Eosinophilic esophageal myositis: a comparative study of distinguishing features,diagnostic challenges,and therapeutic insights

Abstract

Background:

Eosinophilic esophageal myositis (EoEM) involves eosinophilic infiltration of muscle layers but spares the epithelium, differing from eosinophilic esophagitis (EoE). EoEM is rare and underrecognized, with challenges in diagnosis and treatment.

Objectives:

To compare the clinical, endoscopic, pathological, and manometric characteristics of EoEM with mucosal-predominant and muscle-predominant EoE, and to elucidate differences and interconnections between these conditions.

Design:

Retrospective cohort study with literature review.

Methods:

The retrospective study included patients diagnosed with mucosal-predominant EoE (n = 21), muscle-predominant EoE (n = 6), and EoEM (n = 7) at Nanjing Drum Tower Hospital from 2019 to 2024, along with a review of EoEM cases reported in the literature (n = 11). Muscle thickness was measured using endoscopic ultrasonography and computed tomography (CT). High-resolution esophageal manometry was performed to evaluate esophageal motility and diagnose motility disorders according to the Chicago Classification v4.0 criteria. Clinical, endoscopic, pathological, manometric, and treatment data were analyzed.

Results:

EoEM patients were older and had more severe dysphagia, food impaction, and weight loss. Endoscopic findings revealed a distinct corkscrew-like appearance and luminal compression without typical mucosal inflammation. High immunoglobulin E (IgE) may be a marker of EoEM. Compared with muscle-predominant EoE, EoEM patients had higher maximum distal contractile integral values and a higher frequency of Jackhammer esophagus, although they exhibited similar muscle thickness. EoEM patients resisted proton pump inhibitors but responded favorably to oral steroids and Per-Oral Endoscopic Myotomy (POEM).

Conclusion:

EoEM is characterized by severe esophageal motility dysfunction. Muscle biopsy should be considered in patients with frequent dysphagia, motility disorders, and high serum IgE levels. Oral steroids and POEM effectively relieve symptoms.

Plain Language Summary

Study comparing a rare muscle-focused swallowing disorder (EoEM) with the more common type of swallowing disorder (EoE)

Why was the study done? Eosinophilic Esophageal Myositis (EoEM) is a rare disease where immune cells attack the muscle layer of the swallowing tube, causing severe symptoms. It is often confused with the more common Eosinophilic Esophagitis (EoE), which affects the inner lining. This study aimed to clearly define EoEM and identify better ways to diagnose and treat it. What did the researchers do? Researchers analyzed data from 34 patients and 11 published cases, comparing three groups: common EoE, a severe muscle-involved EoE subtype, and pure EoEM. They examined symptoms, scan results, swallowing tests, biopsy findings, and treatment responses. What did the researchers find? Patients with EoEM were older and had worse symptoms like food stuck and weight loss. Their scans showed a twisted “corkscrew” esophagus but no typical inner lining inflammation. Swallowing tests revealed severe muscle spasms. Diagnosing EoEM requires a muscle biopsy (taken via a special needle), as standard biopsies of the inner lining are normal. EoEM does not respond to common acid medicines but improves significantly with oral steroid pills. A procedure called POEM effectively relieves severe swallowing difficulties. What do the findings mean? Doctors should suspect EoEM in patients with long-lasting severe swallowing problems, muscle spasms on tests, and high allergy-related (IgE) blood levels, even if the esophageal lining looks normal. Confirming it requires a muscle biopsy. Recognizing this distinct condition is crucial, as effective treatments like steroids and POEM are available.

Keywords

dysphagia eosinophilic esophageal myositis EUS-FNB motility dysfunction POEM

Introduction

Eosinophilic esophagitis (EoE) is a chronic, immune-mediated disease characterized by eosinophilic infiltration in the esophageal mucosa and esophageal dysfunction.¹ It is understood that an allergic response to food or environmental allergens causes chronic inflammation of the esophageal epithelium.² Histologically, EoE is defined by an eosinophil count of ⩾15 eosinophils per high-power field (eos/hpf).³ As the disease progresses, inflammation can extend beyond the mucosa, with deep tissue biopsies showing eosinophil infiltration and fibrosis in the subepithelial layer.⁴ Endoscopic ultrasonography (EUS) demonstrated that the total thickness of the distal esophagus was the most relevant measurement for diagnosing EoE and relevant with dysphagia in EoE patients.^5,6 EoE relates to motility disorders, with the pooled prevalence of 2%, 10%, and 1%, for achalasia, esophagogastric-junction outflow obstruction, and hypercontractile disorders confirmed by manometry.⁷ Scientists raised the identification of a muscle-predominant subtype of EoE, primarily involving the muscular layer and linked with esophageal motility disorders.⁸

Eosinophilic esophageal myositis (EoEM) is a rare but severe condition characterized by eosinophilic infiltration of the esophageal muscularis propria, without involvement of the epithelium.⁹ The nomenclature of EoEM was established in 2015, but similar cases had been reported earlier as a form of EoE. In 1978, Landres et al. reported a case of vigorous achalasia with significant hypertrophy and eosinophilic infiltration of the esophageal muscular layer, notably without eosinophilia in the epithelium or intestines.¹⁰ Over the subsequent decades, EoEM cases were sporadically reported and regarded as a subtype of EoE.^3,8,10 However, EoEM has different eosinophilic infiltration patterns from EoE. EoE is marked by epithelial eosinophilia, whereas multi-point mucosal biopsies in EoEM reveal no epithelial involvement.^8,11,12 Emerging evidence suggests that EoEM may have a distinct immunological profile from EoE.¹³ EoEM can closely resemble muscle-predominant EoE, so the two conditions are often confused. It is essential to pay greater attention to their differentiation.

EoEM patients frequently exhibit esophageal motility disorders, such as Jackhammer esophagus (JE), as detected by high-resolution esophageal manometry (HREM).^{9,10,13
–21} Microscopically, eosinophils in EoEM predominantly infiltrate the perivascular and perimysial regions of the muscularis propria.^{9,10,13,15,16} Autopsy findings have also revealed eosinophilic infiltration within the myenteric and endomysial tissues surrounding the myenteric plexus.²⁰ Damage to the myenteric plexus can result in esophageal motility disorders, where peristalsis becomes non-functional, and the lower esophageal sphincter fails to relax properly, leading to dysphagia and food retention.²² This observation implies an intrinsic link between muscle-predominant EoE, EoEM, and esophageal motility disorders.

We hypothesize that EoEM may be part of the spectrum of eosinophilic esophageal disorders, sharing overlapping but distinct features with EoE in terms of pathology, clinical presentation, and treatment response. This study aims to elucidate the differences and interconnections between EoEM and EoE, raising awareness of EoEM while contributing to its early detection and treatment. By analyzing the clinical, endoscopic, pathological, and manometric characteristics of EoEM, we seek to refine its classification and lay the groundwork for future research into the underlying mechanisms of this complex disorder.

