Friday, 13 Jul 2018

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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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Nailfold Capillary Density Predicts Dermatomyositis Lung Involvement

In patients with juvenile dermatomyositis (DM), an association was seen between low nailfold capillary density and pulmonary involvement, European researchers reported.

Systemic Sclerosis: More Common than Expected

Systemic sclerosis is more common in the United Kingdom than previously reported, a nationwide population-based study determined.

Best of 2017: Death Rates from Lupus Remain Disproportionately High

The Annals of Internal Medicine reports that despite improving trends in mortality, death rates from systematic lupus erythematosus (lupus) remain high compared to those in the general population, and disparities persist between subpopulations and geographic regions. Underreporting of lupus on death certificates may have resulted in underestimates of mortality rates. 

Best of 2017: Are ANA Tests Unreliable?

Pisetsky and colleagues have reported in the Annals of Rheumatic Disease that ANA tests done on established SLE patients may yield surprisingly disparate results.

While ANA negative lupus was a problem of old assays and the loss of ANA positivity may be seen with chronicity or age, most rheumatologists believe that ANA positivity is an absolute requirement for the diagnosis of systemic lupus erythematosus.

Abatacept Misses in Lupus Nephritis

Dr. Richard Furie, MD, of Northwell Health in New York, presented the findings ofa large phase III trial of abatacept (Orencia) in lupus nephritis showing the agent failed to achieve a complete renal response.

Data presented at EULAR 2018 showed that at one year, a total of 35.1% of patients receiving abatacept achieved complete renal response, as did 33.5% of those randomized to placebo (P=0.73).