Current Rheumatology Reviews - Volume 9, Issue 2, 2013

The catastrophic variant of the antiphospholipid syndrome (APS) is the most severe and acute form of APS with multiorgan involvement. Patients with this condition have the following features in common: a) clinical evidence of multiple organ involvement developed over a very short time period; b) histopathological evidence of multiple small vessel occlusions, and c) laboratory confirmation of the presence of antiphospholipid antibodies, usually in high titer. The two key pathobiologic mechanisms that cause the clinical manifestations of catastrophic APS are the development of thrombosis and the systemic inflammatory response syndrome. A precipitating factor is reported in more than half of patients. A combination of anticoagulants, corticosteroids, intravenous immunoglobulins and/or plasma exchanges are the treatment modalities advocated for all patients with this severe condition. Unfortunately, its mortality is still high (around 30%) despite therapy. However relapse is uncommon.

Systemic lupus erythematosus (SLE) is the quintessential complex, multifactorial systemic autoimmune disease. In particular, it is highly polygenic in origin in both humans and animal models. Mouse models of lupus help uncover the genetic and cellular etiologies of the disease, which often implicate homeostatic imbalances in specific cell types and pathways. This review compiles and explores the contributions of specific genes in genetically manipulated mouse models that develop lupus spontaneously. A current version of this compilation can be found on SLE BASE at http://www.mohanlab.org/SLE_BASE/gene.

The increased mortality rate for cardiovascular (CV) diseases in rheumatoid arthritis RA) patients has been mainly attributed to an increased prevalence of some classical CV risk factors in these patients. Nonetheless, the potential role of chronic inflammation itself as an independent CV risk factor has been recently emphasized. Indeed, different basic alterations present in RA may anticipate and/or accelerate the atherosclerotic process in these patients. However, results of clinical studies are conflicting. We therefore considered data of the more recently published meta-analyses aiming to perform a critical reappraisal in such a topic. Unfortunately, there is a significant heterogeneity among the trials, and the role of classical CV risk factors was not taken into account in several studies. Therefore, whether RA is an independent risk for CV diseases still remains unclear.

Interstitial lung disease is a common manifestation in systemic sclerosis and is considered as one of the two main causes of death among these patients. Although the pathogenesis of interstitial lung disease related to systemic sclerosis (SSc-ILD) is very complex and not yet fully understood, diverse mechanisms such as vascular injury, altered immunological response and inflammatory activation have been proposed. Vascular injury is considered as the earliest event in the pathogenesis of this disease and has been associated with an excessive formation of alveolar capillaries, circulating endothelial cells, and increased expression of endothelin-1. Different cells like myofibroblasts, fibroblasts, endothelial cells and T lymphocytes are involved in the inflammatory activation. Meanwhile, lymphocyte activation, release of several cytokines and autoantibody production play an important role in the immunological response. To date, the treatment of SSc-ILD is not totally defined, as studies have shown mixed results. Given the high progression of the disease, it is difficult to enroll patients for clinical trials. Therefore, there is lack of evidence to guide therapeutic approaches. Throughout this paper, we present evidence supporting that the combination of glucocorticoids and cyclophosphamide is considered the best regimen for patients with SSc-ILD. In addition, we present data regarding the use of azathioprine, mycophenolate, anti-fibrosing agents, bosentan, rituximab, and imatinib mesylate as alternative therapies. Finally, for patients who are unresponsive to pharmacologic interventions, we present data regarding the efficacy of highdose immunosuppression with autologous transplantation of hematopoietic stem cells, and lung transplantation.

Thumb carpometacarpal (CMC) joint arthritis is a common and debilitating condition. The mainstay of treatment is conservative management. Surgery is only indicated following failure of a prolonged and comprehensive trial of nonoperative treatment. Once surgery has been designated, an array of surgical alternatives exist, all of which provide specific benefits and disadvantages. The different surgical options and their results are reviewed. The specific surgery will vary depending on the surgeon's experience and preference taking into account the patient's specific needs. The most commonly performed operative management at this time consists of trapeziectomy with ligament reconstruction, but most procedures can produce high patient satisfaction and in general, the results of surgical treatment are good.

Nontuberculous mycobacteria (NTM) are ubiquitous environmental organisms that can cause various diseases, including pulmonary disease (PD). In patients with rheumatoid arthritis (RA), numerous pulmonary manifestations, including bronchiectasis, may develop into Mycobacterium avium-complex (MAC)-PD, which can be lethal in patients who are being treated with a tumor necrosis factor-alpha blocker. However, the bronchiectasis associated with NTM-PD is difficult to distinguish from that associated with RA by radiological imaging alone. In addition, the diagnosis of NTM-PD is often hampered by the ease of NTM contamination. For the serological diagnosis of MAC-PD, the enzyme immunoassay (EIA) kit, Capilia MAC Antibody ELISA®, determines the levels of serum IgA antibody to the glycopeptidolipid core of MAC. Here we describe the efficacy of this EIA kit for diagnosing MAC-PD, and we review the characteristics of NTM and the association between RA patients and NTM infections.

Introduction: The cause of osteoarthritis is inflammation associated with production of proinflammatory cytokines, activation of matrix metalloproteinases, dysregulation of extracellular matrix, and weakening of mechanical cartilage integration. The joint damage is mediated by reactive oxygen species. Biomarkers influencing the course of the disease can be found in the peripheral blood. Aim of the study: The aim of the study was to confirm whether total hip replacement due to osteoarthritis changes the levels of biochemical parameters associated with degenerative changes. Material and methods: The study enrolled 34 patients qualified for total hip replacement. On the day of surgery, at day 10 after surgery and 6 weeks after surgery, blood was collected for analysis from the patients. Selected erythrocyte redox balance parameters were analyzed, i.e.: concentration of compounds reacting with thiobarbituric acid (TBARS), catalase activity, activity of superoxide dismutase and glutathione peroxidase and serum concentration of cytokines: IL6 and Il-1β/Il-1F2. Conclusion: Hip joint alloplasty leads to a reduction of inflammatory parameters and shifts the redox balance toward a reduced state.

Giant cell arteritis is the most frequently occurring primary vasculitis in European and North American adults over the age of 50 years. It usually presents with constitutional symptoms like fatigue, malaise and fevers with a temporal headache. We present a series of three interesting cases of the disease, discuss the differential diagnosis and review the literature on the same.