Methods and materials

Study and patients

The retrospective cohort study conforms to the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) statement.²³ We retrospectively reviewed medical records of patients with symptomatic esophageal eosinophilia affecting any layer of the esophagus at Nanjing Drum Tower Hospital, Nanjing, China, from January 2019 to January 2024. During the study period, 1309 consecutive patients presented with esophageal symptoms and received gastroscopy. Among them, 220 presented with abnormal endoscopic esophageal manifestations and underwent systematic evaluation, including mucosal biopsies and EUS (Endoscopic Ultrasound-Guided Fine Needle Biopsy (EUS-FNB) if necessary) as clinically indicated. Among them, 84 patients had esophageal eosinophilia (⩾15 eos/hpf) affecting any layer of the esophagus and were included in the study. Exclusion criteria were as follows: (1) Patients diagnosed with secondary causes of eosinophil infiltration (e.g., Gastroesophageal Reflux Disease (GERD) or parasitosis), excluded through: (a) predominant GERD symptoms (heartburn, regurgitation) without dysphagia, (b) upper endoscopy with careful evaluation for GERD-related changes (esophagitis, Barrett’s esophagus), (c) pH monitoring when clinically indicated, (d) exclusion of patients with predominant GERD symptoms responding to proton pump inhibitor (PPI), and (e) exclusion of patients with parasitic infections or drug-induced eosinophilia based on history and laboratory testing; (2) Patients with concomitant high eosinophilic infiltration in the stomach or duodenum; (3) Patients with esophageal tumors; and (4) Incomplete data. All patients were followed up through outpatient visits. After the exclusion process, 34 patients were diagnosed as mucosal-predominant EoE, muscle-predominant EoE, or EoEM following diagnostic criteria. The muscle thickness of all EoEM patients is measured by EUS (radial) and computed tomography (CT). Demographic, laboratory, endoscopic, pathology, EUS, HREM, and treatment data were extracted from electronic medical records. We compared the mucosal-predominant EoE, muscle-predominant EoE, and EoEM within our cohort and with EoEM cases reported in the literature. An additional 11 published cases were included for comparison.^{9,10,13
–21} See Table 1 and Table S1 for details.

Table 1.

Demographic and clinical characteristics of patients with mucosal-predominant EoE, muscle-predominant EoE, and EoEM (n = 34).

Variable	Mucosal-predominant EoE (n = 21)	Muscle-predominant EoE (n = 6)	EoEM (n = 7)	Overall p value
Women, n (%)	6 (28.6)	3 (50.0)	0 (0)	0.118
Age, year, median [IQR]	41.0 [29.0, 54.0]	55.0 [49.5, 62.8]	67.0 [58.0, 69.0]	0.012^a
Allergy history, n (%)	9 (42.9)	1 (16.7)	2 (28.6)	0.455
Asthma, n (%)	2 (9.5)	1 (16.7)	0 (0)	0.563
Allergic rhinitis, n (%)	5 (23.8)	0 (0)	0 (0)	0.163
Eczema, n (%)	4 (19.0)	0 (0)	2 (28.6)	0.389
Symptoms
Dysphagia, n (%)	8 (38.1)	5 (83.3)	7 (100)	0.007^a
Transient food impaction, n (%)	3 (14.3)	4 (66.7)	5 (71.4)	0.009^a,b
Heartburn, n (%)	12 (57.1)	5 (83.3)	4 (57.1)	0.488
Chest pain, n (%)	9 (42.9)	2 (33.3)	1 (14.3)	0.389
Unintentional weight loss, n (%)	3 (14.3)	1 (16.7)	5 (71.4)	0.009^a
Symptom duration at diagnosis, months, median [IQR]	2.0 [1.0, 3.0]	1.0 [1.0, 1.8]	8.0 [4.0, 36.0]	0.088
Symptom frequency				<0.001
Weekly, n (%)	14 (66.7)	0 (0)	1 (14.3)	^a,b
Daily, n (%)	7 (33.3)	4 (66.7)	2 (28.6)
Each meal, n (%)	0 (0)	2 (33.3)	4 (57.1)	^a
Eckardt score, median [IQR]	3.0 [2.0, 4.0]	5.0 [4.0, 7.5]	8.0 [5.0, 8.0]	0.002^a,b
Serum eosinophils, /μL, median [IQR]	240 [160, 360]	170 [100, 1090]	560 [170, 570]	0.711
Percentage of eosinophils, %, median [IQR]	4.6 [2.8, 5.0]	4.4 [1.5, 16.8]	7.3 [2.6, 7.5]	0.753
Endoscopic findings
Edema, n (%)	20 (95.2)	6 (100)	4 (57.1)	0.038^a
White exudates, n (%)	11 (52.4)	3 (50)	0 (0)	0.023^a
Longitudinal furrows, n (%)	17 (81.0)	4 (66.7)	0 (0)	<0.001^a,c
Rings, n (%)	5 (23.8)	2 (33.3)	4 (57.1)	0.263
Strictures, n (%)	0 (0)	2 (33.3)	3 (42.9)	0.011^a,b
Corkscrew-like appearance, n (%)	0 (0)	0 (0)	3 (42.9)	0.011^a
Luminal compression, n (%)	0 (0)	2 (33.3)	5 (71.4)	<0.001^a,b
EREFS, median [IQR]	5.0 [4.0, 6.0]	5.0 [3.3, 6.0]	5.0 [1.0, 6.0]	0.643
Mean EREFS for rings	0.29	1.00	1.71	0.001^a
Mucosal eosinophils,/hpf, median [IQR]	60.0 [50.0, 100.0]	90.0 [57.5, 115.0]	0.0 [0.0, 3.5]	<0.001^a,c
I-SEE (total), median [IQR]	6.0 [5.0, 8.0]	10.0 [8.5, 10.8]	10.0 [7.0, 12.0]	0.009^a,b
I-SEE (symptoms and complications)	2.0 [1.0, 3.0]	4.0 [4.0, 5.5]	6.0 [5.5, 8.0]	<0.001^a,b
I-SEE (inflammatory features)	3.0 [3.0, 4.0]	3.5 [2.3, 4.0]	2.0 [1.5, 2.5]	0.006^a
I-SEE (fibrostenotic features)	0.0 [0.0, 2.0]	2.0 [2.0, 3.5]	1.0 [0.0, 2.5]	0.116

Post hoc pairwise comparison: ^ap < 0.05 for mucosal-predominant EoE versus EoEM; ^bp < 0.05 for mucosal-predominant EoE versus muscle-predominant EoE; ^cp < 0.05 for muscle-predominant EoE versus EoEM.

EoE, eosinophilic esophagitis; EoEM, eosinophilic esophageal myositis; EREFS, Endoscopic EoE Reference Score; IQR, interquartile range; I-SEE, Index of Severity for Eosinophilic Esophagitis; PPI, proton pump inhibitor.

Sample size

Given the rarity of EoEM, formal sample size calculation was not feasible prior to the study. Instead, we employed a consecutive enrollment approach, including all patients who met the diagnostic criteria for EoEM, mucosal-predominant EoE, or muscle-predominant EoE at our institution during the 5-year study period. This resulted in 7 EoEM patients, 6 muscle-predominant EoE patients, and 21 mucosal-predominant EoE patients. While this sample size is small, it represents one of the largest single-institution cohorts of EoEM patients reported to date, given the rarity of this condition.

