Understanding and managing cardiac involvement in systemic sclerosis.

INTRODUCTION: Cardiac involvement is common in systemic sclerosis occurring in up to 80% of patients. Primary myocardial dysfunction results from impairment of coronary microvascular circulation, myocardial inflammation and fibrosis with the prevalence of atherosclerosis remaining contradictory. AREAS COVERED: This review presents the various aspects of cardiac involvement in SSc from a pathophysiological, clinical, diagnostic and therapeutic standpoint. Imaging modalities with emerging role in the understanding of mechanisms and prompt diagnosis of myocardial fibrosis namely cardiac magnetic resonance are also discussed. EXPERT OPINION: Cardiac involvement in SSc - and particularly primary myocardial disease - remains a challenge as clinical symptoms manifest in advanced stages of heart failure and convey poor prognosis. Over the last years the introduction of sophisticated imaging methods of myocardial function has resulted in a better understanding of the underlying pathophysiological processes of myocardial damage such as microvasculopathy, inflammation, diffuse or focal fibrosis. Such developments could contribute to the identification of patients at higher risk for subclinical heart involvement for whom diligent surveillance and prompt initiation of therapy with cardioprotective and/or immunosuppressive drugs coupled with invasive interventions namely radiofrequency ablation, implantable cardioverter-defibrillator when indicated, may improve long-term outcomes.

Cancer risk in systemic sclerosis: identifying risk and managing high-risk patients.

Introduction: Systemic sclerosis (SSc) is associated with a heightened cancer risk compared to the general population. Several pathways including immune system upregulation, cumulative inflammation, environmental factors, and genetic predisposition contribute to the development of both cancer and autoimmunity. Areas covered: This paper provides an overview of studies investigating the relationship between SSc and various types of cancer with a special focus on the identification of patients at higher risk for malignancy development. The demographic, serological, clinical, and disease-related characteristics of SSc individuals who are diagnosed with cancer over the course of their disease are discussed to provide a practical guidance for relevant screening strategies. Expert opinion: Several studies have identified subgroups of SSc patients at higher cancer risk based on the immunological profile (anti-RNAPol III positivity), diffuse disease type, and older age at SSc onset. Additionally, a close temporal association between SSc and cancer onset in certain antibody subsets raises the question as to whether more aggressive screening strategies should be considered. Currently, there are no published studies investigating the cost-effectiveness, efficacy, and safety of a targeted cancer-detection program. Screening procedures should at least follow recommendations for the general population with a special focus on patients at higher risk and specific cancer types.

Automated 3D ιnterstitial lung disease εxtent quantification: performance evaluation and correlation to PFTs.

In this study, the performance of a recently proposed computer-aided diagnosis (CAD) scheme in detection and 3D quantification of reticular and ground glass pattern extent in chest computed tomography of interstitial lung disease (ILD) patients is evaluated. CAD scheme performance was evaluated on a dataset of 37 volumetric chest scans, considering five representative axial anatomical levels per scan. CAD scheme reliability analysis was performed by estimating agreement (intraclass correlation coefficient, ICC) of automatically derived ILD pattern extent to semi-quantitative disease extent assessment in terms of 29-point rating scale provided by two expert radiologists. Receiver operating characteristic (ROC) analysis was employed to assess CAD scheme accuracy in ILD pattern detection in terms of area under ROC curve (A z ). Correlation of reticular and ground glass volumetric pattern extent to pulmonary function tests (PFTs) was also investigated. CAD scheme reliability was substantial for ILD extent (ICC = 0.809) and distinct reticular pattern extent (0.806) and moderate for distinct ground glass pattern extent (0.543), performing within inter-observer agreement. CAD scheme demonstrated high accuracy in detecting total ILD (A z = 0.950 ± 0.018), while accuracy in detecting distinct reticular and ground glass patterns was 0.920 ± 0.023 and 0.883 ± 0.024, respectively. Moderate and statistically significant negative correlation was found between reticular volumetric pattern extent and diffusing capacity, forced expiratory volume in 1 s, forced vital capacity, and total lung capacity (R = -0.581, -0.513, -0.494, and -0.446, respectively), similar to correlations found between radiologists' semi-quantitative ratings with PFTs. CAD-based quantification of disease extent is in agreement with radiologists' semi-quantitative assessment and correlates to specific PFTs, suggesting a potential imaging biomarker for ILD staging and management.

Antiovarian antibodies in primary Sjogren's syndrome.

Our study aimed at screening patients with primary Sjogren's syndrome (pSs) for the presence of antiovarian antibodies (AOAs). Detection of AOAs in patients' sera was achieved by ELISA, using bovine ovarian extract for coating. Western blot analysis and immunohistochemistry were used to characterize the antibody targets in the extract and to determine their locus on the bovine ovary, respectively. Specific AOAs were detected in 27% of 37 patients (two with premature menopause) and in none of the controls. Immunoreactivity mainly resided in five proteins of the extract with molecular masses 42, 49, 55, 64 and 72 kDa, and it might be attributed to their carbohydrate components. The antibody targets were mainly located in the granulosa and theca interna cells of the follicle, and in the endothelial cells and fibroblasts of corpus luteum. The detection, for the first time, of AOAs in a significant percentage of patients with pSs may suggest autoimmune oophoritis, clinical or subclinical.

Arthritis accompanying ileal pouchitis or undifferentiated HLA B27-related spondyloarthropathy?

The case of an HLA B27(+) patient who developed inflammatory arthritis accompanying ileal pouchitis, following total proctocolectomy for familial adenomatous polyposis is presented for the first time in the literature. Based on its clinical presentation and serology, it can be classified either as arthritis accompanying ileal pouchitis, or as an undifferentiated spondyloarthropathy.