Neta Cohen

Acute inflammatory myopathy with severe subcutaneous edema, a new variant? Report of two cases and review of the literature

Abstract. Acute inflammatory myopathy with severe subcutaneous edema is extremely rare and has been reported in only a handful of cases. We describe two similar patients presenting with this disorder and generalized rash. Unlike the five previously reported cases, the clinical and histologic features of our two patients are more suggestive of dermatomyositis than polymyositis. Nevertheless, scrutinizing all seven reported patients, a number of specific characteristics could be defined. All patients were adult males. Dysphagia was present in four. In six patients, acute inflammatory myopathy was idiopathic while malignancy was present in one. Two patients died despite intensive therapy, three improved on corticosteroid treatment, and two recovered spontaneously. In all patients, limb involvement with marked subcutaneous edema was present, clinically mimicking deep vein thrombosis in both our patients. The presence of severe subcutaneous edema may be a hallmark of a distinctive variant of acute inflammatory myopathy. More cases are needed to discern subtypes of this general entity and to establish guidelines for treatment and prognosis.

Efficacy and safety of biological agents for systemic juvenile idiopathic arthritis: a systematic review and meta-analysis of randomized trials

OBJECTIVE To define the optimal biologic agent for systemic JIA (sJIA) based on safety and efficacy data from a randomized controlled trial (RCT). METHODS Through a systematic literature search, sJIA RCTs evaluating biologic agents were identified. The primary efficacy outcome was defined as a 30% improvement according to the modified American College of Rheumatology Paediatric 30 response criteria (JIA ACR30). The primary safety outcome was defined as serious adverse events (SAEs). Outcomes were analysed by pairwise and network meta-analyses. The quality of evidence between biologic agents was assessed by applying the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) methodology. RESULTS From the 493 citations originally identified, 5 RCTs were eligible for inclusion-one each for anakinra, canakinumab and tocilizumab and two for rilonacept: all vs placebo. While all were effective, the network meta-analysis indicated with low-quality evidence (due to indirect comparison and inconsistency) that rilonacept-treated patients were less likely to respond than those treated with canakinumab [odds ratio (OR) 0.10 (95% CI 0.02, 0.38), P = 0.001] or tocilizumab [OR 0.12 (95% CI 0.03, 0.44), P = 0.001]. Risks of SAEs were similar among the biologic agents (supported by very low-quality evidence) and not different from placebo. CONCLUSION Despite heterogeneous eligibility criteria and study designs across the five studies and different modified JIA ACR30 criteria, this meta-analysis of short-term RCTs presents empirical evidence that canakinumab and tocilizumab are more effective than rilonacept. Biologic agents in sJIA seem safe and comparable with respect to SAE risk in the short term.

Ecthyma gangrenosum skin lesions in previously healthy children.

Ecthyma gangrenosum (EG) is an uncommon skin lesion that usually develops in patients with known immune deficiency or who are on immunosuppressive treatment. We report on five previously healthy children presenting with EG. Three of them developed severe neutropaenia. Immunologic work‐up revealed chronic neutropaenia in two.

Biological agents in polyarticular juvenile idiopathic arthritis: A meta-analysis of randomized withdrawal trials ☆ ☆☆

BACKGROUND AND OBJECTIVE Although various biological agents are in use for polyarticular juvenile idiopathic arthritis (pJIA), head-to-head trials comparing the efficacy and safety among them are lacking. We aimed to compare the efficacy and safety of biological agents in pJIA using all currently available randomized withdrawal trials (wRCTs). METHODS A systematic search of MEDLINE, EMBASE, CENTRAL, and clinicaltrials.gov was performed. Eligible wRCTs: patients with pJIA where a biological agent was compared with another biological agent or placebo. Efficacy was evaluated using disease flare as a measure. Adverse events (AEs) and serious AEs were evaluated. Network meta-analysis compared biological agents based on a (empirical Bayes) mixed-effects logistic regression model that combines statistical inference from both direct and indirect comparisons of the treatment effects between biological agents. RESULTS Of 496 references identified, five wRCTs were included-abatacept, adalimumab, anakinra, etanercept, and tocilizumab, one trial each, all vs. placebo. There were no differences in efficacy among biological agents and most showed statistically significant efficacy compared with placebo (nearly all exceptions were in agreement with the original study data). Serious AEs occurred very infrequently (0-8%) and an analysis was not possible. There were no differences for AEs when compared among biological agents or to placebo. CONCLUSION There were no statistical differences among biological agents for efficacy or safety. Overall, biological agents were effective and safe when compared to placebo. Based on these data, other considerations such as price and availability may need to be used to decide among biological agents when treating pJIA patients.