Diagnostic criteria

Eosinophilic esophagitis

The diagnosis of EoE was based on biopsy results showing elevated intraepithelial eosinophils in the esophagus (⩾15 eos/hpf), with no concurrent eosinophil infiltration in the stomach or duodenum, and other alternative causes of esophageal eosinophilia excluded.^24
–26 Muscle-predominant EoE was defined as EoE accompanied by esophageal muscularis propria thickening greater than 2.0 mm on EUS.⁶ Muscle biopsy was performed to confirm eosinophilic infiltration.

Eosinophilic esophageal myositis

EoEM was defined as the presence of symptomatic eosinophilia in the muscle layer (⩾15 eos/hpf) but with no or only mild epithelial eosinophilia (<5 eos/hpf).¹⁹ Patients with both mucosal eosinophilia ⩾5 eos/hpf and muscular infiltration ⩾15 eos/hpf were classified as EoE with muscular involvement (muscle-predominant EoE), rather than EoEM. All 11 literature review patients fulfilled the diagnostic criteria for EoEM, with <5 eos/HPF in the mucosa and ⩾15 eos/hpf in the muscularis propria. Specifically, 9 cases showed no mucosal eosinophils,^{10,13,15
–21} while 2 cases had 1–4 eos/hpf in the mucosa.^9,14

Endoscopy

Endoscopy was performed by experienced endoscopists on all patients presenting with esophageal symptoms such as dysphagia and regurgitation. Typical endoscopic findings of EoE, such as longitudinal furrows, fixed rings, edema, exudate, and strictures, were assessed and graded using the Endoscopic EoE Reference Score (EREFS), a grading system for EoE-related esophageal features.²⁷ In EoEM, circular luminal narrowing was more consistent with spastic contractions rather than fixed fibrotic rings. To avoid confusion, we re-termed these findings accordingly in figures and tables.

High-resolution esophageal manometry

HREM was selectively performed based on clinical indication, not as a routine examination for all patients. The indications for HREM included: (i) clinically significant dysphagia (frequent food impaction, severe dysphagia, or unintentional weight loss); (ii) suspected esophageal motility dysfunction; or (iii) persistent dysphagia with non-diagnostic or negative findings on esophagogastroduodenoscopy (EGD) and mucosal biopsies. This approach is consistent with standard clinical practice for evaluating non-obstructive dysphagia when routine evaluation is unrevealing.

Among the study cohort, HREM was performed in 3 of 21 patients (14.3%) with mucosal-predominant EoE, 6 of 6 patients (100%) with muscle-predominant EoE, and 7 of 7 patients (100%) with EoEM. The differential utilization of HREM reflects the clinical suspicion of underlying motility disorders, particularly in patients with muscle-predominant disease or when initial endoscopic and histologic findings were insufficient to explain the severity of symptoms.

HREM was conducted by experienced operators (with over 200 cases per year) using the Manoscan, a 36-channel, solid-state system (Medtronic), following a standard protocol.²⁸ Data were analyzed by a professional physician (with over 200 diagnoses per year) using Medview 2.1.1 software (MedKinetic). Reports were generated based on the Chicago Classification v4.0 criteria.²⁹

EUS and CT

The muscle thickness was measured by both EUS (radial scanning) and CT. EUS was performed using a radial echoendoscope (GF-UE260; Olympus Medical Systems, Tokyo, Japan) to visualize the esophageal wall layers. CT scans were obtained using a 64-slice multidetector CT scanner (Siemens Healthineers, Erlangen, Germany) with 2.5 mm slice thickness. EUS measurements were standardized to ensure consistency. Esophageal wall thickness was measured at 5 cm intervals starting from the upper esophageal sphincter (20 cm from incisors) down to the lower esophageal sphincter. To minimize artifactual wall thinning caused by over-distension, measurements were obtained with the esophageal lumen minimally distended with water. All measurements were performed with the patient in the left lateral decubitus position under conscious sedation. The esophageal wall layers were identified based on the standard five-layer EUS structure. The muscularis propria was identified as the fourth, hypoechoic layer.³⁰ Among the reported 11 cases in literature, the muscle thickness was measured by both EUS and CT in 4 cases,^9,16,19,21 by EUS only in 5 cases,^{13
–15,17,18} and by specimen measurement after surgery in 2 cases.^10,20 Regarding measurement protocols, there was heterogeneity among the literature cases. Among those with available methodology details: two cases reported measurements at the distal esophagus only^9,10; seven cases reported measurements in the middle and distal esophagus^{13,15
–19,21}; and two cases reported diffuse thickening throughout the entire esophagus.^14,20 Only five cases reported specific maximum thickness values.^{10,15,16,19,21} In contrast, our institutional protocol standardized measurements at 5 cm intervals from the upper esophageal sphincter to the lower esophageal sphincter to ensure consistency.

Biopsy methods

Mucosal biopsy

Conventional six-site esophageal mucosal biopsies were obtained from different anatomical sites within the esophagus. Histological analysis was performed using Hematoxylin-Eosin (HE) staining to assess eosinophil density (per high-power field) in the tissue specimens.

POEM-b and EUS-FNB

During Per-Oral Endoscopic Myotomy with Biopsy (POEM-b), full-thickness muscle biopsies were taken from the muscularis propria layer using biopsy forceps. During EUS-FNB, the linear EUS scope used was GF-UCT 260 (Olympus Medical System, Tokyo, Japan). EUS-FNB was performed using a 22 gauge needle (Cook Medical Inc., Winston-Salem, NC, USA) with an average of 3 passes per 5 cm of lesion length (range 2–4 passes), avoiding blood vessels. Rapid on-site evaluation (ROSE) was performed to ensure adequate sampling, and the procedure was terminated when sufficient diagnostic material was obtained. Among our seven EoEM patients, four underwent both EUS-FNB and POEM-b (during Per-Oral Endoscopic Myotomy (POEM)), two underwent POEM-b only, and one underwent EUS-FNB only. The biopsy results were concordant between EUS-FNB and POEM-b in all four patients who underwent both procedures, demonstrating good agreement between the two biopsy methods. Among the 11 literature cases, biopsy methods included: EUS-FNB in 3 cases,^9,16,21 surgical biopsy during myotomy in 2 cases,^10,20 endoscopic biopsy during POEM in 5 cases,^{13,15,17
–19} and Endoscopic Mucosal Resection (EMR) using a band ligation and snare technique in 1 case.¹⁴

Index of Severity for Eosinophilic Esophagitis

The Index of Severity for Eosinophilic Esophagitis (I-SEE) was used to assess disease severity during clinic visits. It consists of three aspects: symptoms and complications (symptoms: 1, 2, or 4 points; complications: 2, 4, or 15 points), inflammatory features (endoscopy: 1 or 2 points, histology: 1 or 2 points), and fibrostenotic features (endoscopy: 1, 2, or 15 points, histology: 2 points). The scale ranges from 1 to 6 for mild, 7 to 14 for moderate, and ⩾15 for severe EoE.³¹

