Original Articles
 
Rheumatologic Disorders in Patients Undergoing Esophageal Manometry- Prevalence, Clinical Presentation, Endoscopy Findings and Esophageal Manometry Findings
 
Mayank Jain
Department of Gastroenterology, Arihant Hospital and Research Centre, Indore, India.


Corresponding Author:
Dr. Mayank Jain
Email: mayank4670@rediffmail.com

Abstract

Background: Upper gastrointestinal symptoms are commonly experienced in patients with rheumatologic disorders (RD). However, this important aspect is seldom given due importance and rarely investigated in clinical practice. We determined the prevalence of RD amongst patients undergoing high resolution esophageal manometry (HREM) and analysed the symptoms and HREM findings in patients with RD.
Methods: The present study is a retrospective analysis conducted using a prospectively maintained database of patients who underwent HREM between 2012 and 2022. The records were scrutinised to identify patients with RD. The study cohort consisted of patients with RD who were referred for HREM evaluation of upper gastrointestinal symptoms. We excluded patients with incomplete data and those who did not have rheumatologic disorders. The data collected for the study group included age, sex, symptoms, duration of rheumatologic disease, duration of upper gastrointestinal symptoms, endoscopy and HREM findings. The HREM files were reanalysed for all patients using Chicago classification (CC) version 4.0.
Results: A total of 994 HREM studies were conducted during the study period. The study cohort consisted of a total of 32 cases, resulting in a  prevalence of RD among patients undergoing HREM was 3.2%. The majority of the patients were females, and scleroderma was the commonest RD. Regurgitation was the commonest symptom, followed by heartburn. Majority of the cases (28/32, 87.5%) had reflux esophagitis on endoscopy. Ineffective esophageal motility (IEM) was the most common peristaltic disorder, followed by absent contractility (AC). Nearly two-fifths of cases had type 2 or 3 esophagogastric junction. Esophagogastric junction contractile integral (EGJ-CI) was <39.1 mmHg.cm in 26 cases (81.3%). 
Conclusion: Prevalence of RD in patients undergoing HREM is 3.2%. Low EGJ-CI was observed in four-fifths of the patients. IEM and AC are the most common peristaltic abnormalities.

Introduction

Upper gastrointestinal symptoms are commonly experienced in patients with rheumatologic disorders (RD). Patients with RD may experience poor appetite, nausea, vomiting, dysphagia, odynophagia, heartburn, dyspepsia and bloating. Medications alone do not account for these symptoms, suggesting that the underlying conditions predispose to the gastrointestinal symptoms1. Esophageal dysmotility and impaired swallowing can lead to increased risk of aspiration, pneumonia, malnutrition, disability and poor quality of life in patients with RD2. However, this important aspect is seldom given due importance and rarely investigated in clinical practice. The aim of the study was to determine the prevalence of RD among patients undergoing high resolution esophageal manometry (HREM) and to analyse the symptoms and HREM findings in patients with RD.

Methods

The present study is a retrospective analysis conducted using a prospectively maintained database of patients who underwent HREM between 2012 and 2022. The records were scrutinised to identify patients with RD. The study cohort consisted of patients with RD who were referred for HREM evaluation of upper gastrointestinal symptoms. We excluded patients with incomplete data and those who did not have rheumatologic disorders. The data collected for the study group included age, sex, symptoms, duration of rheumatologic disease, duration of upper gastrointestinal symptoms, endoscopy findings and HREM findings. Demographic details and symptoms were recorded in a proforma for all patients undergoing HREM at our centre. Upper gastrointestinal endoscopy reports were obtained from static images and available patient reports. Only 5 patients had both endoscopy and HREM at our centre. The HREM files were reanalysed for all patients using Chicago classification (CC) version 4.03. Esophagogastric junction (EGJ) morphology was classified as per the relationship between lower esophageal sphincter (LES) and crural diaphragm: type I when LES and crural diaphragm were superimposed, type II when separated less than 3 cm, and type III when separated by 3 cm or more4,5. Esophagogastric junction contractile integral (EGJ-CI), is a marker of EGJ barrier function. It was evaluated during a period of quiet rest over exactly 3 respiratory cycles, and divided by the duration of the respiratory cycles. EGJ-CI was considered low when less than 39.1 mmHg·cm6,7.
The collected data was analysed as median, range and percentages. The study was approved by Institutional Ethics committee via letter number AHRC/IEC/2021/16.

