Markedly Effective Steroid Treatment of Three Patients with Allergy-related Jackhammer Esophagus

Funaki, Yasushi; Ogasawara, Nagahisa; Kawamura, Yasuhiko; Yoshimine, Takashi; Tamura, Yasuhiro; Izawa, Shinya; Tsuyuki, Takuji; Ebi, Masahide; Sasaki, Makoto; Kasugai, Kunio

doi:10.2169/internalmedicine.3865-19

Cited by 8 publications

(18 citation statements)

References 28 publications

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“…In many of these cases, eosinophilic myositis is detected by muscle layer biopsy during POEM [ 6 , 7 , 19 ]. Furthermore, from the therapeutic perspective, treatment with steroids has been successfully utilized in these patients [ 18 , 20 ]. In the present study, we found eosinophilic infiltration (i.e., > 15 eosinophils/high power field) in 12 of the 58 patients (21%) with JE who underwent esophageal mucosal biopsies.…”

Section: Discussionmentioning

confidence: 99%

Clinical presentation and therapeutic outcome of patients with jackhammer esophagus—a multicenter cohort study in Japan

et al. 2022

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Background Jackhammer esophagus (JE) is a hypercontractile esophageal motility disorder diagnosed using high-resolution manometry (HRM). We sought to determine the clinical presentation and therapeutic data of patients with JE in Japan. Methods The study included patients with JE, diagnosed through HRM performed for suspicious esophageal motility disorders. Demographics, esophagogastroduodenoscopy, radiology, and therapy data were collected from patient charts. Results Among the 4,412 HRM tests performed, 89 patients (61.6 ± 13.4 years; 64 males, 25 females) were diagnosed with JE (2.0%). Dysphagia was the most frequent symptom (80%), followed by chest pain (40%) and heartburn (25%). Esophagogastroduodenoscopy showed abnormal findings in 32% of patients: corkscrew/rosary beads appearance in 26%, narrowing in 11%. Eosinophilic infiltration (> 15 eosinophils/high power field) was diagnosed in 21%. Esophagography showed abnormal findings in 9% of the patients. For the initial therapy, 47 patients received medical treatment followed by peroral endoscopic myotomy (21 patients) and laparoscopic myotomy (two patients). Thirteen patients did not receive any treatment and 10 of those (77%) reported spontaneous resolution of symptoms. Patients who required invasive treatment experienced severe disability in their quality of life and greater maximal distal contractile integral than those who did not. Conclusions HRM showed that the prevalence of JE was very low (2%). Esophagogastroduodenoscopy revealed some characteristic features of JE in patients. Some patients showed improvement of symptoms without invasive treatments. Follow-up with/without medical treatment should be considered before performing invasive treatment in patients whose distal contractile integral is relatively low and the quality of life is not impaired.

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Section: Discussionmentioning

confidence: 99%

Clinical presentation and therapeutic outcome of patients with jackhammer esophagus—a multicenter cohort study in Japan

et al. 2022

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“…A number of studies in this group report normalization of IEM in response to pharmacological therapy in a proportion of patients with EoE, suggesting that reducing inflammation can improve or normalize motility abnormalities. 23,25,30,46,58,61,66 Eosinophils directly affect esophageal epithelial cells, with basal zone hyperplasia appearing to occur more markedly in EoE than in other types of esophagitis including GORD. 76 Furthermore, these changes can persist even when mucosal eosinophil count is reduced, suggesting ongoing disease processes.…”

Section: Discussionmentioning

confidence: 99%

“…Indeed, concomitant hypertrophy seen on EUS or CT has also been described in cases of muscularis propria eosinophilia. 22,25,[94][95][96][97] Degranulation of eosinophils releases substances that can influence smooth muscle function, particularly transforming growth factor beta 1 secreted by both eosinophils and mast cells, which has been demonstrated to cause smooth muscle contractions in vitro. 98 In addition, neuroactive substances including major basic protein, released as a result of degranulation of eosinophils, may influence neural function and thus disrupt normal motility patterns.…”

Section: Discussionmentioning

confidence: 99%

“…3). 23,25,30,46,58,61 All cases where motility normalization was reported were in response to pharmacological treatment targeted at eosinophilia, rather than endoscopic or surgical intervention. Although not a finding of this review, the authors have examples of normalization of motility in response to a combination of corticosteroid and PD (Fig.…”