Treatment

We reviewed the medication usage and treatment strategies in patients, with a focus on PPIs, steroid therapy, and POEM procedures. PPI responsiveness was defined as the resolution of symptoms and improvement of endoscopic/histologic features after at least 8 weeks of treatment. Some patients who did not respond to PPI therapy received oral steroids with informed consent. Steroid therapy consisted of systemic oral prednisone (0.5 mg/kg/day, tapered). In our cohort, topical steroids were not used. Patients with severe esophageal obstruction were treated with POEM, with the planned myotomy length determined based on endoscopic and HREM data. Among our 7 EoEM patients, 6 (85.7%) underwent POEM, including 3 receiving oral steroids after the POEM procedure, and 1 patient (14.3%) was treated with oral steroids alone due to patient preference and less severe symptoms. Treatment approaches among the 11 literature cases varied (Table 1 and Table S1). Six patients (54.5%) underwent POEM or surgical myotomy,^{10,13,15,17
–19} five received systemic oral steroids,^13
–16,19 one was treated with Potassium-Competitive Acid Blocker (PCAB) plus topical corticosteroids,²¹ and one underwent esophagectomy for suspected malignancy.⁹ PPI resistance was documented in five cases prior to alternative therapy initiation.^{13,15,17,19,21} Some patients received multiple treatment modalities sequentially.

Statistical analysis

Continuous variables were expressed as medians with interquartile ranges (IQR) and compared using the Wilcoxon rank-sum test. Comparisons among three groups were made using Kruskal–Wallis tests for continuous variables, with post hoc Dunn’s tests where applicable. Previous Wilcoxon results were rechecked with this method and did not alter overall conclusions. Categorical variables were presented as counts (N) and percentages (%) and compared using the Chi-square test or Fisher’s exact test. A p-value of <0.05 was considered statistically significant. R Studio v4.2.0 (Posit, PBC) was used for statistical analyses.

Results

Study flow diagram and patient characteristics

The participant enrollment flowchart is shown in Figure 1. A total of 34 patients at our center were included in the study, categorized into three groups: mucosal-predominant EoE (n = 21), muscle-predominant EoE (n = 6), and EoEM (n = 7). Table 1 and Figure S1 show the demographic, clinical, and endoscopic characteristics. The mucosal-predominant EoE group had an earlier onset age than the EoEM group (41.0 vs 67.0 years, p = 0.012). On symptoms, the EoEM group had more dysphagia (100% vs 38.1%, p = 0.007), transient food impaction (71.4% vs 14.3%, p = 0.009), and unintentional weight loss (71.4% vs 14.3%, p = 0.009) compared with the mucosal-predominant EoE group. Patients with EoEM experienced the most frequent symptoms and had the highest Eckardt scores, followed by those with muscle-predominant EoE (median Eckardt scores: 8 vs 5 vs 3, p < 0.01).

Figure 1.

The flowchart of participant enrollment.

Endoscopic findings revealed that the mucosal-predominant EoE group exhibited more severe mucosal inflammatory features than the EoEM group, including edema (95.2% vs 57.1%, p = 0.038), white exudates (52.4% vs 0%, p = 0.023), and longitudinal furrows (81.0% vs 0%, p < 0.001). Rings ranked a higher stage in EoEM than in mucosal-predominant EoE (mean ERERS for rings: 1.71 vs 0.29, p = 0.001). Compared with typical EoE, EoEM had a higher incidence of corkscrew-like appearance (42.9% vs 0%, p = 0.011), strictures (42.9% vs 0%, p = 0.011), and luminal compression (71.4% vs 0%, p < 0.001). Muscle-predominant EoE had mucosal features resembling mucosal-predominant EoE, and the degree of rings and strictures lay between mucosal-predominant EoE and EoEM. Figure 2 shows typical endoscopic images and histological features of EoE or EoEM.

Figure 2.

Endoscopic images and histological features. (a) Longitudinal furrows and (b) mild rings in the EoE group; (c) corkscrew-like appearance in the EoEM group. Black arrows indicate eosinophilia.

When analyzing the I-SEE, both EoEM (p < 0.001) and muscle-predominant EoE (p = 0.006) groups showed more severe symptoms and complications compared with the mucosal-predominant EoE group (mean I-SEE for symptoms and complications: 6 vs 4 vs 2). EoEM showed a lower degree of inflammatory features than mucosal-predominant EoE (mean I-SEE for inflammatory features: 2 vs 3, p = 0.006). No significant differences were observed between the three groups in terms of gender distribution, prevalence of allergies and associated diseases, serum eosinophil count, and the I-SEE fibrostenotic features.

Comparison of esophageal motility between muscle-predominant EoE and total EoEM groups

Table 1 and Table S2 summarizes the clinical, endoscopic, and manometric characteristics of EoEM groups at our center (n = 7) and those reported in the literature (n = 11), and it revealed no significant difference. Table 2 illustrates a comparative analysis of the key characteristics of muscle-predominant EoE and total EoEM groups, focusing on esophageal motility and EUS findings. EoEM patients experienced a longer duration of symptoms until diagnosis than muscle-predominant EoE patients (median 6 months vs 1 month, p = 0.026). Serum Immunoglobulin E (IgE) levels tended to be higher in EoEM patients, although this difference did not reach statistical significance (median 447.5 IU/mL vs 182.0 IU/mL, p = 0.083). EoEM patients presented with more common luminal compression (82.4% vs 33.3%, p = 0.045) and less frequent edema (41.2% vs 100%, p = 0.019) than muscle-predominant EoE patients. HREM revealed that the EoEM group exhibited significantly higher max distal contraction integral (DCI) values than the muscle-predominant EoE group (median 13462.2 mmHg·s·cm vs 2185.8 mmHg·s·cm, p = 0.027), showing a higher percentage of swallows with DCI > 8000 mmHg/(s·cm) (median 20% vs 0, p = 0.035) and incidence of JE (50% vs 0, p = 0.032). Figure 3 compares the endoscopy, barium esophagram, and manometry findings between the EoEM and muscle-predominant EoE patients.

Table 2.

Comparison between muscle-predominant EoE and total EoEM groups (n = 24).

