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Progressive systemic sclerosis: Radionuclide esophageal scintigraphy and manometry

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Abstract

Radionuclide esophageal scintigraphy (RES) and manometry were used for prospective evaluation of esophageal involvement and disease severity in 11 patients (nine women and two men; median time since diagnosis, 1 year) with progressive systemic sclerosis (PSS). Quantitation of RES included calculation of the percentage of emptying at 30 seconds, while manometry provided measurements of proximal, distal, and lower esophageal sphincter (LES) pressures. The findings of both RES and manometry were abnormal in all 11 patients. There was a high correlation between the percentage of emptying and either distal esophageal pressure (r = .86, P less than .01) or LES pressure (r = .79, P less than .01). No significant correlation was found between the percentage of emptying and proximal esophageal pressure (r = .28, P = .39). RES is a safe, simple procedure that is readily accepted by patients and can be used in place of manometry for the detection and staging of esophageal involvement in PSS.

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... Systemic sclerosis (SSc) is a connective tissue disease characterized by fibrosis in multiple organs, especially in the skin, lungs and gastrointestinal system [1,2]. The oesophagus is the most frequently involved internal organ [3][4][5], and atrophy and fibrosis of the smooth muscle are probably responsible for the impaired oesophageal motility that is common in this disease [2,5]. ...
... Diminished lower oesophageal sphincter pressure and associated gastro-oesophageal reflux (GER) occur frequently, leading to oesophagitis, strictures and ulcerations [4,6]. Early recognition of the oesophageal dysfunction is important because patients may be asymptomatic for a long time despite the presence of oesophageal motor abnormalities [1,3,4]. ...
... Manometry has been considered the gold-standard examination to detect oesophageal dysfunction in early stages of SSc, but it gives no information on morphology and has low patient acceptance [1][2][3]. Radionuclide oesophageal scintigraphy(RES)standsoutasafrequentlyusedalternative to manometry, since it is a safe, non-invasive, and sensitive method that allows quantitative assessment [1,2,[7][8][9][10]. ...
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The aim of this study was to compare oesophageal abnormalities observed in high-resolution CT with radionuclide transit in patients with systemic sclerosis. 76 patients with systemic sclerosis were evaluated by high-resolution CT and oesophageal transit scintigraphy. Residual activity > or =20% (in relation to peak activity) at 15 s after the beginning of the swallow of the labelled liquid (in supine position) was considered indicative of oesophageal dysfunction. Supra-aortic and infra-aortic oesophageal coronal diameters were measured in high-resolution CT. Oesophageal dilatation was deemed present when the diameters exceeded 10 mm. 19 patients (25%) had supra-aortic oesophageal dilatation and 48 patients (63.1%) had infra-aortic dilatation. The prevalence of radionuclide transit delay was 77.6%. All patients (19/19) with supra-aortic dilatation had oesophageal dysfunction, compared with 70.2% (40/57) of the patients with no supra-aortic dilatation (p = 0.004). Oesophageal dysfunction was present in 97.9% (47/48) of patients with infra-aortic dilatation, compared with 42.9% (12/28) in patients without it (p < 0.001). Receiver operating characteristic (ROC) curves have demonstrated that the supra-aortic and infra-aortic diameters had good discriminatory capacity for oesophageal dysfunction in systemic sclerosis (area under the curve, 95% confidence interval: 0.80, 0.70-0.89 and 0.92, 0.86-0.98, respectively). There is a clinically significant association between oesophageal dysmotility and high-resolution CT findings of oesophageal coronal dilatation. The evaluation of infra-aortic oesophageal coronal diameter can provide additional useful information about the functional and anatomic conditions of the oesophagus in systemic sclerosis.
... The usefulness of an esophageal transit study has been documented in patients with achalasia, esophageal spasm, systemic sclerosis and other motility disorders. To evaluate functional abnormality of the esophagus, several methods have been clinically used, which include a cinematic display of dynamic images and analysis of time-activity curves by setting regions of interest (ROIs) on the esophagus [1,2]. As an method of functional imaging, a condensed image has been used to summarize transit using space-time axes [3][4][5][6]. ...
... In digestive complications, gastroesophageal reflux disease (GERD) symptoms and dysmotility of esophagus are more commonly observed, in comparison to gastric and intestinal complications. Some studies have utilized a radionuclide esophageal transit study in patients with SSc [1,6,[9][10][11][12][13]. A reliable but simple non-invasive method for quantifying esophageal transit would contribute to early diagnosis, treatment and follow-up of esophageal involvement [6,12,13]. ...
Article
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Esophageal complication is common in systemic sclerosis (SSc), but scintigraphic transit patterns based on each subtype have not been understood well. The aim of this study was to develop a new algorithm for integrating a dynamic esophageal transit study and to apply the method to patients with SSc. A total of 40 patients suspected of having SSc were examined by a dynamic esophageal transit study. The subtypes included 32 with definite SSc (15 limited cutaneous type and 17 diffuse cutaneous type) and 8 with probable SSc. The serial esophageal images were shifted and summed to a functional image (sliding sum image) and compared to a conventional condensed image analysis. Esophageal retention fraction at 90 s (R (90)) and half-time (T (1/2)) of transit were also measured. The four patterns of the sliding sum image and condensed image agreed in all patients. Abnormal retention patterns were observed in none of the 8 (0%) patients with the probable SSc and in 15 of 32 (47%) patients with definite SSc (p = 0.014). The severity of scleroderma assessed by modified Rodnan skin thickness score correlated with that of esophageal retention R (90) (p = 0.04). The sliding sum image is a simple and effective method for integrating esophageal transit. Patients with definite SSc and severe scleroderma had significantly higher retention patterns, while probable SSc patients showed no esophageal dysmotility.
