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Summary

  1. Top of page
  2. Summary
  3. Introduction
  4. Patients and methods
  5. Methods
  6. Results
  7. Discussion
  8. Acknowledgement
  9. References

Aim  To assess the prevalence of oesophagitis, Barrett's oesophagus (BE) and other oesophageal mucosal abnormalities in patients with systemic sclerosis (SSc) without prior selection on digestive clinical presentation. We also investigated the association between oesophageal endoscopic and manometric data with clinical manifestations of SSc.

Methods  Oesophageal endoscopy and manometry were performed in 133 consecutive patients with SSc, receiving proton pump inhibitor (PPI) therapy since SSc diagnosis.

Results  Endoscopy revealed oesophagitis in 43 patients (32.3%), BE in 9 patients (6.8%), candidiasis in 7 patients (5.3%) and hyperplastic polyp arising in ectopic gastric mucosa in 1 patient. Patients with severe oesophageal motor impairment further exhibited a higher prevalence of interstitial lung disease (ILD) when compared with those without.

Conclusion  Our study underlines the high frequency of oesophageal mucosal abnormalities in unselected SSc patients receiving long-term PPI therapy. A relationship between oesophagitis/BE and severe manometric motor disturbances was established; these patients may require a higher regimen of PPI. Finally, our series indicates a correlation between severe oesophageal motor disturbances and evidence for ILD in patients, suggesting that gastro-oesophageal reflux may be one of the contributing factors of ILD in SSc; this subgroup of patients may require close monitoring of lung parameters.


Introduction

  1. Top of page
  2. Summary
  3. Introduction
  4. Patients and methods
  5. Methods
  6. Results
  7. Discussion
  8. Acknowledgement
  9. References

Systemic sclerosis (SSc) is a systemic disorder affecting the skin and other organs, especially the gastrointestinal tract. Oesophageal involvement is common in SSc, occurring in as many as 50–90% of patients.1–10 Histological examination of oesophageal biopsy specimens has revealed the replacement of normal smooth muscle by collagenous fibrosis and smooth muscle atrophy, leading to motor activity abnormalities in patients with SSc.2 SSc-related oesophageal motor activity impairment is indeed characterized by low pressure in the lower oesophageal sphincter (LES) and dysfunction of smooth muscle motor activity.2–7, 9–11 Oesophageal involvement is still recognized to be associated with significant morbidity in SSc patients as motor dysfunction may result in erosive oesophagitis, ulcers, as well as peptic stricture formation and Barrett's oesophagus (BE) with its potential for adenocarcinoma degeneration.2, 7–12 Moreover, other oesophageal mucosal abnormalities have been reported in SSc, including oesophageal candidiasis.2, 9, 10, 13, 14 However, previous series have concerned SSc patients who underwent endoscopy for oesophageal symptoms; in turn, symptomatic SSc patients may represent a subgroup with the most severe oesophageal impairment, resulting in overestimation of the entire frequency of oesophageal mucosal abnormalities (on endoscopy) in SSc.

These data, therefore, prompted us to conduct this study in 133 patients receiving proton pump inhibitor (PPI) therapy since SSc diagnosis, without prior selection of patients on digestive clinical presentation to (i) assess the prevalence of oesophagitis, BE and oesophageal adenocarcinoma, as well as other oesophageal mucosal abnormalities in patients; (ii) evaluate whether the endoscopic findings are associated with clinical manifestations, SSc duration and subsets, and oesophageal motor disturbances and (iii) assess whether the data of oesophageal manometry are correlated with the clinical characteristics of SSc.

Patients and methods

  1. Top of page
  2. Summary
  3. Introduction
  4. Patients and methods
  5. Methods
  6. Results
  7. Discussion
  8. Acknowledgement
  9. References

Patients

One hundred and thirty-three consecutive patients with a definite diagnosis of SSc were included in the study. The criteria for the diagnosis of SSc were based on the ACR criteria.15 The patient population consisted of 18 men and 115 women, median age 58.5 years (range 25–84 years); the median duration of the disease, considered to have existed from the onset of the first non-Raynaud's phenomenon clinical manifestation, was 6 years (range 1–38 years). Patients were grouped according to the criteria of Leroy et al.;16 35 patients (26.3%) had diffuse cutaneous SSc (dcSSc) and 98 (73.7%) had limited cutaneous SSc (lcSSc). All the patients received PPI therapy (initial standard dose, e.g. omeprazole 20 mg daily) since SSc diagnosis (range 0–3 months following SSc diagnosis).

