Is Helicobacter pylori infection a risk factor for disease severity in systemic sclerosis?

Helicobacter pylori (H. pylori) is suspected to be one of the factors triggering systemic sclerosis (SSc). Data on the possible role of H. pylori are lacking. The aim of this study was to assess the effect of H. pylori infection in SSc patients. Forty-two SSc patients without dyspeptic symptoms were recruited—26 were H. pylori-positive and 16 were H. pylori-negative on the basis of invasive test. We evaluated the disease severity using clinical and laboratory parameters according to the Medsger Severity Scale. The level of SSc activity was evaluated according to Valentini activity score. The prevalence of H. pylori infection in population of SSc patients is 62 %. Severity of skin, gastrointestinal, and joint/tendon involvement was different between H. pylori-positive and -negative SSc patients (p < 0.001 for skin involvement, p = 0.002 and p = 0.03 for gastrointestinal and joint/tendon involvement, respectively) as well as erythrocyte sedimentation rate (p = 0.002). Severity score according to Medsger was higher in the H. pylori-positive than in the H. pylori-negative SSc patients (p < 0.001). Our data suggest that H. pylori infection correlates with severity of skin, gastrointestinal, and joint/tendon involvement in SSc patients. H. pylori-positive SSc patients showed higher severity score compared to H. pylori-negative. Therefore, H. pylori infection may play a role in the pathogenesis of SSc and also can provide some prognostic information.     

Localized scleroderma and systemic sclerosis: Is there a connection?

Excess fibrosis of the skin is a clinical hallmark of both localized scleroderma and systemic sclerosis. Localized scleroderma is generally thought to be a skin-limited disease whereas systemic sclerosis can have a wide range of internal organ involvement. Recent data suggest that a subset of patients with juvenile localized scleroderma can go on to develop systemic involvement of their disease. This raises the question of what the connection is, if any, between localized scleroderma and systemic sclerosis.     

Is anti-topoisomerase I a serum marker of pulmonary involvement in systemic sclerosis?

STUDY OBJECTIVE: To determine the value of the level of anti-topoisomerase I (anti-topo I) to evaluate lung involvement defined by abnormal high-resolution computed tomography (HRCT) score and pulmonary function tests (PFTs) in systemic sclerosis (SS). PATIENTS: Forty-eight patients with SS, 20 with lung involvement and 28 with no lung involvement. DESIGN: PFT measurement, HRCT scoring of lung involvement, and anti-topo I assay by enzyme-linked immunosorbent assay. Normal anti-topo I level was defined as < 30. RESULTS: There was a significant association between cutaneous extent and anti-topo I level (6.5% of patients with limited cutaneous scleroderma had abnormal anti-topo I levels vs 70.6% of patients with diffuse cutaneous scleroderma, p = 0.0001). In patients with diffuse cutaneous scleroderma, pulmonary involvement was associated with a higher percentage of abnormal anti-topo I level: 91.7% vs 20% (p = 0.010). In patients with diffuse cutaneous scleroderma, a significant association was found between the class of anti-topoII level and total lung capacity (median, 69 in patients with abnormal anti-topo I level vs 87 in patients with normal anti-topo I level, p = 0.010), between the class of anti-topo I level and HRCT score (median, 12 in patients with abnormal anti-topo I level vs 5 in patients with normal anti-topo I level, p = 0.05). CONCLUSION: Anti-topo I can be considered as a marker of lung involvement in patients with diffuse cutaneous scleroderma.     

Anti-vinculin autoantibodies in systemic sclerosis: a step toward a novel biomarker?

Clinical Rheumatology -     

Monckeberg’s sclerosis in a patient with systemic sclerosis

Monckeberg’s sclerosis (MS) is one of the non-inflammatory vascular diseases characterized by calcification of the media of small and medium-sized muscular arteries, but is distinct from atherosclerosis. We present a case of MS that was incidentally detected by plain X-ray in a patient with systemic sclerosis. We took CT angiographs of the patient’s lower extremities for the differential diagnosis of vascular calcification, which was confirmed. To determine if systemic sclerosis is a risk factor for MS, we reviewed plain X-rays from 43 well-documented systemic sclerosis patients, but we did not detect any cases of MS. We therefore conclude that systemic sclerosis may not be a risk factor for MS.     

D-penicillamine in systemic sclerosis? Yes!

The use of D-penicillamine in systemic sclerosis (SSc) has been controversial. We have reviewed the major published studies on this drug in SSc with diffuse cutaneous (dc) involvement and summarized our own recent experience in dcSSc patients treated with and without D-penicillamine. We conclude that D-penicillamine favourably alters the natural history of skin involvement in dcSSc, even when used in low dose. Furthermore, recurrence of diffuse skin change after discontinuation of D-penicillamine and improvement in skin thickening after reinitiation of the drug support its effectiveness. We believe that the rheumatologic community should use D-penicillamine in patients with early dcSSc.     

Whole blood viscosity in systemic sclerosis: a potential biomarker of pulmonary hypertension?

Clinical Rheumatology - Our goal was to determine if whole blood viscosity (WBV) can be used to predict the risk of pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc)....