... Simultaneous 12-hr pH monitoring and manometry has shown a correlation between acid exposure and the degree of distal esophageal dysmotility. This study (6), along with a scintigraphic report showing a high direct correlation of esophageal transit to distal esophageal peristalsis in PSS patients (7) provide indirect evidence that decreased clearance of acid reflux events may be the primary abnormality responsible for esophagitis in patients with scleroderma. ...
... The purpose of this study is to provide direct evidence that decreased clearance of reflux events and/or increased frequency of reflux events is pre-dominantly responsible for presence of postprandial refluxant in the esophagus in PSS patients. We do this by detecting individual reflux events and their clearance by simultaneously utilizing scintigraphy [employing 5-sec acquisition intervals to detect individual reflux events rather than cumulative reflux over 30 sec as in most previously published scintigraphic studies of gastroesophageal reflux (7,8)], pH monitoring, and, in most, esophageal manometry with continuous LES pressure measurement. Nine previously published patients (9) with severe gastroesophageal reflux, esophagitis, and no evidence of PSS are used for comparison. ...
Article
The purpose of this study is to determine if frequent reflux events from an incompetent LES or poor clearance from decreased peristalsis is the predominant abnormality in PSS patients with severe reflux esophagitis. Seven patients with both classic manometric findings of PSS and endoscopic findings of esophageal ulcerations and/or Barrett's esophagus were compared to nine patients with similar endoscopic findings but with no evidence of a connective tissue disorder. All patients underwent simultaneous intraesophageal pH monitoring and scintigraphy for a total of 40 min after a radiolabeled meal. Four of the PSS patients and all the non-PSS patients had simultaneous manometry. We found that PSS patients had significantly fewer reflux events (P less than 0.01), but the reflux events had significantly longer duration (P less than 0.01) compared to patients with similar severity of esophagitis and no connective tissue disease. We conclude that decreased smooth muscle peristalsis appears to be the primary contributor to acid exposure and esophageal injury in PSS.
... After the first study, there can be scatter in the ROIs from activity in the gastric fundus or mild retention of radiotracer in the esophagus, and baseline correction is necessary. This aspect is described in greater detail in an earlier publication from our laboratory (4). ...
... We use the supine technique for our initial diagnostic evaluation and also for evaluation of therapeutic drug response because supine imaging excludes the effect of gravity. In P55, emptying ofthe esophagus in the supine position of RES is directly related to distal esophageal contractile amplitude (4). However, in the upright po sition, relatively prompt emptying occurs even with severe P55 involvement because of a flacid LES. ...
Article
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Supine radionuclide esophageal scintigraphy (RES) and manometry were used to prospectively evaluate metoclopramide effect on esophageal function and pressure amplitudes in 14 patients (12 females and two males; median time since diagnosis: 2 yr) with progressive systemic sclerosis (PSS). Quantitation of RES included calculation of percent emptying at 30 sec, and standard manometric measurements were obtained. RES and manometry were performed before and 10 min following the i.v. administration of metoclopramide. RES showed a significant increase in mean percent emptying from 36% to 46% after drug administration (p less than 0.01), while mean lower esophageal pressure (end-expiratory) increased from 2 to 11 mm of Hg (p less than 0.001). Manometry failed to reveal a significant increase in either distal or proximal mean esophageal contractile amplitude, and no correlation was found between the increase in percent emptying at RES and the change in lower esophageal pressure in the individual patient. RES is the only quantitative method presently available to evaluate bolus propagation in the esophagus, and it documented improved esophageal function after metoclopramide administration in a PSS population. When drug therapy is directed at augmentation of esophageal emptying, RES is an ideal method to evaluate drug response.
... Radionuclide oesophageal transit studies have been shown to be effective in the evaluation of oesophageal motility disorders [6] even when compared with oesophageal manometry, the current gold standard technique [7,8]. The high sensitivity of this method in detecting impaired oesophageal motility in patients with systemic sclerosis has been well demonstrated [9]. The aim of this study was to evaluate oesophageal transit time parameters by oesophageal scintigraphy to test the hypothesis that patients undergoing mediastinal RT may develop early oesophageal dysmotility and to assess the relationships between acute oesophagitis symptoms and dysmotility. ...