All patients had a standardized evaluation of organ involvement, which resulted in the detection of complications related to SSc: (i) digital pitting scars; (ii) degree of skin involvement, as determined by the modified Rodnan score and17 (iii) high-resolution computed tomography scan (HRCT-scan) of the lungs and pulmonary function tests (PFT) were performed to investigate interstitial lung disease (ILD). ILD diagnosis was made in SSc patients exhibiting both (i) HRCT-scan abnormalities consistent with ILD including parenchymal micronodules or nodules, linear opacities (septal lines and nonseptal lines), irregularity of the interfaces between pleura and aerated lung parenchyma, ground-glass opacities, honeycombing, traction bronchiectases/bronchiolectases and architecture distortion; (ii) values of vital capacity and diffusing capacity for carbon monoxide (<75% of predicted values) on PFT and (iii) primary pulmonary arterial hypertension (without ILD), characterized by pulmonary arterial systolic pressure higher than 40 mmHg at rest on Doppler echocardiography.

Methods

  1. Top of page
  2. Summary
  3. Introduction
  4. Patients and methods
  5. Methods
  6. Results
  7. Discussion
  8. Acknowledgement
  9. References

Oesophageal manometry

Medication that might affect oesophageal motility (metoclopramide, domperidone and calcium channel antagonist) was discontinued at least 72 h prior to manometry. After a 12-h overnight fast, oesophageal manometry was performed. For the oesophageal manometric tracings, the recording chart was analysed to determine LES pressure and oesophageal motor function (i.e. characteristics of peristalsis).

After analysis, all the patients were classified according to Hurwitz's criteria18 for the degree of oesophageal involvement on manometry, as follows: (i) normal oesophageal motility (stage I); (ii) uncoordinated peristalsis with normal pressure wave amplitude (stage II); (iii) uncoordinated peristalsis with low-pressure wave amplitude (stage III) and (iv) both aperistalsis and decreased LES pressure (stage IV).

Oesophageal endoscopy

Oesophageal endoscopy was performed by two gastroenterologists (who are experienced in endoscopy assessment) to assess mucosal abnormalities associated with gastro-oesophageal reflux (GER). Mucosal damage of the oesophagus was classified according to the Los Angeles Classification19: grade A, one (or more) mucosal break no longer than 5 mm that does not extend between the top of two mucosal folds; grade B, one (or more) mucosal break more than 5 mm that does not extend between the top of two mucosal folds; grade C, one (or more) mucosal break that is continuous between the top of two or more mucosal folds but which involves less than 75% of the circumference; grade D, one (or more) mucosal break that involves at least 75% of the circumference.

Endoscopy was also made to detect other oesophageal abnormalities, such as Barrett's oesophagus: Multiple oesophageal biopsies were made when macroscopic abnormalities consistent with BE were detected; a definite diagnosis of BE was made by the finding of a villiform columnar-lined mucosa with goblet cells on biopsies obtained from the distal oesophagus. Long-segment BE, which is suspected to be associated with a higher risk of adenocarcinoma, was defined by the presence of lesions >3 cm;20, 21 telangiectasia, polyp, candidiasis and adenocarcinoma.

Comparison of oesophageal involvement on endoscopy and complications of SSc

The following parameters were compared between SSc patients with endoscopic oesophagitis/BE and patients without: (i) median age; (ii) median SSc duration; (iii) SSc subsets; (iv) digestive symptoms; (v) findings of oesophageal manometry and (vi) degree of skin involvement, prevalence of pitting scars, pulmonary arterial hypertension and ILD.

Comparison of oesophageal involvement on manometry and both pulmonary manifestations and other complications of SSc

Data were further compared between SSc patients with severe oesophageal motor impairment (stage IV) and patients without (stages I/II/III): (i) median age; (ii) median SSc duration; (iii) SSc subsets and (iv) degree of skin involvement, frequency of pitting scars, ILD and pulmonary arterial hypertension. This division of the motility disturbances into severe (stage IV) and other stages (stages I/II/III) was made post hoc.

