Faculty Bibliography

Abstract Behcet's disease is a rare autoimmune vasculitis that may cause coronary artery aneurysms. We discuss the evaluation and management decisions for a 19-year-old female with a giant rapidly expanding aneurysm of the proximal left anterior descending coronary artery and Behcet's disease. (J Card Surg 2011;26:268-270)

Juvenile Idiopathic Arthritis (JIA) consists of a collection of all forms of chronic arthritis in childhood with no apparent cause. JIA is the most common rheumatic disease in children and may result in significant pain, joint deformity, and growth impairment, with persistence of active arthritis into adulthood. The extra-articular features of JIA, such as anterior uveitis or macrophage activation syndrome, are often the greater focus of therapy. Prior to the mid 1990s, the therapeutic armamentarium for JIA was more limited, utilizing non-specific agents, many with significant adverse effects. In the current era of target-specific biologic therapy, it is possible to better tailor therapy for patients. Through continued translational research and clinical trials, the biology mediating disease is better understood, and there is the hope of safer, more effective medicine and potential cure. This review will outline the clinical features of JIA as well as provide the latest updates in current and future pharmacotherapy

OBJECTIVE: To describe the course of patients with juvenile dermatomyositis (JDM) treated effectively without systemic corticosteroids. STUDY DESIGN: A retrospective study of 38 patients with JDM treated at a tertiary care children's hospital identified 8 patients who had never received corticosteroids. Disease presentation and course, pharmacologic, and ancillary treatments were recorded. RESULTS: Patients in the no corticosteroid group were followed for a median of 2.8 years (range, 2.1 to 9.5 years). Treatment was primarily with intravenous immunoglobulin (IVIG) (75%) and methotrexate (50%), with favorable response in all. No serious treatment complications were observed; headaches were reported by 3 patients receiving IVIG. Two patients had a myositis flare after discontinuing all medications for more than 1 year; complete resolution of symptoms was observed after either 1 or 2 further doses of IVIG. Two patients had calcinosis (at 1 and 9 years of disease); however, no patient had joint contractures, muscle atrophy, lipodystrophy, or functional limitations. CONCLUSIONS: Systemic corticosteroids can be avoided in a select group of patients with JDM. Alternative agents such as methotrexate and IVIG may be prescribed to effectively treat JDM and prevent complications

OBJECTIVE: Male (NZW x BXSB)F(1) mice develop antiphospholipid syndrome (APS) and proliferative glomerulonephritis that is markedly accelerated by the Yaa locus encoding an extra copy of Tlr7. Female (NZW x BXSB)F(1) mice with only 1 active copy of Tlr7 develop late-onset glomerulonephritis but not APS. Because a major function of Toll-like receptor 7 is to induce type I interferons (IFNs), our goal was to determine whether IFNalpha can induce or accelerate the manifestations of systemic lupus erythematosus (SLE) in female (NZW x BXSB)F(1) mice. METHODS: Eight-week-old female (NZW x BXSB)F(1) mice were injected with a single dose of adenovirus expressing IFNalpha. Mice were monitored for the development of thrombocytopenia and proteinuria. Sera were tested for anticardiolipin and anti-Sm/RNP antibodies. Mice were killed at 17 or 22 weeks of age, and their kidneys and hearts were examined histologically and by immunohistochemistry. Spleen cells were phenotyped, and enzyme-linked immunospot assays for autoantibody-producing B cells were performed. RESULTS: IFNalpha markedly accelerated nephritis and death in female (NZW x BXSB)F(1) mice. A significant increase in spleen cell numbers associated with a striking increase in the number of activated B and T cells was observed. Marginal-zone B cells were retained. IFNalpha-induced increased titers of autoantibodies were observed, but thrombocytopenia was not observed. Cardiac damage was milder than that in male mice. CONCLUSION: IFNalpha accelerates the development of renal inflammatory disease in female (NZW x BXSB)F(1) mice but induces only mild APS and does not induce thrombocytopenia. The effect of IFNalpha on SLE disease manifestations is strain dependent. These findings are relevant to our understanding of the physiologic significance of the IFN signature