Article
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To quantitatively evaluate radiation-induced impaired oesophageal transit with oesophageal transit scintigraphy and to assess the relationships between acute oesophagitis symptoms and dysmotility. Between January 1996 and November 1998, 11 patients affected by non-small-cell carcinoma of the lung not directly involving the oesophagus, requiring adjuvant external beam radiotherapy (RT) to the mediastinum were enrolled. Oesophageal transit scans with liquid and semisolid bolus were performed at three pre-defined times: before (T0) and during radiation at 10 Gy (T1) and 30 Gy (T2). Two parameters were obtained for evaluation: 1) mean transit time (MTT); and 2) ratio between peak activity and residual activity at 40 seconds (ER-40s). Acute radiation toxicity was scored according to the joint EORTC-RTOG criteria. Mean values with standard deviation were calculated for all parameters. Analysis of variance (ANOVA) tests and paired t-Tests for all values were performed. An increase in the ER-40s from T0 to T1 or T2 was seen in 9 of 11 patients (82%). The mean ER-40s value for all patients increased from 0.8306 (T0) to 0.8612 (T1) and 0.8658 (T2). These differences were statistically significant (p < 0.05) in two paired t-Tests at T0 versus T2 time: overall mean ER-40s and upright ER-40s (p = 0.041 and p = 0.032, respectively). Seven patients (63%) showed a slight increase in the mean MTT value during irradiation but no statistically significant differences in MTT parameters were found between T0, T1 and T2 (p > 0.05). Using oesophageal scintigraphy we were able to detect early alterations of oesophageal transit during the third week of thoracic RT.
... As it is, the screening method should focus on evaluating motility and peristalsis of the GI tract. In this aspect, esophageal scintigraphy seems more appropriate than the uncomfortable and poorly tolerated manometric study, especially because sensitivity and specificity of both diagnostic modalities are comparable [8,13,14]. ...
Article
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Systemic sclerosis is a rare connective tissue disease, distinctive features of which are fibrosis and mi-croangiopathy. The esophagus is one of the most commonly involved internal organs. Most patients experience dysphagia, difficulties in swallowing and gastro-esophageal reflux. However, in up to one third of cases, the initial onset of esophageal disease may be clinically silent. There are several diagnostic modalities available for assessing both morphological and functional abnormalities of the esophagus. If structural abnormalities are suspected, endoscopy is the method of choice. Functional evaluation is best achieved with manometry. Both endoscopy and manometry are invasive techniques, with low patient acceptance. Barium-contrast study is well tolerated, but qualitative assessment of functional abnormalities is imprecise. Esophageal scintigraphy is an easy, non-invasive, sensitive and specific diagnostic modality. It can detect esophageal dysfunction even in asymptomatic patients. In patients already diagnosed with systemic sclerosis, scintigraphy is useful in evaluating severity and progression of the disease.
... This causes dysmotility characterized by low amplitude contractions of the esophagus and reduced pressure of the esophagogastric junction (EGJ). 1,2 Consequently, most patients with SSc report symptoms of reflux and dysphagia. 3 In severe cases, reflux may cause pneumonitis and strictures while dysphagia may necessitate percutaneous endoscopic gastrostomy 4 or parenteral nutrition. ...
Article
Background: Fibrosis and atrophy of esophageal smooth muscle cells cause gastro-esophageal reflux and dysphagia in most patients with systemic sclerosis (SSc). Recent studies indicate that distensibility of the esophagogastric junction (EGJ), assessed with the Functional Lumen Imaging Probe (FLIP) may be a more sensitive and accurate measure of sphincter function than manometry. We aim to describe and compare distension parameters of the EGJ in a well-characterized group of patients with SSc. Method: Twelve patients with SSc reporting reflux or dysphagia (11 women, median age 53 [range 35-72], duration of disease: 1-20 years) were investigated using distensibility testing of the EGJ. Patients were compared with 11 healthy volunteers (HV) (10 women, median age 53 [range 40-68]). The pressure and minimum diameter along the EGJ during ramp distension were used for distensibility analysis. Key results: Patients with SSc had significantly lower EGJ yield pressure (median: 4.0 mm Hg [Inter Quartile Range (IQR): 2.8-7.7]) than HV (median: 6.2 mm Hg [IQR: 9.4-26]) (P=.007). Likewise, the pressure-strain elastic modulus was lower in SSc patients (median 1.73 kPa [IQR: 1.16-2.15]) than in HV (median 2.41 kPa [IQR: 1.85-2.67]) (P=.03), indicating the reduced resistance to distension in SSc patient. Conclusion & inferences: Patients with SSc and symptoms of reflux and dysphagia have significantly reduced resistance to distension of the EGJ.
... It affects the skin and various internal organs like gastrointestinal tract, lungs, heart and kidneys [1]. Gastrointestinal tract involvement is very common, affecting about 90% of the systemic sclerosis patients [2,3]. Esophagus is the most frequently affected part of the gastrointestinal tract [4]. ...
Article
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Background: Systemic 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. Methods: 56 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. Results: Esophageal 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. Conclusion: While 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.
... It is known that about 75% of patients with SSc develop a significant esophageal involvement (3). Several authors (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15) have demonstrated that radionuclide esophageal transit is a safe, noninvasive, highly sensitive method that might be used as an alternative to esophageal manometry in patients with SSc. As shown in Table 1, esophageal scintigraphy compared to esophageal manometry has a high sensitivity (range 82%-100%) using a liquid and semisolid radiolabeled bolus in the supine posi tion. ...
