Elimination Diet or Swallowed Topical Steroid Treatment of Pediatric Eosinophilic Esophagitis: Five-Year Outcomes.

BACKGROUND: Eosinophilic esophagitis (EoE) is a chronic, antigen-mediated disease of the esophagus commonly treated with swallowed topical steroids (STS) or elimination diets (EDs). Evidence of a long-term response to EDs in pediatric patients is sparse. OBJECTIVE: Our study sought to understand the natural history of pediatric EoE treated exclusively with EDs and to examine a similar population of STS-treated EoE subjects. We hypothesized that long-term adherence to an effective ED would result in ongoing EoE disease remission. METHODS: We conducted a retrospective study of pediatric EoE subjects who had at least 2 visits to a multidisciplinary clinic. Subjects were identified who had (1) a new referral with a suspected diagnosis of EoE; (2) received either EDs or STS alone, and (3) completed both a diagnostic and a posttreatment endoscopy. Concomitant proton-pump inhibitor use was allowed. We collected demographics, clinical features, treatment plans, and associated side effects on each subject. Remission was defined as fewer than 15 eosinophils/high-powered field. RESULTS: We screened the electronic medical record from 2015 to 2016 for subjects cared for in the Gastrointestinal Eosinophilic Diseases Program who fit criteria for inclusion in this analysis. One hundred ninety-nine subjects were identified, 16 who received exclusive EDs and 15 who were treated with STS. Treatment of these subjects was documented for 4.8 and 5.2 years, respectively (P = .51). Significant differences between the groups were observed in average age at EoE diagnosis (3.5 y ED vs 7.8 y STS; P = .002) and in number of endoscopies (6.6 in ED vs 4.5 in STS; P = .03). Fifteen of 16 subjects treated with ED attained histological remission. The initial effective ED removed a mean of 7.7 foods and the final ED removed a mean of 4 foods. No food impactions or esophageal dilations occurred in the ED group. The STS group required an average of 3.7 dose/formulation changes, 4 subjects required 1 or more dilations, 1 subject had 2 food impactions, and 2 were diagnosed with adrenal insufficiency. CONCLUSIONS: Treatment with either ED or STS can lead to long-term remission of EoE. In this study, fewer side effects developed in the ED group than the STS group, but the validity of this conclusion is limited by the small sample size and reinforces the need for prospective study to explore these initial findings.

Impact of Dose Reduction of Topical Steroids to Manage Adrenal Insufficiency in Pediatric Eosinophilic Esophagitis.

OBJECTIVE: To evaluate the impact of type and dose of swallowed topical steroids (STS) and concurrent steroid therapy on the development and resolution of adrenal insufficiency (AI) in pediatric eosinophilic esophagitis (EoE). METHODS: We performed a retrospective case-control study of pediatric EoE subjects in a single tertiary care center, who were treated with STS for at least 3 months and diagnosed with AI based on a peak stimulated cortisol level of <18 µg/dL (500 nmol/L). Steroid forms and doses, and endoscopy data were collected at the time of AI diagnosis and AI resolution or the last known evaluation. Steroid formulations were converted to a fluticasone-equivalent dose for analysis. RESULTS: Thirty-two EoE subjects with AI were identified, and 20 had AI resolution, including 12 who remained on lower dose STS. Eight of the 32 patients were also treated with extended-release budesonide (ER budesonide), which resulted in a 7-fold higher total daily steroid dose, and thus were analyzed separately. When the 24 cases that were not on ER budesonide were compared to the 81 controls, no difference was found in the STS dose nor total daily steroid dose, although the inhaled steroid dose had marginal significance. Peak eosinophil counts tended to increase when STS doses were decreased, except in subjects on ER budesonide at AI diagnosis. CONCLUSION: Altering the total daily steroid regimen can lead to resolution of AI in patients with EoE, though this may come at the expense of disease control.

Ciclesonide Impacts Clinicopathological Features of Eosinophilic Esophagitis.

BACKGROUND: Eosinophilic esophagitis (EoE) is a chronic allergen-mediated disease of the esophagus. Pharmacologic treatment has largely relied on repurposing corticosteroids. Ciclesonide (CIC) is a corticosteroid for the treatment of asthma with biochemical properties that improve topical potency. OBJECTIVE: To determine whether CIC decreased clinicopathological features of EoE. METHODS: We performed a retrospective cohort study of patients with EoE treated with CIC at a pediatric hospital from 2010 to 2019. Data were extracted from the electronic health record. Patients who were prescribed CIC with pre- and post-CIC endoscopic and histological data available were included for analysis. RESULTS: A total of 281 patients were treated with CIC and 81 met criteria for inclusion. Use of CIC was associated with reduced symptoms including dysphagia (P < .001), abdominal pain (P < .001), vomiting (P = .01), heartburn (P = .02), and behavior changes (P = .02). Average composite endoscopic reference scores decreased from 2.54 to 1.37 (P < .001), with improvement in exudates, edema, and furrows (all P < .001). Peak eosinophil counts decreased from 48 to 23 eosinophils/hpf (P < .001). Forty-three patients (53%) achieved remission (<15 eosinophils/hpf). Esophageal Candida was reported in 1 patient. Fasting morning cortisol concentrations were low in 10 of 31 patients tested. Six of these 10 patients had abnormal adrenocorticotropic hormone stimulation testing, 5 of 6 diagnosed with adrenal insufficiency before transition to CIC and 3 of 6 with subsequent normalization of adrenal function on CIC therapy. CONCLUSIONS: Patients with EoE treated with CIC experienced significant reductions in clinicopathological features of EoE. CIC can be considered an alternative therapy in patients with known adrenal insufficiency or at risk of developing adrenal insufficiency.

The Girl With the Bleeding Earlobe Mass.

ABSTRACT: Pilomatrixomas, also known as epithelioma calcificans, are benign tumors of hair follicle matrix cells that are often mistaken for other lesions, especially cutaneous abscesses. We report an illustrative case in which a teenage girl developed a red, swollen earlobe that required multiple care visits and interventions until definitive diagnosis and treatment were provided. Although the lesion was initially treated as an abscess, it continued to progress in size and discomfort. The correct diagnosis was established after imaging and complete excision with pathologic examination. Ultimately, our patient was subjected to avoidable procedures that carried the risk of potentially negative cosmetic sequelae before the proper intervention. Although abscesses are common, it is important for clinicians to avoid incision and drainage of lesions, unless the diagnosis is certain.

Ulcerative colitis mucosal transcriptomes reveal mitochondriopathy and personalized mechanisms underlying disease severity and treatment response.

Molecular mechanisms driving disease course and response to therapy in ulcerative colitis (UC) are not well understood. Here, we use RNAseq to define pre-treatment rectal gene expression, and fecal microbiota profiles, in 206 pediatric UC patients receiving standardised therapy. We validate our key findings in adult and paediatric UC cohorts of 408 participants. We observe a marked suppression of mitochondrial genes and function across cohorts in active UC, and that increasing disease severity is notable for enrichment of adenoma/adenocarcinoma and innate immune genes. A subset of severity genes improves prediction of corticosteroid-induced remission in the discovery cohort; this gene signature is also associated with response to anti-TNFα and anti-α4ß7 integrin in adults. The severity and therapeutic response gene signatures were in turn associated with shifts in microbes previously implicated in mucosal homeostasis. Our data provide insights into UC pathogenesis, and may prioritise future therapies for nonresponders to current approaches.