Variable	Muscle-predominant EoE (n = 6)	EoEM (total; n = 18)	p Value
Baseline characteristics
Women, n (%)	3 (50.0)	4 (22.2)	0.307
Age, years, median [IQR]	55.0 [49.5, 62.8]	59.5 [51.3, 69.0]	0.463
Allergy history, n (%)	1 (16.7)	3 (18.8) (n = 16)	1
Dysphagia, n (%)	5 (83.3)	18 (100)	0.250
Chest pain, n (%)	2 (33.3)	7 (38.9)	1
Symptom duration at diagnosis, months, median [IQR]	1.0 [1.0, 1.8]	6.0 [1.3, 18.0] (n = 15)	0.026
High serum eosinophils, %, median [IQR]	2 (33.3)	5 (33.3) (n = 15)	1
Serum IgE, IU/mL, median [IQR]	182.0 [159.5, 211.5]	447.5 [193.5, 638.5] (n = 12)	0.083
Endoscopic findings		n = 17
Luminal compression, n (%)	2 (33.3)	14 (82.4)	0.045
Corkscrew-like appearance, n (%)	0 (0)	6 (35.3)	0.144
Edema, n (%)	6 (100)	7 (41.2)	0.019
EUS findings
Hypertrophy of the muscle layer, n (%)	6 (100)	17 (94.4)	1
Thickness of the muscle layer (maximum), mm: median [IQR]	4.9 [4.3, 5.9]	4.9 [4.1, 6.0] (n = 12)	0.541
Manometry findings		n = 14
Mean LES resting pressure, mmHg: median [IQR]	26.3 [19.4, 29.5]	25.0 [15.6, 32.2] (n = 8)	0.699
IRP > 15 mmHg, n (%)	2 (33.3)	4 (28.6)	1
Max DCI, mmHg/(s·cm): median [IQR]	2362.5 [1715.6, 3361.8]	13462.2 [6861.1, 19666.7] (n = 11)	0.027
Mean DCI, mmHg/(s·cm): median [IQR]	1718.4 [1217.4, 2117.9]	5120.4 [1606.3, 6499.5] (n = 7)	0.199
Percentage of swallows with DCI > 8000 mmHg/(s·cm), %, median [IQR]	0 [0, 0]	20.0 [0.0, 30.0] (n = 9)	0.035
Spastic contraction, n (%)	1 (16.7)	9 (64.3)	0.141
Achalasia, n (%)	0 (0)	1 (7.1)	1
EGJOO, n (%)	2 (33.3)	3 (21.4)	0.613
Jackhammer esophagus, n (%)	0 (0)	7 (50)	0.032
Nutcracker esophagus, n (%)	0 (0)	1 (7.1)	1

DCI, distal contraction integral; EGJOO, esophagogastric junction outflow obstruction; EoE, eosinophilic esophagitis; EoEM, eosinophilic esophageal myositis; EUS, endoscopic ultrasonography; IgE, immunoglobulin E; IQR, interquartile range; IRP, integrated relaxation pressure; LES, low esophageal sphincter.

Bold values indicate statistical significance.

Figure 3.

The endoscopy, barium esophagram, and manometry images of two (a) EoEM and (b) muscle-predominant EoE patients. The EoEM patient presented with serrated changes in the barium esophagram and extremely high maximum DCI (19,518.5 mmHg/[s·cm]).

Comparison of EUS findings and correlation between CT and EUS measurement

EUS findings showed no significant difference in maximum muscle thickness (median thickness: 4.9 mm between EoEM and muscle-predominant EoE groups (Figure 4). Figure 4(b) compares esophageal muscle thickness at 5 cm intervals along the esophagus and shows that the most significant muscle thickness was located in the distal esophagus, around 35–40 centimeters from the incisors. The mean maximum esophageal wall thickness was 8.2 mm (range 6.7–10.5 mm) on CT versus 7.1 mm (range 5.8–9.0 mm) on EUS. CT tended to slightly overestimate thickness compared to EUS, likely attributable to the absence of esophageal distension during CT imaging. Esophageal wall thickness measured by CT and muscle-layer thickness measured by EUS at 5 cm intervals in our seven EoEM patients demonstrated strong correlation (n = 35 measurements; Pearson r = 0.808, p < 0.001), suggesting that CT may serve as an effective tool for screening muscle-layer hypertrophy in EoEM (Figure 4(c)).

Figure 4.

(a) EUS images and (b) comparison of muscle layer thickness at various esophageal lengths between muscle-predominant EoE (black) and EoEM (gray), showing no significant differences. (c) Correlation between EUS muscle thickness and CT esophageal wall thickness measurements in seven EoEM patients (n = 35 measurements; Pearson r = 0.808, p < 0.001).

Comparison among muscle-involved patients grouped by number of mucosal eosinophils

Table 1 and Table S3 presents a comparison among three groups of muscle-involved patients: EoEM without mucosal eosinophils (n = 4), EoEM with 1–4 mucosal eosinophils/hpf (n = 3), and muscle-predominant EoE (n = 6). As expected by group definition, mucosal eosinophil counts differed significantly among the three groups (p = 0.005). No statistically significant differences were observed between the subgroups for other parameters; however, several showed trends toward significance. Ineffective esophageal motility was observed exclusively in the EoEM (no mucosal eosinophils) group (50.0%, p = 0.070). JE was most common in the EoEM (1–4 mucosal eosinophils/hpf) group (66.7%), followed by the EoEM (no mucosal eosinophils) group (25.0%), and was absent in muscle-predominant EoE (0%, p = 0.081). Luminal compression was present in all patients (100%) in the EoEM (no mucosal eosinophils) group, but only in 33.3% of patients in the other two groups (p = 0.084). Regarding disorders of peristalsis, EoEM patients without mucosal eosinophils appeared to have either ineffective esophageal motility or hypercontractile swallows, whereas esophageal motility disorders in EoEM patients with 1–4 eos/hpf were primarily characterized by hypercontractile swallows.

Comparison of treatment and prognosis among the three groups at the center

The results in Table 3 demonstrate notable differences in treatment response and prognosis among the three groups. Patients with mucosal-predominant EoE showed the highest responsiveness to PPIs at 71.4%. In comparison, this rate significantly dropped to 33.3% in the muscle-predominant EoE group and was absent in the EoEM group (p = 0.003). Steroid use was progressively more common across the groups, from 9.5% in mucosal-predominant EoE to 33.3% in muscle-predominant EoE and 57.1% in EoEM (p = 0.030), with all groups responding favorably to steroids (100% responsiveness). Among our seven EoEM patients, six (85.7%) underwent POEM, including three received oral steroids after POEM procedure, and achieved a 100% symptom relief rate; while one patient (14.3%) was treated with oral steroids alone due to patient preference and less severe symptoms, and achieved satisfactory symptom relief with steroid therapy without requiring endoscopic intervention. The duration of systemic steroid therapy was 6–12 weeks (median 8 weeks) with gradual tapering (typically reducing by 5 mg every 2 weeks). Follow-up data showed that symptom recurrence occurred in 1/4 (25%) EoEM patients after steroid discontinuation, requiring repeat courses of systemic steroids and ultimately requiring maintenance low-dose steroids (5 mg prednisone every other day) for sustained symptom control. No serious steroid-related side effects were reported. One patient developed mild hyperglycemia that resolved after tapering to a maintenance dose. Follow-up durations were similar across the groups. The mucosal-predominant EoE group showed the highest proportion of patients (81%) reporting no symptoms at the latest follow-up. In comparison, the EoEM group reported the highest rate of self-assessment relief (100%). Notably, the muscle-predominant EoE group showed the lowest rate of self-assessment relief (66.7%), probably because its PPI responsiveness was not high, and the priority and willingness to use steroids were not high (33.3%). Table 1 and Table S4 shows the detailed treatment and prognosis information of EoEM patients in our cohort.

Table 3.

Comparison of treatment and prognosis in our cohort (n = 34).