Article
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Esophageal transit scintigraphy seems to be a valid methodology to assess impaired esophageal motility in early stages of disease. The purpose of this study was to discriminate patients with primary Raynaud's phenomenon (RP) and patients with systemic sclerosis (SSc) from healthy subjects by esophageal scintigraphy with a semisolid meal. We studied 32 patients with primary RP, 18 with SSc and 13 healthy subjects. Dysphagia, acid regurgitation and heartburn were scored. After an overnight fast, all subjects underwent esophageal scintigraphy, using a semisolid orally ingested bolus (10 mL apple puree) labeled with 99mTc-sulfur colloid. Esophageal transit and emptying time and integral value were evaluated with the subjects in the upright (sitting) and supine positions. Transit time was defined as the time from the entry of 50% of radioactivity into the upper esophagus until the clearance of 50% of the bolus from the whole esophagus. Emptying time was defined as the time from the entry of 50% of radioactivity into the upper esophagus, until the clearance of 100% of the bolus from the whole esophagus. Integral value was defined as the total counts under the time-activity curve normalized to the maximum. Esophageal transit and emptying time and integral value, evaluated in both positions, were significantly higher in patients with SSc than in healthy subjects and than in patients with RP. Moreover, patients with RP had all three parameters, assessed in supine position, significantly longer compared to healthy subjects. Clinical scores regarding dysphagia, acid regurgitation and heartburn were not significantly different between patients with RP and SSc. Esophageal transit and emptying time and integral value appear to be able to discriminate patients with primary RP from patients with SSc and patients with RP from healthy subjects, suggesting an early mild esophageal dysfunction in RP.
Article
Management of patients with progressive systemic sclerosis requires a thorough gastroenterological examination and a profound knowledge of possible gastrointestinal manifestations of the disease. The esophagus is the gastrointestinal segment most often involved. Smooth muscle atrophy leads to a loss of esophageal peristalsis, a defect of the lower esophageal sphincter, and gastric hypomotility, thus predisposing to severe reflux esophagitis. A rational diagnostic approach includes standard manometry and endoscopy. The prevention of strictures is the main aim of therapeutic efforts that may consist of lifestyle changes, prokinetic drugs, long-term application of proton pump inhibitors, and, if inevitable, surgical intervention. Involvement of the small intestine and colon is less common but may lead to life-threatening complications like chronic pseudoobstruction or pneumatosis cystoides intestinalis. The main therapeutic options consist of antibiotics for bacterial overgrowth and nutritional supplementation. Recently, a preliminary study with octreotide yielded promising results. Anorectal dysfunction can lead to fecal incontinence or rectal prolapse.
Article
The predictive value of esophagus-related symptoms for the diagnosis of esophageal dysmotility induced by systemic sclerosis (SSc) was prospectively evaluated in 50 consecutive patients with SSc. Patients were classified as symptomatic when either dysphagia or repeated episodes of heartburn were present. All patients underwent esophageal manometry; SSc-induced esophageal dysfunction was diagnosed when there was aperistalsis or marked hypocontractility of the distal two-thirds of the esophageal body. Twenty-nine patients (58%) had a history of esophagus-related symptoms, while 21 patients (42%) were asymptomatic. Compared to esophageal manometry, esophagus-related symptoms had a sensitivity of 64%, a specificity of 52%, a negative predictive value of 50% and a positive predictive value of 62% for the diagnosis of SSc-induced esophageal dysfunction. In conclusion, the association of esophagus-related symptoms and esophageal motility pattern is poor. As clinical management strategies depend on proof of esophageal dysfunction, screening examinations are mandatory in all patients with SSc.
Article
Background and objective It is unknown whether oesophageal disease is associated with systemic sclerosis‐associated interstitial lung disease (SSc‐ILD) severity, progression or mortality. Methods High‐resolution computed tomography (HRCT) scans from 145 SSc‐ILD patients were scored for fibrosis score, oesophageal diameter and presence of hiatal hernia. Fibrosis asymmetry was calculated as: (most affected side − least affected side)/(most affected side + least affected side). Mixed effects models were used for repeated measures analyses. Results Mean fibrosis score was 8.6%, and most patients had mild‐to‐moderate physiological impairment. Every 1 cm increase in oesophageal diameter was associated with 1.8% higher fibrosis score and 5.5% lower forced vital capacity (FVC; P ≤ 0.001 for unadjusted and adjusted analyses). Patients with hiatal hernia had 3.9% higher fibrosis score, with persistent differences on adjusted analysis (P = 0.001). Oesophageal diameter predicted worsening fibrosis score over the subsequent year (P = 0.02), but not when adjusting for baseline fibrosis score (P = 0.16). Oesophageal diameter was independently associated with mortality (P = 0.001). Oesophageal diameter was not associated with asymmetric disease or radiological features of gross aspiration. Conclusion Oesophageal diameter and hiatal hernia are independently associated with SSc‐ILD severity and mortality, but not with ILD progression or asymmetric disease. Oesophageal disease is unlikely to be a significant driver of ILD progression in SSc.