Statistical analysis

Statistical analyses were conducted using sas version 8.02 (SAS Institute Inc., Cary, NC, USA).

For group comparison involving binary data, we used either the chi-squared test or Fisher's exact test, depending on the cells’ expected count (more or less than five, respectively). Comparisons involving continuous data were performed using the Mann–Whitney test. The results were regarded as significant when the P-value was less than 0.05.

Moreover, we performed a univariate logistic regression to identify the predictive factors of the presence of severe oesophageal motor disturbances (stage IV) on manometry; these results are reported as odds ratio (OR) and 95% confidence interval (95% CI); the used level of significance was P < 0.05 in all performed tests. Variables were further selected for multivariate logistic regression to determine the independent predictive factors for the criteria: presence of severe oesophageal motor disturbances (stage IV) on manometry; the results were also expressed as OR (95% CI) and the used level of significance was P < 0.05.

Results

  1. Top of page
  2. Summary
  3. Introduction
  4. Patients and methods
  5. Methods
  6. Results
  7. Discussion
  8. Acknowledgement
  9. References

Oesophageal symptoms and patients’ therapy

Before undergoing endoscopy, patients were asked for the presence of oesophageal symptoms. SSc patients had the following symptoms: heartburn (n = 103; 77.4%), dysphagia (n = 19; 14.3%) and nausea/vomiting (n = 13; 9.8%).

Furthermore, before undergoing systematic endoscopy, patients were also asked for PPI regimen; SSc patients’ PPI regimen was as follows: omeprazole 20 mg/day (n = 119; 89.4%) and 40 mg/day (n = 14; 10.5%).

Oesophageal manometric involvement

According to Hurwitz's criteria for the degree of oesophageal involvement on manometry, 32 patients had normal oesophageal motility (stage I), 24 patients had uncoordinated peristalsis with normal pressure wave amplitude (stage II), 13 patients had uncoordinated peristalsis with low-pressure wave amplitude (stage III) and 64 patients had both aperistalsis and decreased LES pressure (stage IV).

After analysis, oesophageal motor disorders were divided in the SSc patients as severe (stage IV; 48.1%), moderate (stages II and III; 27.8%) and normal (stage I; 24.1%).

Oesophageal endoscopic involvement

As shown in Table 1, oesophageal endoscopy revealed oesophagitis in 43 patients with SSc (32.3%). Oesophageal mucosal abnormalities, according to the Los Angeles Classification, were as follows – (i) grade A: n = 17 (12.8%), (ii) grade B: n = 9 (6.8%), (iii) grade C: n = 11 (7.5%) and (iv) grade D: n = 6 (4.5%).

Table 1.   Oesophageal mucosal abnormalities on systematic endoscopy in patients with SSc (n = 133)
 Patients
n (%)
  1. SSc, systemic sclerosis.

Oesophagitis43 (32.3)
Barrett's oesophagus9 (6.8)
Oesophageal candidiasis7 (5.3)
Oesophageal hyperplastic polyp arising in ectopic gastric mucosa1 (0.8)
Oesophageal adenocarcinoma0 (0)

Moreover, nine patients had BE (6.8%). None of the latter SSc patients exhibited long-segment BE. Furthermore, only one of the nine patients had histological dysplasia, i.e. low-grade dysplasia.

Seven other SSc patients (5.3%) had oesophagitis related to Candida albicans (Table 1). Endoscopy showed both erosions and white plaques involving the oesophagus, and cultures of oesophageal biopsy specimens proved positive for C. albicans; three of these patients had dcSSc and four had lcSSc.

One additional patient with SSc (0.8%) had oesophageal polyp. In this patient, endoscopy showed a bilobar (10 mm and 8 mm in diameter) sessile polyp arising in the posterior wall of the oesophago-gastric junction. Oesophageal biospy specimens showed evidence of hyperplastic polyp arising in ectopic gastric mucosa. Endoscopic mucosal resection of the whole polyp was made. At 1-year follow-up, the patient exhibited no recurrence of oesophageal hyperplastic polyp arising in ectopic gastric mucosa.

