Thursday, 13 Dec 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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Genetic Diagnosis for Previously Undiagnosed Disorders

The NEJM has reported the NIH's Undiagnosed Diseases Network (UDN) study results of genetically identifying new diseases from prospectively followed persons with undiagnosed disorders. The UDN was formed in 2014 as a network of seven clinical sites, two sequencing cores, a coordinating center, central biorepository, a metabolomics core, and a model organisms screening center. It was established to apply a multidisciplinary model in the evaluation of the most challenging cases and to identify the biologic characteristics of newly discovered diseases.

SLE and Risk of Malignancy

The risk for cancer in systemic lupus erythematosus (SLE) patients has been inconsistently studied.  A new metanalysis shows that SLE has an increased risk for 16 specific cancers and decreased risk for prostate cancer and cutaneous melanoma. 

The Benefits of a Lupus Clinic

A comparative cohort study assessing the quality of care in patients with systemic lupus erythematosus (SLE) suggests that a dedicated lupus clinic has better quality measure performance compared to lupus management in a general rheumatology clinic.

A study from Rush University enrolled 150 consecutive SLE patients - with 73 followed in the general rheumatology clinic and 77 followed in a dedicated lupus clinic. 

Diagnosing Early Lupus from Mimics

A multicenter study of early systemic lupus erythematosus (SLE) patients examined manifestations at disease onset and found that certain clinical features can help to distinguish early SLE from other SLE‐mimicking conditions. 

High CV Risk in Lupus

Patients with systemic lupus erythematosus (SLE) remain at risk for accelerated atherosclerosis, and important questions have yet to be answered, British investigators reported.