Juvenile idiopathic arthritis (JIA) consists of a group of heterogeneous disorders of chronic arthritis in childhoodwith no apparent cause. JIA is the most common rheumatic disease in children and may still result in signifcant morbidity, with joint deformity, growth impairment, and persistence of active arthritis into adulthood. In addition to arthritis, the extra-articular features that may be present in JIA, such as anterior uveitis or the fever of systemic-onset JIA, are often the greater focus of therapy. Prior to the mid 1990s, the therapeutic armamentarium for JIA was more limited, utilizing nonspecifc agents, many with signifcant adverse effects. In the new era of target-specifc biologic therapy, the clinician is now able to better tailor therapy for patients with JIA. Still, despite the bells and whistles of biologics, the consistent performance of methotrexate as the gold standard disease-modifying anti-rheumatic drug (DMARD), against which all other agents are compared, cannot be overemphasized. Through continued translational research, rheumatologists better understand the biology behind the clinical symptoms. This review will discuss the clinical features of JIA, as well as past, present, and future therapeutic approaches in the care of children with arthritis

OBJECTIVE: This study was undertaken to determine whether BAFF blockade can be used to prevent or treat antiphospholipid syndrome in a mouse model. METHODS: Eight- and 12-week-old (NZW x BXSB)F(1) mice were treated with BAFF-R-Ig or TACI-Ig alone or in addition to a short course of CTLA-4Ig. Mice were monitored for thrombocytopenia and proteinuria. Sera were tested for anticardiolipin antibodies (aCL), BAFF levels, and levels of soluble vascular cell adhesion molecule and E-selectin. Mice were killed at 17, 22, or 32 weeks of age, and kidneys and hearts were subjected to histologic examination. Spleen cells were phenotyped and enzyme-linked immunospot assays for autoantibody-producing B cells were performed. RESULTS: Both BAFF-R-Ig and TACI-Ig prevented disease onset and significantly prolonged survival. Treated mice had significantly smaller spleens than controls, with fewer B cells and fewer activated and memory T cells. BAFF blockade did not prevent the development of aCL, and there was only a modest delay in the development of thrombocytopenia. However, treated mice had significantly less nephritis and myocardial infarcts than did controls. CONCLUSION: Our findings suggest that aCL are generated in the germinal center, which is relatively independent of BAFF. Effector function of antiplatelet antibodies was only modestly affected by BAFF blockade. In contrast, myocardial infarctions were prevented, suggesting that triggering of thromboses requires both autoantibodies and mediators of inflammation. Similarly, renal damage requires both immune complexes and effector cells. The dissociation between autoantibody production and inflammation that may occur with B cell-depleting therapies underscores the role of B cells as effector cells in the autoimmune response

PURPOSE/OBJECTIVE:Atrial arrhythmias, especially supraventricular tachycardia (SVT) and atrial fibrillation, are common after thoracotomy and lung surgery. There are few existing data on the incidence of postoperative arrhythmias after video-assisted thoracoscopy (VAT). The purpose of the present investigation was to retrospectively determine the incidence of postoperative arrhythmias in patients who underwent VAT compared with those who underwent thoracotomy, and which factors are associated with an increased risk for arrhythmias in both groups. DESIGN/METHODS:A retrospective investigation. SETTING/METHODS:A metropolitan university hospital. PARTICIPANTS/METHODS:The medical records of 124 patients who underwent thoracotomy and 81 patients who underwent VAT over a 2-year period were reviewed. MEASUREMENTS AND MAIN RESULTS/RESULTS:There was a 17% incidence of atrial arrhythmias after thoracotomy and 10% after VAT, but the difference was not statistically significant. In both groups, atrial fibrillation was the most common atrial arrhythmia. CONCLUSION/CONCLUSIONS:Patients receiving digoxin were at higher risk for postoperative arrhythmias. Patients older than 65 years were at risk for arrhythmias after thoracotomy and patients older than 80 years were at risk for arrhythmias after VAT. Patients who had postoperative arrhythmias had prolonged hospital stays compared with patients who did not have arrhythmias.