Predictive factors of oesophagitis/Barrett's oesophagus

As shown in Table 2, there were no significant differences between patients with and without endoscopic oesophagitis/BE as regards: median age (P = 0.783) and median SSc duration (P = 0.870). In addition, no significant difference between subtypes of SSc for oesophagitis/BE frequency was observed (Table 2).

Table 2.   Clinical characteristics of SSc patients with oesophagitis/Barrett's oesophagus, on systematic endoscopy, compared with those without
 Oesophagitis/Barrett's oesophagus (n = 52)Absence of oesophagitis/ Barrett's oesophagus (n = 81)P-value
  1. SSc, systemic sclerosis; lcSSc, limited cutaneous SSc; dcSSc, diffuse cutaneous SSc; ILD, interstitial lung disease. Except where indicated (values in percentages), values are median.

Age (years)59600.783
Age > 50 years71.1%61.7%0.265
SSc duration (years)860.870
SSc duration > 5 years57.7%58%0.970
SSc subset36.7% of lcSSc63.2% of lcSSc0.350
 45.7% of dcSSc54.3% of dcSSc 
Digital pitting scars44.2%46.9%0.762
ILD40.4%31.2%0.329
Pulmonary arterial hypertension5.8%8.6%0.739
Dysphagia26.9%6.2%0.0008
Nausea/vomiting15.2%6.2%0.02

The following symptoms were more frequent in patients with endoscopic oesophagitis/BE when compared with those without: dysphagia (26.9% vs. 6.2%; P = 0.0008), nausea/vomiting (15.4% vs. 6.2%; P = 0.02). Moreover, the subgroup of patients with BE also exhibited more commonly dysphagia (44.4% vs. 12.1%; P = 0.024) and nausea/vomiting (33.3% vs. 8.1%; P = 0.043).

Furthermore, we observed that patients with severe oesophageal motor disorders (stage IV) had more frequently endoscopic oesophagitis/BE when compared with those without (stages I/II/III) (65.4% vs. 37%; P = 0.0014); no patient with normal oesophageal motility had either grades C/D oesophagitis or BE on endoscopic examination. The prevalence of severe oesophageal motor disorders (stage IV) on manometry was further higher in patients with BE (100% vs. 44.4%; P = 0.001).

In addition, median values of the modified Rodnan score were not different in patients with and without oesophagitis/BE (10 vs. 7); as shown in Table 2, the prevalence of the following manifestations related to SSc was similar in patients with and without oesophagitis/BE: pitting scars, ILD and pulmonary arterial hypertension.

Comparison of oesophageal involvement on manometry and pulmonary parameters and other characteristics of SSc

As shown in Table 3, the prevalence of ILD was higher in patients with severe oesophageal motor dysfunction (stage IV) than in patients without (stages I/II/III) (45.3% vs. 26.1%; P = 0.021) (Table 3).

Table 3.   Clinical characteristics of SSc patients with severe oesophageal motor impairment on manometry, compared with those without*
 Stage IV* (n = 64)Stages I/II/III* (n = 69)P-value
  1. SSc, systemic sclerosis; lcSSc, limited cutaneous SSc; dcSSc, diffuse cutaneous SSc; ILD, interstitial lung disease; PAH, primary pulmonary arterial hypertension. Except where indicated, values are median.

  2. * Oesophageal manometric motor abnormalities – stage I: normal oesophageal motility, stage II: uncoordinated peristalsis with normal pressure wave amplitude, stage III: uncoordinated peristalsis with low-pressure wave amplitude, stage IV: both aperistalsis and decreased LES pressure.

Age (years)61580.821
Age > 50 years68.8%62.3%0.342
SSc duration (years)6.560.657
SSc duration > 5 years57.8%57.9%0.951
SSc subset48% of lcSSc52% of lcSSc0.950
 48.6% of dcSSc51.4% of dcSSc 
Digital pitting scars46.9%44.9%0.822
ILD45.3%26.1%0.020
PAH6.3%8.7%0.592

Finally, we failed to find differences between patients with severe oesophageal motor impairment when compared with patients without: median age (P = 0.821), SSc duration (P = 0.657), SSc subsets, median values of the modified Rodnan score, pitting scars (P = 0.822) and pulmonary arterial hypertension (P = 0.592) (Table 3).