Article
Esophageal motility was assessed in patients with systemic sclerosis (SSc) by scintigraphy and compared with (i) extent of scleroderma, (ii) duration of disease, (iii) index of anti-topoisomerase I antibody (topo I), and (iv) pulmonary involvement. A multiple-swallow test was performed in 47 patients with SSc in the supine position with 99mTc-DTPA. A region of interest on the entire esophagus was defined and the retention ratio (RR) was calculated from a time-activity curve. Patients with diffuse scleroderma had higher RRs than those with limited scleroderma (48.8% vs. 30.0%; p < 0.05). There was no correlation between the RRs and the duration of disease. Patients with positive topo I had higher RRs than those who were negative (53.8% vs. 29.7%; p < 0.05). Patients with reduced % diffusion capacity for carbon monoxide (%DLCO) had higher RRs than those with normal %DLCO (40.5% vs. 19.6%; p = 0.03). Patients with reduced % vital capacity (%VC) had higher RRs than those with normal %VC (54.6% vs. 25.0%; p < 0.005). Patients with pulmonary fibrosis had higher RRs than those who were negative (58.5% vs. 20.3%; p < 0.00005). Esophageal dysfunction in patients with SSc showed a correlation with the extent of scleroderma, positive topo I, and pulmonary involvement. The RR can be an objective clinical marker for the severity of organ fibrosis.
Article
Despite improvement in pharmacologic management, the reflux seen in patients who have scleroderma is significantly greater than the reflux seen in patients who have idiopathic reflux. Furthermore, even with significant symptom improvement, half of the patients who have scleroderma do not show complete healing of esophagitis, owing to residual gastroesophageal reflux. Acid and bile reflux monitoring and endoscopic control examination should be used routinely to provide quantitative information on reflux damage and control. These patients need repeated adjustment of maintenance drug doses.
Article
In most patients with progressive systemic sclerosis the esophagus is affected. Reflux symptoms are most frequent whilst dysphagia also occurs. The radionuclide esophageal transit study is a sensitive screening test for esophageal dysfunction. In this study, we evaluated the esophageal motility of patients with progressive systemic sclerosis using a solid-phase radionuclide esophageal study. Thirty-two patients with progressive systemic sclerosis and 30 normal volunteers were studied with solid-phase radionuclide esophageal study. Each subject was placed in a supine position above a gamma camera linked to a computer and was given a 4-mL bolus of solid gelatin containing 1 mCi of Tc-99m phytate. Data were acquired in the list mode. Twenty-nine of the 32 patients (91%) had abnormal findings from the study. The radionuclide esophageal transit study can be regarded as a useful tool for evaluating the esophageal function in patients with progressive systemic sclerosis and in the follow-up of treatment.
Article
The purpose of this paper is to study the esophageal motility pattern and the frequency of acid reflux in patients diagnosed to have progressive systemic sclerosis and compare the results to that of normal controls. All consecutive patients diagnosed to have progressive systemic sclerosis between 1417-1419 (Hijra year) at King Khalid University Hospital, Riyadh, Kingdom of Saudi Arabia were included (Group I). History of heartburn, dysphagia and regurgitation was reviewed. Drugs that might suppress acid or alter motility were discontinued 2 weeks before inclusion. The results were compared to that of 21 symptomatic controls of similar age and sexes that were seen at the same period (Group II). Esophageal manometry and upper gastrointestinal endoscopy were performed in all patients. Ambulatory 24 hour-pH monitoring was carried out in 6 patients of group 1 and 20 patients of group II. Thirteen progressive systemic sclerosis patients (12 females) mean age was 38.7 years and 21 (19 females) mean age was 34.8 years were included. The symptom scores, lower esophageal sphincter pressure, esophageal contractions amplitude were significantly worse in patients compared to control, dysphagia was mostly due to aperistalsis. All progressive systemic sclerosis patients showed the typical esophageal manometry pattern of lower esophageal sphincter pressure and diminished amplitude with aperistalsis. Gastroesophageal reflux was detected in 83% of patients with progressive systemic sclerosis. Moreover, all 24-hour pH monitoring variables were significantly worse in group I. Patients with progressive systemic sclerosis usually present with heartburn, dysphagia and regurgitation. Esophageal manometry typically shows lower pressure and aperistalsis. Gastroesophageal reflux is frequent.
Article
To look for the frequency of oesophageal dysfunction using radionuclide oesophageal transit scintigraphy in 145 patients with undifferentiated connective tissue disease (UCTD); to seek the correlation between the clinical/laboratory data and scintigraphic alterations; and to determine predictive value of radionuclide oesophageal transit scintigraphy for evolution to established connective tissue disease (CTD). One hundred and forty-five patients with UCTD were examined by 99mTc-DTPA oesophageal transit scintigraphy. The intraoesophageal transport of the radiopharmaceutical was followed and imaged by a gamma camera, a series of 128 x 128 images were stored and evaluated. The correlation between the scintigraphic data and clinical and laboratory parameters was analysed statistically. Unequivocally positive scintigraphy, indicative of motor abnormality was found in 46% of patients (66), 71% (47) of whom were totally asymptomatic. Significant correlation was found between the presence and severity of scintigraphic alterations and antinuclear antibodies, the anti-beta2GPI, IgM, IgG, the aCL antibody positivity, and the skin symptoms. Scintigraphic positivity was significantly more frequent in patients evolving to definitive CTD (P = 0.0178), and abnormal scan predisposed to transition into the definitive CTD (odds ratio, 2.292; CI, 1.610-4.525). Its cumulative positive predictive value was found to be 43% and cumulative negative predictive value 73% with regard to the development of a definitive CTD. Our results show that scintigraphic alterations together with clinical and laboratory alterations can help the clinician in the prediction of final outcome.
