Systemic lupus erythematosus presenting as Stevens-Johnson syndrome/toxic epidermal necrolysis.

Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN)-like lesions in acute cutaneous lupus erythematosus [LE]) are an unusual manifestation of systemic LE. We describe a patient with widespread vesiculobullous lesions diagnosed as SJS/TEN-like acute cutaneous LE as the initial presentation of systemic LE. Stevens-Johnson syndrome/TEN-like LE may be differentiated from other vesiculobullous lesions by factors including a history of recent LE exacerbation, photodistribution of lesions, lack of a precipitating infection or medication exposure, minimal mucosal involvement, a prolonged course, response steroid treatment, and histologic and immunofluorescence findings. It is paramount to identify SJS/TEN-like LE as this condition requires early and aggressive intervention. The optimal treatment approach for SJS/TEN-like LE is unclear, and although some case reports have shown glucocorticoids to be useful, there are also reports of cases in which additional measures, such as intravenous immunoglobulin and plasmapheresis, were required to achieve a response. Our patient's condition was refractory to high-dose corticosteroids and intravenous immunoglobulin but was successfully treated using plasma exchange. As such, this treatment may hold potential for improving the care of other patients with refractory SJS/TEN-like LE.

Isocitrate ameliorates anemia by suppressing the erythroid iron restriction response.

The unique sensitivity of early red cell progenitors to iron deprivation, known as the erythroid iron restriction response, serves as a basis for human anemias globally. This response impairs erythropoietin-driven erythropoiesis and underlies erythropoietic repression in iron deficiency anemia. Mechanistically, the erythroid iron restriction response results from inactivation of aconitase enzymes and can be suppressed by providing the aconitase product isocitrate. Recent studies have implicated the erythroid iron restriction response in anemia of chronic disease and inflammation (ACDI), offering new therapeutic avenues for a major clinical problem; however, inflammatory signals may also directly repress erythropoiesis in ACDI. Here, we show that suppression of the erythroid iron restriction response by isocitrate administration corrected anemia and erythropoietic defects in rats with ACDI. In vitro studies demonstrated that erythroid repression by inflammatory signaling is potently modulated by the erythroid iron restriction response in a kinase-dependent pathway involving induction of the erythroid-inhibitory transcription factor PU.1. These results reveal the integration of iron and inflammatory inputs in a therapeutically tractable erythropoietic regulatory circuit.

Contrast-enhanced magnetic resonance imaging positively impacts the management of some patients with rheumatoid arthritis or suspected RA.

OBJECTIVE: Early diagnosis of rheumatoid arthritis (RA) is important given the availability of highly effective disease-modifying antirheumatic (DMARD) medications, including biologics. However, because of associated risks and cost, accurately assessing disease activity is critical. Because magnetic resonance imaging (MRI) can detect synovitis and bone marrow edema, both of which may precede erosion development, we sought to determine the impact of enhanced MRI on patient management in a group of patients referred for MRI by rheumatologists. MATERIALS AND METHODS: After institutional review board approval, we evaluated all hand MRI examinations referred by the rheumatology department for synovitis evaluation between September 2007 and May 2009. The magnetic resonance images were classified as positive or negative and later reviewed by 2 musculoskeletal radiologists. A musculoskeletal radiologist and rheumatologist jointly reviewed the patients' medical records to determine the following: (1) Did the MRI findings alter treatment? (2) Were the treatment alterations beneficial? RESULTS: The study included 48 patients (39 women and 9 men) with a mean age of 51 years (range, 18-79 years). Significant management changes initially occurred in 79% (23/29) of the positive (DMARDs added in 20) and in 11% (2/19) of the negative MR examinations with average follow-up of ~300 days. Eighty percent (16/20) of the patients with DMARDs added experienced symptom improvement, none of the patients whose medications were discontinued experienced symptom relapse, and 18% (4/22) of patients without initial therapeutic changes required delayed treatment modifications. CONCLUSIONS: Enhanced MRI significantly altered clinical management in 50% of these patients with RA or suspected RA. Therefore, when the clinical picture in a patient with RA or suspected RA is unclear, enhanced MRI can provide useful guidance for treatment modifications.

Clinical implication of autoantibodies in patients with systemic rheumatic diseases.

Autoantibodies have been used extensively as a useful biomarker in systemic lupus erythematosus and other autoimmune rheumatic diseases. Antinuclear antibodies by immunofluorescence are a standard clinical test to screen for evidence of systemic autoimmunity. Different specific autoantibodies are associated with particular diagnoses, symptoms, unique syndromes, subsets of the disease and clinical activity. They are produced prior to the onset of clinical manifestations and have predictive value. This review focuses on a critical re-evaluation of the clinical significance of autoantibodies. Disease subsets defined by autoantibodies, coexistence of disease marker antibodies, and problems in testing and interpreting results are examined. Clinical approaches in differential diagnosis of antinuclear antibodies and the significance of antinuclear antibodies in healthy individuals are also discussed.