The clinical risk factors for the presence of severe oesophageal motor dysfunction, tested by uni- and multivariate analyses, are listed in Table 4. We have only retained ILD, as being independently associated with the presence of severe oesophageal motor dysfunction (stage IV) on manometry [OR 2.480 (95% CI: 1.161–5.29)] (Table 4).

Table 4.   Clinical characteristics of SSc patients with severe oesophageal motor impairment on manometry (stage IV), compared with those without (stages: I/II/III)*
 UnivariateMultivariate
P-valueOR (95% CI)P-valueOR (95% CI)
  1. SSc, systemic sclerosis; ILD, interstitial lung disease; PAH, primary pulmonary arterial hypertension; OR, odds ratio; 95% CI, 95% confidence interval.

  2. * Oesophageal manometric motor abnormalities – stage I: normal oesophageal motility, stage II: uncoordinated peristalsis with normal pressure wave amplitude, stage III: uncoordinated peristalsis with low-pressure wave amplitude, stage IV: both aperistalsis and decreased LES pressure.

Age > 50 years0.3421.414 (0.691–2.895)0.2351.600 (0.736–3.476)
SSc duration > 5 years0.9510.936 (0.469–1.867)0.6690.850 (0.405–1.785)
SSc subset0.9501.025 (0.473–2.218)0.8230.907 (0.385–2.135)
Digital pitting scars0.8221.082 (0.546–2.141)0.7321.135 (0.548–2.351)
ILD0.0202.348 (1.133–4.865)0.0192.480 (1.161–5.294)
PAH0.5920.700 (0.188–2.604)0.6740.745 (0.190–2.927)

Discussion

  1. Top of page
  2. Summary
  3. Introduction
  4. Patients and methods
  5. Methods
  6. Results
  7. Discussion
  8. Acknowledgement
  9. References

In patients with SSc, oesophageal mucosal abnormalities are principally related to both low pressure in the LES and abnormal peristalsis, leading to decreased acid clearance and prolonged acid–mucosal contact time.2, 7, 13, 22 Previous series have evaluated the prevalence of oesophagitis to be 56–60% in SSc patients exhibiting symptoms consistent with oesophageal dysfunction;13, 23 in a series of 53 symptomatic SSc patients, oesophageal stricture has also been reported in more than 40% of cases.13 In our large cohort of SSc patients who underwent systematic endoscopy, we have observed a lower prevalence of oesophagitis (32.3% of cases). We have further found a decreased frequency of the more severe mucosal damage, as 12.8% of our patients had grades C/D oesophagitis on endoscopy. We suggest that our findings may be explained by the following parameters. First, our study concerned 133 consecutive patients with SSc who were not selected according to digestive presentation; thus, a selection bias, based on the severity of SSc-related digestive involvement, could be excluded. Secondly, all of our patients received long-term PPI therapy (at standard dose, e.g. omeprazole 20 mg daily) following SSc diagnosis, leading to a probable decreased prevalence of oesophageal mucosal abnormalities; in earlier series, many SSc patients were given antisecretory agents other than PPI drugs that are less effective than PPIs in GER management, and the time of antisecretory agent initiation in SSc course was not provided. Finally, in this instance, none of our patients received nonsteroidal anti-inflammatory drugs, which may have resulted in decreased onset of oesophageal ulcers and efficacy of antisecretory therapy.24, 25