Article
Esophageal functional abnormalities may lead to regurgitation, chronic esophagitis and life-threatening conditions such as aspiration pneumonia. In mixed connective tissue disease patients, previous reports showed that esophageal dysfunction varies according to the method employed for investigation. Our study was conceived to: (i) assess esophageal motility and mucosal aspects in patients with mixed connective tissue disease by endoscopy, cine-esophogram and scintigraphy focusing on the prevalence of each exam; and (ii) verify the association between pulmonary and esophageal dysfunctions. Twenty-four mixed connective tissue disease patients were enrolled for this study. Cine-esophogram and upper digestive endoscopy with mucosal biopsy were performed according to previous standardization. Radionuclide esophageal scintigraphy was performed with a semisolid meal with (99m)Tc. Eleven healthy individuals voluntarily submitted to scintigraphy as controls. Cine-esophogram showed esophageal delayed emptying in 90% of patients. At scintigraphy there was a significant delay in total esophageal transit time in the group of patients when compared to healthy controls (35.3 +/- 8.2 s. vs. 13.6 +/- 9.5 s.; P < 0.0001). The whole esophageal body showed dysmotility in 96% of patients. The cine-esophogram detected functional esophageal impairment similar to scintigraphic findings. Histopathologic examination found esophagitis in 95% of studied patients. Reduced lung volumes were associated with esophagitis and delayed esophageal clearance at scintigraphy, observed at the distal portion of the esophagus. Esophageal scintigraphy is easy to perform, with good acceptance by patients with low radiation exposition. It is a useful non-invasive test for follow-up and interventional studies concerning esophagus dysfunction.
Article
Progressive systemic sclerosis (PSS) causes smooth muscle atrophy and fibrosis of the distal two-thirds of the esophagus. Motility studies show reduced-amplitude or absent peristaltic contractions in this region and normal or decreased lower esophageal sphincter pressure. Patients complain of dysphagia, heartburn, and regurgitation due to reflux and dysmotility. Complications include strictures found in 17% to 29% of patients and Barrett esophagus is 0% to 37%. Candida esophagitis is a complication of PSS not seen with non-PSS reflux. Esophageal disease correlates with pulmonary involvement but not with disease in the stomach or intestines. Whether reflux contributes to the pulmonary disease is an open question. Although manometry is the gold standard for diagnosis, cine-esophagram and scintography are only slightly less sensitive and should be considered for following the patients. Symptoms correlate poorly with evidence of esophagitis or abnormal 24-hour pH recordings. As a result, it is unclear which patients should receive acid-reducing or prokinetic medications and which medication to use. Aspiration precautions are important in those with severe esophageal dysmotility. This review of the literature highlights many areas of uncertainty in the diagnosis and treatment of esophageal disease in PSS that can be addressed in clinical studies.
Article
Gastric emptying, antral motility, and gallbladder emptying after a liquid fatty meal were studied by ultrasound in 25 patients with progressive systemic sclerosis and in 25 sex- and age-matched controls. In patients with systemic sclerosis, the possible role of autonomic dysfunction was evaluated by four noninvasive cardiovascular reflex tests. Despite a significant delay of gastric emptying and a significant postprandial antral hypomotility in the patients with systemic sclerosis, the fat-induced gallbladder emptying was only slightly reduced, reaching no significant level when compared to the controls. The prolongation of gastric emptying correlated significantly with the duration of the disease. Although 36% of the patients in the systemic sclerosis group exhibited signs of autonomic cardiac dysfunction, there was no evidence of an association between these signs and gastric motor dysfunction. In conclusion motility disorders of the gallbladder seem to play a minor role in the upper gut involvement of systemic sclerosis, whereas motility disorders of the stomach are frequent and can be easily recognized noninvasively by real-time ultrasound.
Article
Scleroderma (systemic sclerosis) is a connective tissue disorder characterized by thickening and fibrosis of the skin and visceral involvement that may include the heart, lungs, kidneys, and gastrointestinal tract. At least 40-50% of patients with scleroderma experience esophageal symptoms such as heartburn and dysphagia, while up to 90% of patients have esophageal dysfunction on objective testing at some point in their disease. The disease results in smooth muscle dysfunction that causes esophageal aperistalsis and reduced lower esophageal sphincter pressures. Gastroesophageal reflux with poor acid clearance results with an increased incidence of complications such as peptic stricture and Barrett's esophagus. Aggressive medical therapy is necessary to prevent these and other complications of gastroesophageal reflux.