Variable	Mucosal-predominant EoE (n = 21)	Muscle-predominant EoE (n = 6)	EoEM (n = 7)	p Value
Treatment
PPI responsiveness, n (%)	15 (71.4)	2 (33.3)	0 (0)	0.003
POEM, n (%)	0 (0)	0 (0)	6 (85.7)	<0.001
Steroids use, n (%)	2 (9.5)	2 (33.3)	4 (57.1)	0.030
Steroid responsiveness (%)	100	100	100	1
Improvement of muscle layer hypertrophy after steroids, n (%)	—	1 (50) (n = 2)	2 (50) (n = 4)	1
Time of follow-up, months, median [IQR]	31.0 [17.0, 48.0]	50.5 [44.5, 60.3]	47.0 [24.0, 53.5]	0.205
Symptoms on the latest follow-up
Dysphagia, n (%)	2 (9.5)	2 (33.3)	2 (28.6)	0.280
Heartburn, n (%)	4 (19.0)	3 (50.0)	0 (0)	0.081
Chest pain, n (%)	3 (14.3)	2 (33.3)	0 (0)	0.238
Unintentional weight loss, n (%)	0 (0)	0 (0)	0 (0)	1
Eckardt score, median [IQR]	0.0 [0.0, 0.0]	0.5 [0.0, 4.8]	0.0 [0.0, 0.5]	0.268
Symptom frequency				0.266
Never, n (%)	17 (81.0)	3 (50.0)	5 (71.4)
Weekly, n (%)	2 (9.5)	1 (16.7)	2 (28.6)
Daily, n (%)	2 (9.5)	1 (16.7)	0 (0)
Each meal, n (%)	0 (0)	1 (16.7)	0 (0)
Self-assessment relief, n (%)	17 (81.0)	4 (66.7)	7 (100)	0.280

EoE, eosinophilic esophagitis; EoEM, eosinophilic esophageal myositis; IQR, interquartile range; POEM, Per-Oral Endoscopic Myotomy; PPI, proton pump inhibitor.

Bold values indicate statistical significance.

Discussion

The study provides critical insights into EoEM and its differentiation from mucosal-predominant and muscle-predominant subtypes of EoE. To our knowledge, this represents the first comparative study systematically differentiating EoEM from muscle-predominant EoE, addressing a significant gap in the literature where these two entities have often been conflated or inadequately distinguished.^8
–10,14 The findings reveal distinct clinical, endoscopic, pathological, and motility characteristics of EoEM. EoEM is marked by more severe dysphagia, food impaction, and weight loss, with symptoms persisting longer before diagnosis. Endoscopically, EoEM exhibits structural changes, such as a corkscrew-like appearance and luminal compression, but lacks the typical mucosal inflammation seen in EoE. On manometry, EoEM often displays hypercontractile patterns, such as JE, with a higher maximum DCI. Advanced diagnostic techniques such as EUS-FNB and POEM-b are critical for accurate diagnosis, with POEM providing rapid dysphagia relief. Oral steroids show greater efficacy in EoEM than PPIs.

Around 50% of EoE patients presented with dysphagia in the literature.³² While in our cohort, the symptom correlated with muscle involvement, with 83.3% in muscle-predominat EoE and 100% in EoEM patients, compared with 38.1% in mucosal-predominant EoE patients. Our larger cohort (7 institutional cases + 11 literature cases) provides more robust evidence for this characteristic feature of EoEM than prior small case series.^{9,10,14
–21} It aligns with previous studies finding thickness of the distal esophagus in EUS correlated with EoE symptoms.³³ Typical endoscopic features of EoE, including edema, white exudates, and longitudinal furrows, reflect mucosal inflammation.²⁷ In contrast, the EoEM group only exhibited mild edema without exudates or furrows, consistent with its distinct eosinophil infiltration patterns. Fixed rings, commonly found in mucosal-predominant EoE, were observed in another “corkscrew-like” form in EoEM, with the so-called “corrugated esophagus” challenging endoscope passage and leading to strictures, probably relating to esophageal spastic contraction.²⁷ Fixed rings in typical EoE are considered an endoscopic feature of esophageal remodeling resulting from lamina propria fibrosis.³⁴ However, no significant fibrosis was linked to these rings in EoEM, as the I-SEE for fibrostenotic features was not high. Instead, the severity of the rings in EoEM correlated more with motility dysfunction, particularly spastic contractions (incidence rate 64.3%), which also contributed to the corkscrew-like appearance. Although present in about 35% of EoEM cases, this corkscrew-like feature provides an important diagnostic clue, which could also be verified as serrated changes in the barium esophagram (Figure 3(a)). Luminal compression, detected with adequate air supply, was another distinguishing feature of EoEM.

EoEM remains under-recognized, as endoscopic and mucosal biopsy alone are insufficient for diagnosis. Utilizing HREM revealed unique hypercontractile patterns in EoEM, distinguishing it from muscle-predominant EoE. A significant proportion of EoEM patients (50%) presented with JE and higher DCI values, reflecting that the pathophysiology may overlap between EoEM and hypercontractile esophageal disorders. This explains the distinctive symptoms such as dysphagia, food impaction, and unintended weight loss in EoEM patients.

Elevated serum IgE may assist in diagnosis as a valuable marker, which needs further research to confirm. Notably, this study represents the first attempt to correlate serum IgE levels with EoEM diagnosis, identifying a potential non-invasive biomarker that could facilitate earlier recognition of this rare entity. It also suggests an immune-related pathogenesis of EoEM. Emerging evidence indicates that damage to the myenteric plexus may play a critical role in the pathogenesis of EoEM. Eosinophils primarily infiltrate the perivascular and perimysial regions of the muscularis propria.^{9,10,13,15,16} Autopsy findings have shown eosinophilic infiltration in the myenteric and endomysial tissues surrounding the myenteric plexus.²⁰ Damage to myenteric neurons can lead to achalasia or hypercontractility of the esophagus.³⁵ Eosinophils secrete cytokines and chemokines that alter neural function, induce tissue remodeling through pro-fibrotic factors, and directly damage neurons and muscles.⁸ Unlike muscle-predominant EoE, where eosinophils infiltrate surface-to-deep, EoEM shows localized eosinophilic infiltration within the muscular layer, suggesting distinct immune mechanisms. Further research into eosinophilic infiltration and its effects on the myenteric plexus could provide crucial insights into the pathogenesis of EoEM.

For diagnosis, muscle-layer biopsies obtained via EUS-FNB or POEM-b remain the gold standard for confirming eosinophilic infiltration in the muscularis propria. This study is the first to compare EUS measurements for muscle thickness at 5 cm intervals in EoEM and muscle-predominant EoE, establishing a standardized approach for objective assessment of muscle involvement that may guide clinical decision-making. POEM-b, though effective, requires myotomy and involves surgical trauma, which makes EUS-FNB a preferable, less invasive alternative. However, because EUS-FNB provides limited tissue samples, multiple-point biopsies under ultrasound guidance and ROSE are recommended to ensure sufficient sampling of the muscularis propria. Performing a muscle-layer biopsy before POEM is advisable for patients with a hypercontractile esophagus, as it may reveal underlying EoEM.³⁶ Our findings suggested a muscle-layer biopsy should be considered in patients presenting with frequent dysphagia, especially lasting long periods, endoscopic and manometry features for motility disorders, high serum IgE levels, and no evidence of mucosal eosinophilia.

