Prevalence and factors associated with left ventricular dysfunction in the EULAR Scleroderma Trial and Research group (EUSTAR) database of patients with systemic sclerosis.

OBJECTIVES: To measure the prevalence of, and factors associated with, left ventricular (LV) dysfunction in systemic sclerosis (SSc). METHODS: The EUSTAR database was first searched. A case-control study of a patient subset was then performed to further identify independent factors associated with LV dysfunction by simple and multiple regression. RESULTS: Of 7073 patients, 383 (5.4%) had an LV ejection fraction (EF) of <55%. By multiple regression analysis, age, sex, diffuse cutaneous disease, disease duration, digital ulcerations, renal and muscle involvement, disease activity score, pulmonary fibrosis and pulmonary arterial hypertension were associated with LV dysfunction. In the second phase, 129 patients with SSc with LVEF <55% were compared with 256 patients with SSc with normal LVEF. Male sex (OR 3.48; 95% CI 1.74 to 6.98), age (OR 1.03; 95% CI 1.01 to 1.06), digital ulcerations (OR 1.91; 95% CI 1.05 to 3.50), myositis (OR 2.88; 95% CI 1.15 to 7.19) and use of calcium channel blockers (OR 0.41; 95% CI 0.22 to 0.74) were independent factors associated with LV dysfunction. CONCLUSION: The prevalence of LV dysfunction in SSc is 5.4%. Age, male gender, digital ulcerations, myositis and lung involvement are independently associated with an increased prevalence of LV dysfunction. Conversely, the use of calcium channel blockers may be protective.

Systemic sclerosis at the cellular level: molecular pathways of pathogenesis and its implication on future drug design.

Endothelial cell abnormalities and the effects on the surrounding microvasculature is a focal point in the pathogenesis of Systemic Sclerosis disease and may even be the sentinel event for the initiation of this disorder. A better understanding of these processes may improve our understanding of the pathophysiology of Systemic Sclerosis and more specifically the vasculopathy observed. Such knowledge will help us to further current treatments options and design novel therapies for Systemic Sclerosis and other fibrotic disorders.

The spectrum of clinical manifestations, outcome and treatment of pericardial tamponade in patients with systemic lupus erythematosus: a retrospective study and literature review.

Pericardial effusions causing pericardial tamponade are rare in patients with systemic lupus erythematosus (SLE). The goal of this study is to describe in detail the clinical and laboratory characteristics of a group of patients with pericardial effusions and pericardial tamponade secondary to SLE. We retrospectively reviewed the records of 71 patients with SLE, admitted to our Hospital between 1985 and 2006 with a diagnosis of pericarditis, pericardial effusion and tamponade. Clinical features in the patients with tamponade were compared with those with pericardial effusions without tamponade. Pericardial effusion and SLE was confirmed in 41 patients. Pericardial tamponade occurred in nine of these patients (21.9%) at the time of presentation. All tamponade patients were women. Patients with pericardial effusions who developed tamponade had a statistically significant (P = 0.05) lower C4 level as compared with patients who did not develop tamponade. A pericardial window was required in five patients even though the patients were receiving high-dose corticosteroids. In the present series, all patients with tamponade were treated with high-dose corticosteroids though five of nine patients required a pericardial window in contrast to previous studies. A low C4 level at presentation was predictive of the development of tamponade physiology.

Premenopausal osteoporosis.

While osteoporosis is a major public health concern, guidelines for diagnosis and treatment of low bone mass in the premenopausal population is lacking. Dual-energy x-ray absorptometry (DEXA) is a poor diagnostic tool to evaluate bone density in this population and the World Health Organization's definition of osteoporosis based on DEXA is not applicable to women before menopause. Bisphosphonates, while commonly used to treat postmenopausal osteoporosis, are not recommended in most premenopausal patients due to their long half-lives and side-effect profiles, therefore limiting the pharmacological interventions available. Secondary causes of low bone mass in premenopausal women include malnutrition, gastroenterological and hepatic disorders, endocrine disorders, and pharmaceutical use as well lifestyle characteristics. It is important to identify these risk factors in young women in order to encourage a lifestyle and a diet that minimize bone loss and to determine when pharmacologic intervention is necessary. In cases of secondary osteoporosis, treatment of the underlying disease process or cessation of the inciting medication, if possible, often results in normalized bone mass. Newer drugs with more benign side effect profiles and new methods of evaluating bone mass are being investigated and are likely to improve the evaluation and management of premenopausal women with low bone mass.

A retrospective randomly selected cohort study of D-penicillamine treatment in rapidly progressive diffuse cutaneous systemic sclerosis of recent onset.

BACKGROUND: Several uncontrolled studies in systemic sclerosis have shown that D-penicillamine may cause improvement in skin sclerosis, decrease the rate of new visceral organ involvement, and improve overall survival. OBJECTIVES: To undertake a single-centre retrospective randomly selected cohort study to examine the effects of D-penicillamine treatment on skin and visceral organ involvement in patients with rapidly progressive systemic sclerosis of recent onset. METHODS: Eighty-four patients with diffuse cutaneous systemic sclerosis who had received D-penicillamine within 24 months of clinically detectable onset of skin sclerosis were randomly selected from the systemic sclerosis cohort followed at the Scleroderma Center of Thomas Jefferson University. Employing a previously described severity scale, disease severity and skin involvement were compared from initiation of D-penicillamine to end of study and a correlated matched t-test was used to establish statistical significance. RESULTS: At a mean+/-SD duration of D-penicillamine therapy of 29.2+/-5.5 months and at a median dose of 750 mg per day statistically significant improvement in skin (P<0.01) and cardiac, pulmonary and renal involvement (P<0.05) was observed. At last follow-up, 17 (20%) patients were still receiving D-penicillamine, 25 (30%) had discontinued it owing to disease improvement, and 18 (21%) had discontinued it owing to side-effects. CONCLUSIONS: In a population of patients with diffuse cutaneous systemic sclerosis, with progressive disease of recent onset, D-penicillamine treatment at a median dose of 750 mg per day caused a statistically significant reduction in skin involvement and improvement of renal, cardiac and pulmonary involvement.

Osteoporosis in females prior to menopause: current concepts regarding definition, screening and management.

Few practice guidelines exist to guide the clinician to the diagnosis and management of osteoporosis in the premenopausal woman. Many clinical concerns exist due to the paucity of clinical research studies, while at the same time widespread screening has resulted in an increase number of premenopausal women having their bone mineral density measured. The major issue that has arisen is not therapy, but what is the definition of osteoporosis in premenopausal women. We have no specific definition, for thus it is very difficult to make and specific recommendation on therapy based on bone mineral density numbers alone. Concerns over the definition, screening and management of osteoporosis in premenopausal women are addressed in this review based on the most recent available clinical data and consensus reports.

Inclusion body myositis in connective tissue disorders: case report and review of the literature.

We report a patient with systemic lupus erythematosus (SLE) and secondary Sjögren's syndrome (SS) who developed inclusion body myositis (IBM) which, contrary to the typical presentation of this disorder, was symmetrical in nature although the diagnosis was only made after electron microscopy was performed. Therapy with increased doses of methotrexate proved to be beneficial, with the patient having full recovery after 8 months of therapy. It appears that a subset of IBM may be related to autoimmune disorders, an issue that was disputed in the past, and these patients may have a better prognosis than typical IBM patients. This is the first case report of IBM in a patient who had the dual diagnosis of SLE and SS.