Article
Liquid esophageal transit and gastric emptying, mouth-to-cecum transit, and whole gut transit of a solid-liquid meal were measured in 14 patients with PSS, 16 control subjects (esophageal transit), and 20 control subjects (gastrointestinal transit), respectively, by using scintigraphic techniques, the hydrogen breath test, and stool markers. In patients with PSS, the glucose hydrogen breath test for detection of small intestinal overgrowth was performed and various gastrointestinal symptoms were determined. Esophageal transit and gastric emptying were significantly prolonged in PSS patients with 11 of 14 PSS patients (79%) disclosing delayed esophageal transit and eight of 14 PSS patients (57%) disclosing delayed gastric emptying. All PSS patients with prolonged gastric emptying also had delayed esophageal transit and there was a significant positive correlation between esophageal transit and gastric emptying (r = 0.696, P < 0.01). No significant differences between PSS patients and controls were detected concerning mouth-to-cecum transit and whole gut transit, but abnormally delayed mouth-to-cecum transit was found in four of 10 PSS patients (40%) and abnormally prolonged whole gut transit was detected in three of 13 PSS patients (23%). Small bacterial overgrowth was diagnosed in three of 14 PSS patients (21%). Delayed esophageal transit and gastric emptying were associated with dysphagia, retrosternal pain, and epigastric fullness, while prolonged whole gut transit was associated with constipation. It is concluded that delayed gastric emptying is frequently associated with esophageal transit disorders in PSS patients and may be one important factor for the development of gastroesophageal reflux disease in these patients.
Article
This study examines the scintigraphic transit pattern in a variety of esophageal disorders. Scintigraphy was performed with a semi solid bolus and the patient in an upright position. Condensed esophageal images were obtained from which we derived the esophageal transit time. The pattern of bolus transit was graded by the duration of transit and by the presence of hold up or retrograde motion. Scintigrams were performed in 11 volunteers and 88 patients whose esophageal function had been confirmed by conventional gastroesophageal techniques. Esophageal disorders examined included achalasia (20), scleroderma (9), esophageal carcinoma (8), Barrett esophagus (5), and reflux esophagitis (27). We also examined the effects of gastroesophageal surgery on esophageal function. Transit times distinguished grossly abnormal esophageal function from normal but did not distinguish between different esophageal disorders. Graded transit patterns were a more sensitive indicator of esophageal function and permitted some differentiation between esophageal disorders and allowed evaluation of the effects of gastroesophageal surgery.
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A better understanding of scintigraphic findings may lead to a wider acceptance of esophageal transit studies. The purpose of this study, therefore, was to correlate standard manometric parameters with the quantitative and qualitative characteristics of liquid and semi-solid bolus transport. Twenty-nine patients were simultaneously investigated with esophageal scintigraphy and manometry. Single-swallow and sum-image data of six consecutive swallows were analyzed. No significant relationship between transit time and the velocity of the peristaltic wave could be identified, which suggests that factors other than peristaltic velocity (e.g., pharyngeal pump) essentially modulate esophageal transit. There was also no linear correlation between esophageal emptying and peristaltic amplitudes. Emptying was normal in patients with amplitudes greater than 30 mmHg and reduced in those with amplitudes less than 30 mmHg. This suggests that a threshold pressure greater than 30 mmHg is necessary to propel a test bolus adequately. Patterns in condensed images have been shown to specifically reflect the events in corresponding manometric recordings. Normal and different pathologic types of peristalsis presented analogous findings in both modalities. Thus, an analysis of the relationship between bolus transport and contraction parameters in simultaneous studies increases understanding of quantitative and qualitative scintigraphic results.
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Three modalities for assessing esophageal dysfunction in patients with systemic sclerosis were prospectively compared. Seventeen patients underwent (a) esophageal manometry with measurement of distal esophageal peak contraction pressure amplitude, percentage of peristaltic waves, and lower esophageal sphincter pressure; (b) cine-esophagography with scoring based on residual contrast and the character of visualized waves; and (c) esophageal transit scintigraphy with quantification of residual swallowed tracer. Highly significant correlations were found between scintigraphic residual and cine-esophagography score, between scintigraphic residual and manometric amplitude, and indeed between all pairs of measured esophageal function parameters except those involving lower esophageal sphincter pressure. In addition, scintigraphy and cine-esophagography showed comparable ability to discriminate between patients with abnormal and normal esophageal motor function. Symptoms did not significantly correlate with quantitative parameters, nor did they have diagnostic discriminating ability. Induction of Raynaud's phenomenon in a subgroup of patients had no detectable effect on esophageal function. It was concluded that these three diagnostic modalities are approximately equivalent in their ability to detect esophageal dysmotility in systemic sclerosis and measure its severity.
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The current available methods for diagnosis of GORD are symptom questionnaires, catheter and wireless pH-metry, impedance-pH monitoring and Bilitec(@). Osophageal pH monitoring allows both quantitative analysis of acid reflux and assessment of reflux-symptom association. Impedance-pH monitoring detects all types of reflux (acid and non-acid) and allows assessment of proximal extent of reflux, a relevant parameter for understanding symptoms perception and extraoesophageal symptoms. Bilitec provides a quantitative assessment of duodeno-gastro-oesophageal reflux. Oesophageal motor abnormalities have been associated with GORD symptoms as well as chest pain and dysphagia. High-resolution manometry contributed to re-classify oesphageal motor disorders. However, barium swallows are still essential for evaluation of oesophageal anatomy and combined oesophageal manometry-impedance can assess oesophageal motility and bolus transit simultaneously in a non-radiological way. Still in experimental phase, high-frequency ultrasound allows monitoring of the oesophageal wall thickness and exaggerated longitudinal muscle contraction that might be associated to chest pain and dysphagia. This chapter provides a critical evaluation of the clinical application of these techniques.