Legionella in Two Splenectomized Patients. Coincidence or Causal Relationship

Abstract.We describe two splenectomized patients admitted with pneumonia. The course in one was complicated by overwhelming multiorgan failure when the only indicative laboratory result was seropositivity for Legionella hackeliae and Legionella longbeachae. He was initially treated with ceftriaxone and roxithromycin, followed by levofloxacin as well as intensive supportive treatment, and survived. The second patient was seroreactive for Legionella micdadei. In some cases of pneumonia in splenectomized patients tentatively considered to be caused by Streptococcus pneumoniae, the causative agent might have, in fact, been Legionella. We suggest that splenectomy be considered a possible predisposing factor for Legionella pneumonia. Since prompt diagnosis of Legionella infection, especially the non-pneumophila species, is extremely difficult, alertness to this diagnostic option and early empirical initiation of appropriate aggressive antibiotic treatment may be of critical importance.

Spacing of cue-approach training leads to better maintenance of behavioral change

The maintenance of behavioral change over the long term is essential to achieve public health goals such as combatting obesity and drug use. Previous work by our group has demonstrated a reliable shift in preferences for appetitive foods following a novel non-reinforced training paradigm. In the current studies, we tested whether distributing training trials over two consecutive days would affect preferences immediately after training as well as over time at a one-month follow-up. In four studies, three different designs and an additional pre-registered replication of one sample, we found that spacing of cue-approach training induced a shift in food choice preferences over one month. The spacing and massing schedule employed governed the long-term changes in choice behavior. Applying spacing strategies to training paradigms that target automatic processes could prove a useful tool for the long-term maintenance of health improvement goals with the development of real-world behavioral change paradigms that incorporate distributed practice principles.

Trends in the diagnosis and management of anaphylaxis in a tertiary care pediatric emergency department

BACKGROUND Underdiagnosis of anaphylaxis is a major concern in the pediatric emergency department (PED), leading to failure to administer and prescribe intramuscular epinephrine treatment. OBJECTIVE To examine the clinical features, triggers, and management of anaphylaxis in the PED, with a special focus on the rate of cases diagnosed and treated correctly over time, and to compare correctly diagnosed and misdiagnosed cases. METHODS All records of patients presenting to a tertiary care PED between 2013 and 2016 with a final diagnosis of anaphylaxis or allergic reaction were reviewed. RESULTS The rate of anaphylaxis increased from 0.1% between 2013 and 2014 and 0.24% between 2015 and 2016. Symptoms such as breathing difficulties and wheezing were found significantly less among misdiagnosed patients compared with patients correctly diagnosed with anaphylaxis. Food was the most common causative agent in both of the study periods (88% in 2013-2014 and 91% in 2015-2016), with milk (20% in 2013-2014 and 28% in 2015-2016) and tree nuts (23.1% in 2013-2014 and 23.7% in 2015-2016 as the most prevalent identified triggers. Intramuscular epinephrine treatment in the prehospital and hospital settings and the automatic epinephrine injector prescription rate did not change significantly throughout the study. Referral to an allergist increased from 68% in 2013 to 2014 to 90% in 2015 to 2016. CONCLUSION The rate of visits attributable to anaphylaxis in our PED doubled during the study period, with milk allergy as the most common trigger. Most cases of misdiagnosed and undertreated anaphylaxis had no respiratory signs and symptoms. Novel methods to improve recognition of anaphylaxis and adherence to treatment guidelines are needed.