Assessment of esophageal involvement in systemic sclerosis and morphea (localized scleroderma) by clinical, endoscopic, manometric and pH metric features: a prospective comparative hospital based study

Abstract

BackgroundSystemic sclerosis (SSc) is a generalized disorder of unknown etiology affecting the connective tissue of the body. It affects the skin and various internal organs. Gastrointestinal tract involvement is seen in almost 90% of the patients. Esophagus is the most frequently affected part of the gastrointestinal tract. Esophageal motility disturbance classically manifests as a reduced lower esophageal sphincter pressure (LESP) and loss of distal esophageal body peristalsis. Consequently, SSc patients may be complicated by erosive esophagitis and eventually by Barrett’s esophagus and esophageal adenocarcinoma. Morphea, also known as localized scleroderma, is characterized by predominant skin involvement, with occasional involvement of subjacent muscles and usually sparing the internal organs. The involvement of esophagus in morphea has been studied very scarcely. The proposed study will investigate the esophageal involvement in the two forms of scleroderma (systemic and localized), compare the same and address any need of upper gastrointestinal evaluation in morphea (localized scleroderma) patients.Methods56 and 31 newly and already diagnosed cases of SSc and morphea respectively were taken up for the study. All the patients were inquired about the dyspeptic symptoms (heartburn and/or acid regurgitation and/or dysphagia). Upper gastrointestinal endoscopy, esophageal manometry and 24-hour pH monitoring were done in 52, 47 and 41 patients of SSc; and 28, 25 and 20 patients of morphea respectively.ResultsEsophageal symptoms were present in 39 cases (69.6%) of SSc which were mild in 22 (39.3%), moderate in 14 (25%), severe in three (5.3%); while only four cases (7.1%) of morphea had esophageal symptoms all of which were mild in severity. Reflux esophagitis was seen in 17 cases (32.7%) of SSc and only two cases (7.14%) of morphea. Manometric abnormalities were seen in 32 cases (68.1%) of SSc and none in morphea. Ambulatory 24-hour esophageal pH monitoring documented abnormal reflux in 33 cases (80.5%) of SSc and no such abnormality in morphea.ConclusionWhile the esophageal involvement is frequent in SSc, no such motility disorder is seen in morphea. Meticulous upper gastrointestinal tract evaluation is justified only in SSc and not in morphea.

The efficacy of early immunosuppression in a morphea patient complicated with pulmonary interstitial fibrosis

“…Its incidence rate is rare counting from 7% to 9% in total morphea patients. (Bielsa Marsol, ) Previous seldom cases have been reported that generalized morphea could accompany with organ dysfunction (Arif, Masood, Singh, & Hassan, ).…”

Abnormal esophageal acid exposure on high‐dose proton pump inhibitor therapy is common in systemic sclerosis patients

“…evaluated reflux parameters via ambulatory 24‐hour pH testing in 41 SSc patients, and found that 80.5% had abnormal results, with a mean AET of 8.5% . However, the study lacked impedance‐pH metry, which is the gold standard for the assessment of GERD . Furthermore, all patients were studied off PPI therapy.…”

“…Weakly acid reflux events 24 However, the study lacked impedance-pH metry, which is the gold standard for the assessment of GERD. 24 Furthermore, all patients were studied off PPI therapy.…”

“…Weakly acid reflux events 24 However, the study lacked impedance-pH metry, which is the gold standard for the assessment of GERD. 24 Furthermore, all patients were studied off PPI therapy. Given that many SSc patients are on a PPI for their upper gastrointestinal (GI) symptoms, and some experts recommend all SSc patients be on PPI, the findings have limited generalizability.…”

Scleroderma Mimickers

“…Arthralgias and myalgias are common. Patients may develop dysphagia secondary to external compression (76). In the setting of normal CT scans, restrictive patterns have been observed on pulmonary function tests; attributed to external limitations of rib cage mobility (77).…”