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SSc is a connective tissue, multisystem disorder of unknown aetiology. The gastrointestinal tract (GIT) is affected in up to 90% of patients. The exact pathophysiology of GIT involvement is not known, but it is related to both neurogenic and myogenic abnormalities as well as possible vascular and ischaemic changes. Thinning of the internal anal sphincter (IAS) has been demonstrated in SSc with faecal incontinence. We aimed to investigate anal sphincter structure in patients with SSc. Forty-four SSc patients [24 symptomatic (Sx) and 20 asymptomatic (ASx)] and 20 incontinent controls (ICs) were studied. Patients underwent anorectal manometry and endoanal US. In the ICs, external anal sphincter defects were more common, but the IAS was less atrophic, evident by both atrophy scores and IAS thickness. There was no significant difference in atrophy scores [Sx: 2 (1.5-3) vs ASx: 2 (1-2)] or IAS thickness [Sx: 1.85 (1.5-2.3) vs ASx: 1.8 (1.7-2.25)] between the Sx and ASx SSc patients. Patients with SSc (both Sx and ASx) have thin and atrophic IAS, suggesting that IAS atrophy develops even in ASx patients and this may be amenable to treatment with sacral neuromodulation.
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Gastric emptying, antral motility, and gallbladder emptying after a liquid fatty meal were studied by ultrasound in 25 patients with progressive systemic sclerosis and in 25 sex- and age-matched controls. In patients with systemic sclerosis, the possible role of autonomic dysfunction was evaluated by four noninvasive cardiovascular reflex tests. Despite a significant delay of gastric emptying and a significant postprandial antral hypomotility in the patients with systemic sclerosis, the fat-induced gallbladder emptying was only slightly reduced, reaching no significant level when compared to the controls. The prolongation of gastric emptying correlated significantly with the duration of the disease. Although 36% of the patients in the systemic sclerosis group exhibited signs of autonomic cardiac dysfunction, there was no evidence of an association between these signs and gastric motor dysfunction. In conclusion motility disorders of the gallbladder seem to play a minor role in the upper gut involvement of systemic sclerosis, whereas motility disorders of the stomach are frequent and can be easily recognized noninvasively by real-time ultrasound.
Article
Systemic diseases can affect any part of the gastrointestinal system, including the esophagus. Clinical manifestations of systemic disorders affecting the esophagus can range from debilitating symptoms, such as severe dysphagia with associated weight loss, to asymptomatic esophageal dysmotility. Evaluation of the esophagus may permit diagnosis of a systemic disorder, such as scleroderma. This chapter reviews the esophageal manifestations of systemic diseases, including connective tissue diseases, endocrine and metabolic diseases, inflammatory diseases, neuromuscular disorders, and dermatologic disorders. Pathogenesis, pathology, clinical manifestations, diagnostic features, and treatments are discussed.
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Patients suffering from systemic sclerosis can suffer from significant esophageal dysmotility. Standard tests such as esophageal manometry are invasive and can be distressing to the patient. Esophageal scintigraphy would be ideal, but most protocols are too time consuming for a busy clinical department. The aim of this study was to produce an optimal imaging and reporting protocol that would allow esophageal scintigraphy to become a standard clinical tool. Methods: We imaged 301 patients using a single swallow of a radiolabeled puree meal with the patient erect and in the supine position. A condensed image was produced, which was reported semiquantitatively using a five-point grading scale (0-4), where 0 was normal and 4 represented severe dysmotility. Results: All patients, even those with severe dysphagia, were able to tolerate the test. A sample of 25 studies was reported twice by two observers, who were blind to this study. There was no significant intra- or interobserver variation. Conclusion: We recommend this method in routine assessment of patients with systemic sclerosis.
Chapter
Despite advances in medical imaging techniques, such as computed tomography, ultrasound and magnetic resonance, nuclear medicine remains a growing speciality. The clinical practice of nuclear medicine is based on the use of radioactive materials (radionuclides) for the purposes of diagnosis and therapy. This technique is noninvasive and safe; in the majority of cases the administration of diagnostic doses of radiolabeled tracers results in no more radiation exposure than X-ray procedures. The behaviour of a biological substance of interest may be studied by introducing a small quantity of the tracer in a suitably radiolabeled form. The introduction of the tracer should not affect the behaviour of the substance under investigation, there must be stable attachment of the radiolabel to the parent molecule, and the radiolabel should be easily and accurately detected.
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Motor abnormalities of the oesophagus are characterised by a chronic impairment of the neuromuscular structures that co-ordinate oesophageal function. The best-defined entity is achalasia, which is discussed in a separate chapter. Other motor disorders with clinical relevance include diffuse oesophageal spasm, oesophageal dysmotility associated with scleroderma, and ineffective oesophageal motility. These non-achalasic motor disorders have variable prevalence but they could be associated with invalidating symptoms such as dysphagia, chest pain and gastro-oesophageal reflux disease. New oesophageal diagnostic techniques, including high-resolution manometry, high-frequency intraluminal ultrasound and intraluminal impedance, allow (1) better definition of peristalsis and sphincter function, (2) assessment of changes in oesophageal wall thickness, and (3) evaluation of pressure gradients within the oesophagus and across the sphincters that can produce normal or abnormal patterns of bolus transport. This chapter discusses recent advances in physiology, pathophysiology, diagnosis and treatment of non-achalasic oesophageal motor disorders.
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