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Friedman, Deborah; Rupel, Ann; Buyon, Jill
doi: 10.1007/s11926-007-0003-4pmid: 17502039
Neonatal lupus syndrome is a model of passively acquired autoimmunity in which the pregnant woman’s serum contains specific antibodies to 52 or 60 kd SSA/Ro and/or 48 kd SSB/La, which cross the placenta and are associated with the development of congenital heart block in the fetus and/or a transient rash or various liver and blood cell abnormalities in the newborn. To date, congenital heart block is a permanent condition that entails significant morbidity and mortality, with nearly all affected infants requiring pacemakers and with an 80% cumulative probability of survival at 3 years of age. An intensive search is on for the specific etiopathophysiology and for new clinical tools to approach and treat this disease.
doi: 10.1007/s11926-007-0004-3pmid: 17502040
Antiphospholipid syndrome is an important cause of neurologic morbidity. The clinical criteria for antiphospholipid syndrome include only cerebrovascular arterial and venous thrombosis, but many other neurologic manifestations have been associated with antiphospholipid antibodies (aPL). This review discusses the role of aPL in cerebrovascular manifestations and in some of the other neurologic manifestations commonly associated with these antibodies, as well as data pertaining to the pathophysiology of aPL-associated neurologic manifestations and treatment issues.
doi: 10.1007/s11926-007-0005-2pmid: 17502041
Patients with systemic lupus erythematosus (SLE) experience a wide array of neurologic (N) and psychiatric (P) events, some of which are directly attributable to lupus. Regardless of attribution, NP events have a significant impact on individual patient’s health-related quality of life. Primary immunopathogenic mechanisms of NP-SLE include vasculopathy, autoantibody production, and intrathecal inflammatory mediators. The recently described anti-NR2 glutamate receptor antibodies have been implicated in animal models of neuronal injury, but their role in the pathogenesis of human NP-SLE is unclear. The diagnosis of NP-SLE remains largely one of exclusion, although the detection of select autoantibodies, CSF analysis, and appropriate use of neuroimaging and neuropsychometric testing may provide support in the evaluation of individual patients. Therapeutic options include symptomatic therapies, immunosuppression, and anticoagulation.
Elliott, Jennifer; Manzi, Susan; Edmundowicz, Daniel
doi: 10.1007/s11926-007-0006-1pmid: 17502042
With improved therapeutic advances in the care of systemic lupus erythematosus patients, cardiovascular disease has emerged as a leading cause of death. Premature atherosclerosis in lupus patients is probably an interaction between traditional cardiovascular risk factors, inflammatory factors, and factors related to lupus itself. Despite knowledge of this accelerated cardiac risk, evaluation of traditional risk factors has been sub-par. We propose that lupus patients be evaluated by preventive cardiologists and have access to their expertise and resources. In addition to nephrologists and dermatologists, preventive cardiologists should be an integral part of the care of patients with lupus.
Abraham, David; Eckes, Beate; Rajkumar, Vineeth; Krieg, Thomas
doi: 10.1007/s11926-007-0008-zpmid: 17502044
The concept of mesenchymal fibroblasts has evolved over the past two decades from a relatively inert structural cell type to a dynamic, pluripotent cell lineage controlling normal connective tissue formation, homeostasis, and repair and as principle players in pathogenic scarring and fibrosis. In wound healing and tissue repair, fibroblasts provide proinflammatory signals and synthesize interstitial collagens, fibronectins, and other matrix components to repair the damaged tissue. Fibroblasts can differentiate into the myofibroblast, a specialized contractile cell type responsible for wound closure, tissue contraction, and scarring. This article reviews our current understanding of the origins of mesenchymal cells and their role in excessive scarring and fibrogenesis and in the systemic fibrotic disease scleroderma.
Fathi, Nihal; Furst, Daniel; Clements, Philip
doi: 10.1007/s11926-007-0009-ypmid: 17502045
Interstitial lung disease (ILD) is a leading cause of death in systemic sclerosis (SSc). Two randomized controlled trials recently demonstrated the modest effects of cyclophosphamide on lung physiology (forced vital capacity) and extrapulmonary outcomes (dyspnea, function, quality of life, and skin thickening). Recommendations can now be made about the short-term management for SSc-ILD. However, many questions remain unanswered, including how long to treat with cyclophosphamide; whether patients should take maintenance therapy after the initial or induction phase; whether there are alternative therapies; how to treat patients with ILD and pulmonary hypertension; and how to treat patients with severe ILD.
doi: 10.1007/s11926-007-0010-5pmid: 17502046
Substantial progress has been made in the past 10 years in the development and validation of outcome measures and refinement of trial methodology for systemic sclerosis (scleroderma, SSc). These advances in outcome measures have focused mostly on specific organ systems involved in SSc. Many of these new tools have been validated and subsequently adapted for use in multicenter clinical trials. Newer outcome measures in development are intended to more precisely quantify clinically meaningful change in specific organ systems, and some methods aim to assess overall disease burden in SSc. This review provides an update on currently used outcome measures in SSc with specific attention on newer tools and also describes methodology under development.
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