Friday, 22 Mar 2019

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Hemophagocytic Syndrome in Adults

Hemophagocytic lymphohistiocytosis(HLH) is a rare complication of many disorders. In children, systemic JIA is by far the most common cause of HLH. In this review of 73 adult patients from an academic center the most common associations were with infections (41.1%), malignancies (28.8%), autoimmune disorders (6.8%) and idiopathic disorders (17.8%).

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Resistant Hypertension Common in SLE

Rates of resistant hypertension were doubled among patients with systemic lupus erythematosus (SLE), significantly increasing their mortality risks, a retrospective single-center study found.

Among patients with SLE, the prevalence of resistant hypertension was 10.2% compared with 5.3% among controls (OR 2.02, 95% CI 1.60-2.55, P<0.0001), according to Cecilia P. Chung, MD, and colleagues from Vanderbilt University in Nashville.

Improved Mortality Trends in Lupus Nephritis

The mortality rate from end- stage renal disease (ESRD) in lupus nephritis (LN) patients has significantly decreased in the last decade.

Cyclosporin and IVIG Effective in Kawasaki's Disease

A Lancet study has shown that adding cyclosporin to intravenous immunoglobulin (IVIG) Kawasaki disease patients, who were predicted to be resistant to IVIG, was both safe and effective in averting severe coronary artery outcomes.

Brazilian Guidelines for Systemic Autoimmune Myopathies

The Myopathy Committee of the Brazilian Society of Rheumatology (BSR) has put forward recommendations for the management of patients with systemic autoimmune myopathies (SAM).

The challenge has been that there is a paucity of effective controlled clinical trials, and hence, glucocorticoids (GC) have played a first-line and prominent role in the treatment of SAM.

EULAR Consensus Guidelines for Juvenile Localized Scleroderma

A EULAR international committee of 15 experts in pediatric rheumatology have established consensus-based recommendations for the management of juvenile localised scleroderma (JLS).

JLS is an uncommon disorder that is commonly seen by pediatric and adult rheumatologists. Evidence-based guidelines are sparse and management is mostly based on physicians’ experience.