Results

A total of 994 HREM studies were conducted during the study period. Among these, 34 HREM were performed for patients with RD. Data was incomplete for 2 patients and hence, they were excluded from the analysis. The study cohort consisted of a total of 32 cases, resulting in a prevalence of RD among patients undergoing HREM of 3.2%.
As noted from table 1, majority of the patients were females (30, 93.8%) and scleroderma (24, 75%) was the commonest RD. Regurgitation (26, 81.3%) was the most common symptom, followed by heartburn (24, 75%). The majority of the cases (28/32, 87.5%) had reflux esophagitis on endoscopy. Ineffective esophageal motility (IEM) was the most common peristaltic disorder (17, 53.1%), followed by absent contractility (AC, 13, 40.1%). Nearly two-fifths of cases had type 2/3 esophagogastric junction. EGJ-CI was <39.1 mmHg.cm in 26 cases (81.3%). 
As noted from figure 1 and 2, achalasia cardia and esophagogastric junction outflow obstruction (EGJOO) were observed in one case each of systemic lupus erythematosus (SLE) and rheumatoid arthritis, respectively. In patients with scleroderma, IEM and AC were the most common peristaltic disorders.










Discussion

The prevalence of RD in patients undergoing HREM was 3.2%. Three-fourths of patients had reflux esophagitis and EGJ dysfunction was present in 81.3% cases. Ineffective esophageal motility and absent contractility were the most common peristaltic abnormalities.
In a study by Qureshi et al8, it was noted that out of 1,003 patients studied, 90 (9%) had RD (mean age: 55.3 ± 1.4 years, 73.3% females). Systemic sclerosis, rheumatoid arthritis and SLE were the most common RDs noted. The common upper gastrointestinal symptoms included heartburn, regurgitation, nausea, and dysphagia. Nearly 60% of patients with RD had esophageal motility disorders which included AC, IEM and EGJ obstructive disorders in 25 (27.7%), 18 (20%) and 11 (12.2%) cases, respectively8. In the present study, we noted that only 3.2% of cases who underwent HREM had RD. The age at presentation was younger (median 44 years) and nearly all were females (93.8%). Scleroderma was the most common RD followed by SLE. AC, IEM and EGJ obstructive disorders were noted in 40.6%, 53.1% and 6.2% cases, respectively. The differences in findings may be attributed to referral bias, ethnic variations, gender influences, differences in drug therapy, age differences in the studied groups, duration and severity of RD, as well as reflux. Dysphagia was less frequent in our cases, probably due to lesser severity of reflux and younger age at presentation.
Scleroderma causes smooth muscle atrophy and fibrosis of the distal two-thirds of the esophagus leading to dysphagia, heartburn, and regurgitation. Complications such as strictures and Barrett’s esophagus have also been described9. It is well documented that gastrointestinal abnormalities may be detected in patients even when they are asymptomatic10. Esophageal dysmotility in systemic sclerosis is linked to the severity of reflux disease, and the presence of reflux symptoms acts as a simple warning sign which should prompt an evaluation by manometry11. Use of lifestyle intervention and drug therapy has favourable outcomes in such patients12.
Very few cases of systemic lupus erythematosus and achalasia cardia have been described in the literature13,14. Swallowing disorders are more common in patients with rheumatoid arthritis and are associated with disease severity15. Temporomandibular joint involvement, Sicca syndrome, eosinophilic infiltration of esophagus, rheumatoid vasculitis, synovitis, impairment in esophagus distal peristalsis, and decrease in lower esophageal sphincter tone may be reasons for this complaint2,16,17,18,19. In Sjogren’s syndrome, patients may experience lack of salivation, esophageal dysmotility, myositis and damage to parasympathetic function. It has been noted that in patients with Sjogren’s syndrome, both upper and lower esophageal sphincter function is impaired, and esophageal motility is decreased20,21,22. In idiopathic inflammatory myositis, pharyngoesophageal manometry findings like low amplitude pharyngeal constrictor contraction, normal resting tone and relaxation of the upper esophageal sphincter, and diminished lower esophageal sphincter pressure have been described23.
The dysfunction of lower esophageal sphincter noted in RD is highlighted by low EGJ-CI in four-fifths of our patients. Low EGJ-CI provides evidence for impaired EGJ barrier function.
The present study is a single-centre experience and is limited by small sample size. Only five cases had endoscopy and HREM conducted at our centre. For others, we analysed the available static images or noted the findings mentioned in their endoscopy reports. This may have resulted in errors in assessing disease severity and could have affected the results. Barium swallow was not performed in our patients. Timed barium esophagogram could have helped us in determining esophageal emptying.
Despite these limitations, the present study highlights that many patients with RD in the Indian setting have esophageal dysmotility, and there is need to investigate these patients thoroughly for upper gastrointestinal symptoms.  

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