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Objective: To investigate the characteristics and risk factors of a novel parenchymal lung disease (LD), increasingly detected in systemic juvenile idiopathic arthritis (sJIA).Methods: In a multicentre retrospective study, 61 cases were investigated using physician-reported clinical information and centralised analyses of radiological, pathological and genetic data.Results: LD was associated with distinctive features, including acute erythematous clubbing and a high frequency of anaphylactic reactions to the interleukin (IL)-6 inhibitor, tocilizumab. Serum ferritin elevation and/or significant lymphopaenia preceded LD detection. The most prevalent chest CT pattern was septal thickening, involving the periphery of multiple lobes ± ground-glass opacities. The predominant pathology (23 of 36) was pulmonary alveolar proteinosis and/or endogenous lipoid pneumonia (PAP/ELP), with atypical features including regional involvement and concomitant vascular changes. Apparent severe delayed drug hypersensitivity occurred in some cases. The 5-year survival was 42%. Whole exome sequencing (20 of 61) did not identify a novel monogenic defect or likely causal PAP-related or macrophage activation syndrome (MAS)-related mutations. Trisomy 21 and young sJIA onset increased LD risk. Exposure to IL-1 and IL-6 inhibitors (46 of 61) was associated with multiple LD features. By several indicators, severity of sJIA was comparable in drug-exposed subjects and published sJIA cohorts. MAS at sJIA onset was increased in the drug-exposed, but was not associated with LD features.Conclusions: A rare, life-threatening lung disease in sJIA is defined by a constellation of unusual clinical characteristics. The pathology, a PAP/ELP variant, suggests macrophage dysfunction. Inhibitor exposure may promote LD, independent of sJIA severity, in a small subset of treated patients. Treatment/prevention strategies are needed. [ABSTRACT FROM AUTHOR]

Introduction: Clinical and electrophysiological studies to measures disease activity in juvenile myasthenia gravis (JMG) are limited.Methods: Retrospective review of the clinical profile, Myasthenia Gravis Foundation of America (MGFA) scores, serial stimulated jitter analysis (Stim-JA) of the orbicularis oculi muscle, grip strength, and spirometry of patients with JMG who were followed in a multidisciplinary clinic was performed.Results: Thirteen patients with JMG (9 females) with mean age of 13.2 ± 4.8 years and follow-up duration of 25.3 ± 8.3 months (range, 6-39) with ≥ 2 Stim-JA recordings were included. The mean jitter, mean percentage of apparent single-fiber action potentials (%ASFAP) with increased jitter, and mean %ASFAP with blocking at baseline values (77.3 ± 54.7 µs, 64.3% ± 35.8%, 39% ± 38.6%, respectively) and at follow-up (53 ± 45.4 µs, 51.2% ± 34.5%, 17% ± 29.4%, respectively) were abnormal; however, no statistically significant interval difference was noted. The electrophysiological data correlated significantly with Myasthenia Gravis Foundation of America (MGFA) class. Grip strength and spirometry did not correlate with MGFA class.Discussion: Stimulated jitter values are sensitive biomarkers in JMG. Muscle Nerve 58: 729-732, 2018. [ABSTRACT FROM AUTHOR]

Introduction: Weight concerns may inhibit smoking quit attempts and may be more influential amongst African-Americans who are more likely to be overweight.Aims: To assess if weight perception is associated with readiness to quit and whether this relationship is modified by race.Methods: We used data from a cohort of current smokers undergoing routine health examinations. Based on differences between ideal and measured BMI, participants’ weight perceptions were classified as within, somewhat above, or far above ideal weight. Logistic regression analysis was used to evaluate adjusted associations of weight perception and race with readiness to quit.Results: Of 2,831 current smokers, 23% were obese and 38% overweight. Amongst white smokers, those who perceived being far above ideal weight were more likely to be ready to quit (OR: 1.45, 95% CI: 1.03–2.03), but this association was not observed for African-American smokers who perceived themselves to be somewhat or far above their ideal weight (OR: 0.35, 95% CI: 0.10–1.24 and OR: 0.36, 95% CI: 0.11–1.19, respectively).Conclusions: Perception of being overweight is associated with increased readiness to quit amongst white but not African-American smokers. Smoking cessation programmes may need to culturally tailor interventions based on smokers’ weight perceptions. [ABSTRACT FROM PUBLISHER]

Introduction: This study's objective was to evaluate quantitative electromyography (QEMG) using multiple-motor-unit (multi-MUP) analysis in Duchenne muscular dystrophy (DMD).Methods: Ambulatory DMD boys, aged 5-15 years, were evaluated with QEMG at 6-month intervals over 14 months. EMG was performed in the right biceps brachii (BB) and tibialis anterior (TA) muscles. Normative QEMG data were obtained from age-matched healthy boys. Wilcoxon signed-rank tests were performed.Results: Eighteen DMD subjects were enrolled, with a median age of 7 (interquartile range 7-10) years. Six-month evaluations were performed on 14 subjects. QEMG showed significantly abnormal mean MUP duration in BB and TA muscles, with no significant change over 6 months.Conclusions: QEMG is a sensitive electrophysiological marker of myopathy in DMD. Preliminary data do not reflect a significant change in MUP parameters over a 6-month interval; long-term follow-up QEMG studies are needed to understand its role as a biomarker for disease progression. Muscle Nerve 56: 1361-1364, 2017. [ABSTRACT FROM AUTHOR]