PPI-responsive esophageal eosinophilia is now considered part of the EoE disease spectrum rather than a distinct entity, as supported by recent guidelines and consensus statements from the American College of Gastroenterology (ACG), American Gastroenterological Association (AGA), and British Society of Gastroenterology (BSG).^1
–3 Our comparison focuses on the phenotypic differences between mucosal-predominant and muscle-predominant eosinophilic esophageal disorders, recognizing that PPI response represents a treatment characteristic rather than a diagnostic criterion for disease classification. Both EoEM and muscle-predominant EoE exhibit resistance to PPIs but show positive responses to oral steroids. Oral steroids are preferred over topical corticosteroids in EoEM probably due to better penetration into the muscle layer. Further research is needed to evaluate the effectiveness of topical steroids for treating muscle-layer eosinophilia. POEM is also beneficial for relieving dysphagia in EoEM. This study provides a comprehensive analysis of treatment outcomes comparing PPI, steroids, and POEM in EoEM. Additionally, muscle-predominant EoE in our cohort had relatively low relief rates, often due to the unawareness of switching to oral steroids. Thus, EUS or CT assessment of muscle layer thickness may be recommended in EoE patients with frequent dysphagia and food impaction to help identify muscle involvement, which may respond better to steroids than PPIs. Our findings suggest that CT may serve as a complementary screening tool for identifying muscle layer thickening in EoE patients, thereby informing decisions on muscle biopsy and steroid therapy. Notably, this study is the first to propose CT as a screening alternative to EUS for initial evaluation of suspected EoEM. Future prospective studies should establish optimal cutoff values for CT-measured wall thickness and evaluate whether this assessment can effectively guide biopsy decisions and predict muscle-predominant disease that may respond more favorably to steroids than to PPIs. These findings carry significant clinical relevance for gastroenterologists managing patients with dysphagia and suspected eosinophilic esophageal disorders.

Several limitations should be acknowledged. First, the sample size is small, particularly for the EoEM group (n = 7), which limits the statistical power and generalizability of our findings. This reflects the rarity of EoEM but necessitates cautious interpretation of the results. Second, the retrospective study design introduces inherent limitations, including potential selection bias from referral patterns and incomplete data collection. Third, in cases where steroids were administered following POEM, clinical improvement could not be solely attributed to medication, representing a confounding factor. Fourth, the single-center nature of our institutional cohort may limit external validity. Finally, the follow-up duration, while reasonable, may not capture long-term outcomes or recurrence rates adequately. Given these limitations, our findings should be considered hypothesis-generating rather than definitive, and prospective multicenter studies with larger patient populations are needed to validate these findings.

Conclusion

In conclusion, EoEM is a rare disease within the EoE disease spectrum, characterized by eosinophilic infiltration primarily in the esophageal muscularis propria, without significant epithelial involvement. It presents with severe dysphagia, food impaction, and weight loss, often accompanied by esophageal motility disorders like JE. Endoscopic findings include corkscrew-like appearance and luminal compression. Diagnosis requires advanced techniques (EUS-FNB or POEM-b) for muscle-layer biopsies, while CT imaging may serve as a useful screening tool. EoEM may respond well to oral steroids and may benefit from POEM for symptomatic relief in severe cases. Future research should focus on pathophysiological mechanisms, diagnostic standardization, non-invasive biomarkers, personalized treatment strategies, and long-term outcomes to optimize patient care.

Supplemental Material

sj-docx-1-tag-10.1177_17562848261449690 – Supplemental material for Eosinophilic esophageal myositis: a comparative study of distinguishing features, diagnostic challenges, and therapeutic insights

Supplemental material, sj-docx-1-tag-10.1177_17562848261449690 for Eosinophilic esophageal myositis: a comparative study of distinguishing features, diagnostic challenges, and therapeutic insights by Rui Wu, Yunyun Zhang, Wei Zhao, Huaqing Zhu, Hongjiao Wu, Xinyi Lu, Wei Zhang, Xiaoping Zou and Nina Zhang in Therapeutic Advances in Gastroenterology

Supplemental Material

sj-docx-2-tag-10.1177_17562848261449690 – Supplemental material for Eosinophilic esophageal myositis: a comparative study of distinguishing features, diagnostic challenges, and therapeutic insights

Supplemental material, sj-docx-2-tag-10.1177_17562848261449690 for Eosinophilic esophageal myositis: a comparative study of distinguishing features, diagnostic challenges, and therapeutic insights by Rui Wu, Yunyun Zhang, Wei Zhao, Huaqing Zhu, Hongjiao Wu, Xinyi Lu, Wei Zhang, Xiaoping Zou and Nina Zhang in Therapeutic Advances in Gastroenterology

Supplemental Material

sj-jpg-4-tag-10.1177_17562848261449690 – Supplemental material for Eosinophilic esophageal myositis: a comparative study of distinguishing features, diagnostic challenges, and therapeutic insights

Supplemental material, sj-jpg-4-tag-10.1177_17562848261449690 for Eosinophilic esophageal myositis: a comparative study of distinguishing features, diagnostic challenges, and therapeutic insights by Rui Wu, Yunyun Zhang, Wei Zhao, Huaqing Zhu, Hongjiao Wu, Xinyi Lu, Wei Zhang, Xiaoping Zou and Nina Zhang in Therapeutic Advances in Gastroenterology

Supplemental Material

sj-xlsx-3-tag-10.1177_17562848261449690 – Supplemental material for Eosinophilic esophageal myositis: a comparative study of distinguishing features, diagnostic challenges, and therapeutic insights

Supplemental material, sj-xlsx-3-tag-10.1177_17562848261449690 for Eosinophilic esophageal myositis: a comparative study of distinguishing features, diagnostic challenges, and therapeutic insights by Rui Wu, Yunyun Zhang, Wei Zhao, Huaqing Zhu, Hongjiao Wu, Xinyi Lu, Wei Zhang, Xiaoping Zou and Nina Zhang in Therapeutic Advances in Gastroenterology

Footnotes

Acknowledgements

We thank to Dr. Yang Tian, Dr. Guo Huimin, Dr. Shi Liangliang, and Dr. Xie Ying for supporting data management.

Declarations

ORCID iDs

Rui Wu

Xiaoping Zou

Supplemental material

Supplemental material for this article is available online.

References

Dellon

Muir

Katzka

, et al. ACG Clinical guideline: diagnosis and management of eosinophilic esophagitis. Am J Gastroenterol 2025; 120(1): 31–59.

Hirano

Chan

Rank

, et al. AGA Institute and the Joint Task Force on Allergy-Immunology Practice Parameters clinical guidelines for the management of eosinophilic esophagitis. Gastroenterology 2020; 158(6): 1776–1786.

Dhar

Haboubi

Attwood

, et al. British Society of Gastroenterology (BSG) and British Society of Paediatric Gastroenterology, Hepatology and Nutrition (BSPGHAN) joint consensus guidelines on the diagnosis and management of eosinophilic oesophagitis in children and adults. Gut 2022; 71(8): 1459–1487.

Lucendo

Arias

De Rezende

, et al. Subepithelial collagen deposition, profibrogenic cytokine gene expression, and changes after prolonged fluticasone propionate treatment in adult eosinophilic esophagitis: a prospective study. J Allergy Clin Immunol 2011; 128(5): 1037–1046.

Yang

Jung

KW.

