variables. In SSc, ASE proved unrelated to the extent of skin thickness. Psychological interventions aimed at boosting ASE should primarily target the psychological factors acceptance and anxiety. PubMed. Rheumatology (Oxford). 2007 Feb 17. (Also see: Emotional Adjustment )

Systemic sclerosis in childhood: clinical and immunologic features of 153 patients in an international database. This study provides information on the largest collection of patients with juvenile SSc ever reported. Juvenile SSc appears to be less severe than in adults because children have less internal organ involvement, a less specific autoantibody profile, and a better long-term outcome. PubMed. Arthritis Rheum. 2006 Dec;54(12):3971-8. (Also see: Juvenile Scleroderma )

Pulmonary-renal syndrome (PRS) in systemic sclerosis: a report of three cases and review of the literature. Clinical courses of the patients with PRS with thrombotic microangiopathy suggest that high-dose corticosteroid therapy is a trigger of diffuse alveolar hemorrhage in patients with diffuse SSc with signs of thrombotic microangiopathy. PubMed. Mod Rheumatol. 2007;17(1):37-44. (Also see: Renal Involvement, Pulmonary Involvement, Medications, and Clinical Trials: Negative Results )

Changes in causes of death in systemic sclerosis, 1972- 2002. Survival of scleroderma has changed since the treatment of renal crisis became possible. The change in pattern of scleroderma- related mortality over the past 30 years implicates the lung (both pulmonary hypertension and pulmonary fibrosis) as the primary causes of scleroderma related deaths today. PubMed. Ann Rheum Dis. 2007 Feb 28. (Also see: SSc: Prognosis and Mortality, Pulmonary Involvement, and Renal Involvement )

Fabricating sectional-collapsible complete dentures for an edentulous patient with microstomia induced by scleroderma. Sectional-collapsible maxillary and sectional mandibular complete dentures were fabricated by means of sectional trays. With the use of palatal midline hinge and stud attachments, the sectional dentures were successfully and easily inserted and removed. PubMed. Quintessence Int. 2007 Jan;38(1):15-22. (Also see: Dental Involvement )

Myasthenia gravis and scleroderma: Two cases and a review of the literature. Myasthenia gravis is uncommon in patients with scleroderma, and when diagnosed is usually associated with previous use of d-penicillamine. Clinically, both myasthenia and scleroderma may present with fatigue, weakness and bulbar symptoms, so one of diagnoses may be delayed. PubMed. Clin Neurol Neurosurg. 2007 Feb 3. (Also see: Myasthenia Gravis, and Clinical Trials: Negative Results )

Evaluation of oral methotrexate in the treatment of systemic sclerosis. It was concluded that methotrexate for 6 months only provides subjective improvement, and further studies after 1 year of treatment with methotrexate are recommended. PubMed. Int J Dermatol. 2007 Feb;46(2):218-23. (Also see: Clinical Trials: Methotrexate and Medications )

Combination of intravenous pulses of cyclophosphamide and methylprednizolone in patients with systemic sclerosis and interstitial lung disease. The results suggest that the employed combination is safe and effective, mainly in stabilizing the respiratory function of the patients. This goal is more realistic when treatment is given before significant functional compromise has ensued. PubMed. Rheumatol Int. 2007 Feb;27(4):357-61. (Also see: Pulmonary Fibrosis, Cyclophosphamide for Scleroderma Lung Disease, and Medications )

Recombinant human anti-transforming growth factor beta1 antibody therapy in systemic sclerosis: a multicenter, randomized, placebo-controlled phase I/II trial of CAT-192. In this pilot study, CAT-192, in doses up to 10 mg/kg, showed no evidence of efficacy. The utility of clinical and biochemical outcome measures and the feasibility of multicenter trials of early dcSSc were confirmed. PubMed. Arthritis Rheum. 2007 Jan;56(1):323-33. (Also see: Clinical Trials )

Classification criteria of scleroderma. Those patients with systemic scleroderma involving the trunk are classified as "Cutaneous diffuse systemic scleroderma"; the association of Raynaud's phenomenon, capillaroscopic abnormalities and specific autoantibodies defines "limited systemic scleroderma"; among the latter patients, those with distal skin involvement are classified "Cutaneous limited systemic sclerosis". The old term "CREST" tends to be abandoned due to its lack of specificity. PubMed. Presse Med. 2006 Dec;35(12 Pt 2):1916-22. (Also see: Types of Scleroderma )

Cell therapy for autoimmune diseases. Autologous haemopoietic stem cell transplantation (HSCT) has become a curative option for conditions with very poor prognosis such as severe forms of scleroderma, multiple sclerosis, and lupus, in which targeted therapies have little or no effect. PubMed. Arthritis Res Ther. 2007 Mar 14;9(2):206. (Also see: Stem Cell Transplants )

Clinical risk assessment of organ manifestations in systemic sclerosis - a report from the EULAR Scleroderma Trials And Research (EUSTAR) group data base. Diffuse cutaneous (dcSSc) and a limited cutaneous (lcSSc) subsets are associated with particular organ manifestations, but in this analysis the clinical distinction appeared superseded by an antibody based classification in predicting some scleroderma complications. PubMed. Ann Rheum Dis. 2007 Feb 1. (Also see: Types of Scleroderma and Antibodies )

Update on pathophysiology of scleroderma with special reference to immunoinflammatory events. Scleroderma or systemic sclerosis (SSc) is a complex disease in which the vasculopathy and the activation of the immune system with production of inflammatory mediators lead to dysregulated fibroblast activation. The resulting excessive deposition of collagens and other extracellular matrix proteins ends in fibrosis and organ dysfunction. The cause is unknown, but environmental factors are thought to play a role by triggering abnormal responses in genetically susceptible hosts. PubMed. Ann Med. 2007;39(1):42-53. (Also see: What is Scleroderma and Causes of Scleroderma: Genetics, and Environmental )