Section: Overviewmentioning

confidence: 99%

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Assessment of Esophageal Motility in Patients With Eosinophilic Esophagitis

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Fox

et al. 2022

Journal of Clinical Gastroenterology

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Eosinophilic esophagitis (EoE) is a chronic, immunemediated condition causing esophageal symptoms, particularly dysphagia. Despite the important progress in the treatment of EoE, a significant proportion of patients continue to report symptoms that negatively impact quality of life. Esophageal manometry is used to assess motility and function, but is not routinely used in EoE. We aimed to systematically review and describe current literature evaluating esophageal manometry in EoE. Forty-eight studies meeting the criteria were identified, describing 802 patients. Using standard water swallow protocols, the proportion of abnormalities detected was not dissimilar to other populations, apart from disorders of esophago-gastric outflow, which were found in 5%. Twelve studies described pretreatment and posttreatment manometry, with motility normalization after pharmacological therapy reported in 20%. Early, brief panesophageal pressurization was described in a number of studies and was more prevalent in the few studies utilizing additional provocation testing. Reports in the literature regarding temporal relationships between manometric findings and symptoms are variable. Esophageal manometry may be capable of detecting clinically relevant changes to esophageal function in EoE. Possible mechanisms are altered neuromuscular function because of secretory products of EoE and/or fibroinflammatory processes, manifesting as pressurization because of altered esophageal compliance. Some changes may be reversible with therapy. Drawing strong conclusions from the literature is difficult, with bias toward case reports and retrospective observation. Adaptations to assessment protocols to include provocation testing may provide more robust evaluation and detect clinically relevant, subtle changes in esophageal function, earlier within the patient pathway.

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“…In the assessment of this process and monitoring of treatment related to the duration of steroid therapy, it seems crucial to use EUS and taking biopsies from deeper layers in a targeted manner [ 23 ]. Japanese researchers have also shown that esophageal motility disorders such as diffuse esophageal spasm, corkscrew esophagus and even esophageal achalasia can be related to EoE, and that an effective treatment can be managed according to EoE treatment standards, often without the need for surgical intervention [ 24 ]. These diseases although typical for adults can occur at any age, and EUS is well suited for diagnostic tools in addition to high-resolution manometry.…”

Section: Endoscopic Ultrasonography In Children With Eosinophilic Esophagitismentioning

confidence: 99%

Endoscopic Ultrasonography in Children with Eosinophilic Esophagitis—A Review

et al. 2022

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Endoscopic ultrasonography (EUS) is a diagnostic endoscopy of the upper gastrointestinal tract, during which ultrasound of nearby organs is also performed. It is also possible to perform a fine needle aspiration biopsy. Currently, EUS is performed more frequently in adults. Despite some limitations, this diagnostic method is also more and more often performed in pediatric patients. Eosinophilic esophagitis (EoE) is a chronic inflammatory disease of the esophagus, which also occurs in children, and leads to irreversible fibrosis of the esophagus wall, if left untreated. Traditional methods of diagnosing and monitoring EoE treatment have significant limitations, and the use of EUS and total esophageal wall thickness (TWT) assessment may bring measurable benefits. Several studies have shown an increased thickening of TWT in EoE in children compared to pediatric patients with gastroesophageal reflux disease, and a decrease in TWT in adults who responded to EoE treatment. These results suggest that EUS and TWT measurement may become an important test in diagnostics, monitoring the effectiveness of therapy, assessing disease progression, and in individualizing the method and duration of EoE treatment also in children.

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Markedly Effective Steroid Treatment of Three Patients with Allergy-related Jackhammer Esophagus

Cited by 8 publications

References 28 publications

Clinical presentation and therapeutic outcome of patients with jackhammer esophagus—a multicenter cohort study in Japan

Clinical presentation and therapeutic outcome of patients with jackhammer esophagus—a multicenter cohort study in Japan

Assessment of Esophageal Motility in Patients With Eosinophilic Esophagitis

Endoscopic Ultrasonography in Children with Eosinophilic Esophagitis—A Review

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