Clinical Improvement in Early Onset Interstitial Lung Disease Using Rituximab in Children With Antimelanoma Differentiation-Associated Gene 5-Positive Juvenile Dermatomyositis.

OBJECTIVE: Children with juvenile dermatomyositis (JDM) and antibodies to antimelanoma differentiation-associated gene 5 (anti-MDA5) are at increased risk of severe disease complications, including interstitial lung disease (ILD). Data regarding treatment of disease complications in this patient population are limited. In this study, we examined the disease course of children with JDM and anti-MDA5 antibodies before and after treatment with rituximab (RTX). METHODS: Patients aged 2-21 years and seen at the Children's Hospital at Montefiore between July 2012 and August 2021, with a diagnosis of JDM, positive anti-MDA5 antibodies, and evidence of ILD, and who were treated with RTX were eligible for inclusion. Retrospective clinical and laboratory data were reviewed. RESULTS: Five of 8 patients with positive anti-MDA5 antibodies had evidence of ILD (62.5%). Four patients had data available for review. All patients received at least 5 courses of RTX infusions, with discontinuation of steroids by an average of 12 months after starting RTX and a decrease to fewer than 2 concurrent medications by the fifth course of RTX. Indicators of ILD on high-resolution computed tomography and pulmonary function tests either improved or fully resolved over the course of RTX treatment for all patients. Patients also demonstrated resolution of active cutaneous manifestations and musculoskeletal disease activity. CONCLUSION: To our knowledge, this is the first study to examine the use of RTX in children with JDM and anti-MDA5 antibodies, with notable improvements in ILD, cutaneous, and musculoskeletal manifestations. Further studies are needed to better understand the efficacy of RTX for JDM disease-related complications.

Expanding Understanding of Thyroid Eye Disease Manifestations to Include Hispanic and Black Patients.

PURPOSE: Knowledge of thyroid eye disease (TED) is based on predominantly Caucasian populations. To date, no studies in the United States examine the presentation in Black and Hispanic patients. The purpose of this study is to introduce the presentation of TED in two previously undescribed populations. METHODS: This is a retrospective, cross-sectional, chart review study of patients with TED at a tertiary center using the Strengthening the Reporting of Observational Studies in Epidemiology checklist. The main outcome measure for severity was the European Group on Graves' Orbitopathy 2016 Severity Scale. RESULTS: Of the 2905 charts reviewed, 99 met the inclusion criteria. The mean age was 51 (standard deviation 16) years with 78% women. Race was 49.4% Black, 39.1% Hispanic, 9.2% Caucasian, and 2.3% Asian. Smoking rates were 25% current smokers and 14% former smokers. Manifestations were proptosis (94% Hispanic and 91% Black), eyelid retraction (85% Hispanic and 79% Black), extraocular muscle (EOM) restriction (79% Hispanic and 63% Black), eyelid edema (41% Hispanic and 30% Black), chemosis (24% Hispanic and 14% Black), and optic neuropathy (18% Hispanic and 9% Black). Overall, disease severity was 22% mild, 65% moderate to severe, and 13% sight-threatening. Older patients had increased rates of optic neuropathy (P = 0.04). Younger patients had increased rates of proptosis (P = 0.02). Socioeconomic status was not associated with disease severity (P = 0.67). CONCLUSION: Hispanic and Black patients with TED presented with higher than previously established rates of proptosis, EOM restriction, and optic neuropathy. Including research of different races broadens understanding of presentation and management, improving patient outcomes.

Purpose in Life Among Centenarian Offspring.

OBJECTIVES: Purpose in life (PIL), a feeling of meaning and direction in life, is associated with favorable health outcomes including lower mortality and reduced risk of disease, disability, and cognitive impairment. Since centenarian offspring have been shown to have long health spans we sought to examine whether they have higher PIL than individuals without familial longevity. METHOD: We compared PIL scores from the Ryff Scales of Psychological Well-Being in centenarian offspring from the New England Centenarian Study (N = 361, mean age = 82.0 years) with 3 referent groups: spouses, birth cohort-matched referents, and Health and Retirement Study (HRS) participants. RESULTS: Logistic regression analyses adjusted for age, sex, education, and marital status indicated greater odds of high PIL among centenarian offspring compared with spouse (adjusted odds ratio [aOR] = 1.92, 95% confidence interval [CI] = 1.002-3.68, p = .049) and birth cohort referents (aOR = 2.64, 95% CI = 1.36-5.14, p = .004). Offspring had an almost 3 times greater odds of having high PIL than HRS participants (odds ratio [OR] = 2.93, 95% CI = 2.17-3.96, p < .0001). DISCUSSION: Higher PIL is associated with being an offspring of a long-lived parent and may play a role in the ability to delay age-associated illnesses and functional decline. Increasing purposefulness may be a target for interventions to promote healthy aging.