Role of endoscopy in eosinophilic esophagitis. Clin Endosc 2025; 58(1): 1–9.

Fox

Nurko

Teitelbaum

, et al. High-resolution EUS in children with eosinophilic “allergic” esophagitis. Gastrointest Endosc 2003; 57(1): 30–36.

Reddy

Ketchem

Dougherty

, et al. Association between eosinophilic esophagitis and esophageal dysmotility: a systematic review and meta-analysis. Neurogastroenterol Motil 2023; 35(2): e14475.

Spechler

Konda

Souza

Can eosinophilic esophagitis cause achalasia and other esophageal motility disorders?

Am J Gastroenterol 2018; 113(11): 1594–1599.

Stevoff

Rao

Parsons

, et al. EUS and histopathologic correlates in eosinophilic esophagitis. Gastrointest Endosc 2001; 54(3): 373–377.

10.

Landres

Kuster

Strum

WB.

Eosinophilic esophagitis in a patient with vigorous achalasia. Gastroenterology 1978; 74(6): 1298–1301.

11.

Ruan

Liu

, et al. Eosinophilic gastroenteritis: pathogenesis, diagnosis, and treatment. Chin Med J (Engl) 2023; 136(8): 899–909.

12.

Prasad

Hirano

Eosinophilic esophagitis: are we just scratching the surface?

Gastroenterol Hepatol (N Y) 2013; 9(9): 611–612.

13.

Sato

Takeuchi

Takahashi

, et al. Nutcracker and Jackhammer esophagus treatment: a three-case survey, including two novel cases of eosinophilic infiltration into the muscularis propria. Endoscopy 2015; 47(9): 855–857.

14.

Benias

Matin

Ascunce

, et al. Esophageal obstruction as a result of isolated eosinophilic gastroenteritis. Gastroenterol Hepatol (N Y) 2013; 9(9): 607–610.

15.

Sato

Takeuchi

Takahashi

Eosinophilic infiltration of the muscularis propria in a patient with Jackhammer esophagus treated with per-oral endoscopic myotomy. Clin Gastroenterol Hepatol 2015; 13(4): e33–e34.

16.

Igarashi

Irisawa

Shibukawa

, et al. Eosinophilic esophageal myositis diagnosed by endoscopic ultrasound-guided fine-needle aspiration biopsy: a case report. Clin J Gastroenterol 2016; 9(5): 285–288.

17.

Sato

Nakajima

Hasegawa

, et al. Immunohistochemical differentiation of eosinophilic esophageal myositis from eosinophilic esophagitis. J Gastroenterol Hepatol 2017; 32(1): 106–113.

18.

Sato

Nakajima

Takahashi

, et al. Proposed criteria to differentiate heterogeneous eosinophilic gastrointestinal disorders of the esophagus, including eosinophilic esophageal myositis. World J Gastroenterol 2017; 23(13): 2414–2423.

19.

Tang

Xiong

, et al. Eosinophilic esophageal myositis a plausible cause of histological changes of primary Jackhammer esophagus: a case report. Am J Gastroenterol 2018; 113(1): 150–152.

20.

Song

Kimura

Ogiyama

, et al. Eosinophilia in the muscle layer of the esophagus and the urinary bladder in a Multiple System Atrophy patient with dysphagia and dysuria. Pathol Int 2018; 68(6): 382–387.

21.

Yang

Xiang

Liu

A rare cause of dysphagia: diagnosis and treatment. Gastroenterology 2022; 163(6): e28–e30.

22.

Boeckxstaens

Zaninotto

Richter

JE.

Achalasia. Lancet 2014; 383(9911): 83–93.

23.

von Elm

Altman

Egger

, et al.; STROBE Initiative. The Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) statement: guidelines for reporting observational studies. Ann Intern Med 2007; 147(8): 573–577. Erratum in: Ann Intern Med 2008; 148(2):168.

24.

Furuta

Liacouras

Collins

, et al. Eosinophilic esophagitis in children and adults: a systematic review and consensus recommendations for diagnosis and treatment. Gastroenterology 2007; 133(4): 1342–1363.

25.

Gonsalves

Aceves

SS.

Diagnosis and treatment of eosinophilic esophagitis. J Allergy Clin Immunol 2020; 145(1): 1–7.

26.

Dellon

Gonsalves

Hirano

, et al. ACG clinical guideline: evidenced based approach to the diagnosis and management of esophageal eosinophilia and eosinophilic esophagitis (EoE). Am J Gastroenterol 2013; 108(5): 679–693.

27.

Hirano

Moy

Heckman

, et al. Endoscopic assessment of the oesophageal features of eosinophilic oesophagitis: validation of a novel classification and grading system. Gut 2013; 62(4): 489–495.

28.

Fox

Sweis

Yadlapati

, et al. Chicago classification version 4.0© technical review: update on standard high-resolution manometry protocol for the assessment of esophageal motility. Neurogastroenterol Motil 2021; 33(4): e14120.

29.

Yadlapati

Kahrilas

Fox

, et al. Esophageal motility disorders on high-resolution manometry: Chicago classification version 4.0^© [published correction appears in Neurogastroenterol Motil 2024; 36(2): e14179.]. Neurogastroenterol Motil 2021; 33(1): e14058.

30.

Ferrelli

Machado

Ogata

SK.

Oesophageal wall thickness assessment at endoscopic ultrasound in children and adolescents with eosinophilic oesophagitis: a case-control study. J Ultrasound 2023; 26(4): 785–791.

31.

Dellon

Khoury

Muir

, et al. A clinical severity index for eosinophilic esophagitis: development, consensus, and future directions. Gastroenterology 2022; 163(1): 59–76.

32.

Chehade

Jones

Pesek

, et al. Phenotypic characterization of eosinophilic esophagitis in a large multicenter patient population from the consortium for food allergy research. J Allergy Clin Immunol Pract 2018; 6(5): 1534.e5–1544.e5.

33.

Wong

Tippett

Zobel

, et al. Distal esophageal wall thickness correlates with dysphagia in adult patients with eosinophilic esophagitis. Esophagus 2022; 19(4): 554–559.

34.

Aceves

SS.

Tissue remodeling in patients with eosinophilic esophagitis: what lies beneath the surface?. J Allergy Clin Immunol 2011; 128(5): 1047–1049.

35.

de Bortoli

Gyawali

Roman

, et al. Hypercontractile esophagus from pathophysiology to management: proceedings of the Pisa symposium. Am J Gastroenterol 2021; 116(2): 263–273.

36.

Kawami

Hoshikawa

Momma

, et al. Clinical characteristics of patients with eosinophilic esophagitis and eosinophilic esophageal myositis based on esophageal motility. Esophagus 2025; 22(1): 124–130.

Supplementary Material

Please find the following supplemental material available below.

For Open Access articles published under a Creative Commons License, all supplemental material carries the same license as the article it is associated with.

For non-Open Access articles published, all supplemental material carries a non-exclusive license, and permission requests for re-use of supplemental material or any part of supplemental material shall be sent directly to the copyright owner as specified in the copyright notice associated with the article.

0.00 MB

0.03 MB

0.04 MB

0.94 MB

0.01 MB