Association between SSc and BE has been documented.2, 13, 26 Katzka et al.26 have indeed reported that 37% of symptomatic SSc patients exhibited endoscopic BE. The prevalence of BE in SSc patients has also been found to be higher than the 4–13% prevalence of BE in non-SSc patients with GER.13 In this instance, we have observed a lower prevalence of BE in SSc patients (6.8% of cases) who were not selected on digestive presentation. Because our patients received PPI therapy since SSc diagnosis, the use of PPI therapy may have been beneficial in the prevention of both BE and BE-related complications onset (only one patient with BE developed low-grade dysplasia); although no definite conclusion can be drawn from our data, as we have not included a control group (for ethical reasons). Our findings are in accordance with those described by other investigators in non-SSc patients;27, 28 in a series of 350 non-SSc BE, patients who delayed using PPI therapy at least 2 years after diagnosis of BE had 5.6 times the risk of developing dysplasia when compared with those who used PPI therapy in the first year.28 We therefore suggest that SSc patients should be encouraged to receive both early and long-term PPI therapy. Interestingly, we have also observed that the subgroup of SSc patients with BE more frequently exhibited dysphagia (P = 0.024); our results confirm those of other authors in non-SSc patients.29, 30 Csendes et al.29 have, therefore, noted that non-SSc patients with BE, compared with patients with intestinal metaplasia of the cardia, had higher frequencies of dysphagia and heartburn. In a series of 517 patients with gastro-oesophageal reflux disease, Gerson et al.30 have also reported the following predictive clinical parameters of BE: odynophagia and heartburn. The setting of dysphagia in non-SSc patients with BE has been suggested to be associated with severe oesophageal motor disorders;29, 30 our nine patients with BE had severe oesophageal motor dysfunction (stage IV) in 100% of cases. Furthermore, in the present series, no SSc patient with BE had oesophageal adenocarcinoma. Nevertheless, because an increased risk of adenocarcinoma has been found in patients with BE,31–33 SSc patients with BE should also be screened by endoscopy: (i) every 2–3 years if no dysplasia is found; (ii) yearly if low-grade dysplasia occurs and (iii) every 3 months in the case of high-grade dysplasia.7

Hyperplastic polyps arising in ectopic gastric mucosa have been described in the oesophagus, particularly in the mid-/distal oesophagus and at the oesophago-gastric junction of non-SSc patients.34–36 Ectopic gastric mucosa arising in the oesophagus has been reported to occur in response to a chronic local stimulus, notably to regurgitation of stomach acid.34–36 We report, to our knowledge, the first case of hyperplastic polyp arising in ectopic gastric mucosa in a patient with SSc; a diagnosis of hyperplastic polyp arising in ectopic gastric mucosa related to GER could reasonably be made in our SSc patient. Because ectopic gastric mucosa in the oesophagus has the potential for transformation into adenoma and adenocarcinoma, the lesion was excised in our SSc patient.37–39Candida sp. has been reported to be one of the most common causes of opportunistic infections in patients with connective tissue disorders.40Candida oesophagitis has been described in 38–44% of SSc patients.13, 14 In this instance, oesophageal candidiasis occurred in 5.3% of our patients with dcSSc and lcSSc. All our SSc patients with oesophageal candidiasis had severe oesophageal motor involvement (stage IV). Our findings underscore that oesophageal stasis related to dysmotility contributes to the increased risk of oesophageal candidiasis in SSc patients.2, 13, 14

From a practical point of view, the knowledge of predictive factors of oesophagitis/BE is essential in patients with SSc, as it may result in management at an early stage. Previous investigators have observed a lack of correlation between the frequency of endoscopic oesophagitis and either the duration of SSc or the patients’ age. In the present study, patients’ age and sex, SSc duration, degree of skin involvement, pitting scars, pulmonary arterial hypertension as well as ILD could not be considered to be predictive of oesophagitis/BE in SSc patients. Our series demonstrates that oesophagitis/BE occurs with equal frequency in patients with lcSSc and those with dcSSc (45.7% vs. 36.7%); our results are in accordance with those of other authors.11 Interestingly, we have observed that oesophageal motor impairment may be considered a factor associated with oesophagitis/BE onset; in turn, no SSc patient with normal oesophageal manometry had either grades C/D oesophagitis of BE. Our data, therefore, indicate that oesophageal manometry may be a helpful procedure in identifying high- and low-risk subgroups of SSc patients for oesophagitis/BE, as non-SSc patients with aperistalsis have been found to exhibit a higher rate of reflux episodes than those with preserved peristalsis;41 however, it is questionable whether this group of patients requires systematic endoscopy, which warrants further investigation. Hendel et al.42 have reported that complete healing of oesophagitis was achieved in less than 50% of SSc patients treated for oesophagitis as a result of residual GER. Because we have observed a correlation between more severe motor involvement and oesophagitis on endoscopy, we suggest that patients with severe motor impairment may require higher regimens of PPIs (which may increase acid blockade); however, no definite conclusion can be drawn from our results.

Finally, oesophageal motor disturbances have been implicated as contributing factors of ILD in patients with SSc.3, 43–45 In a previous study of 43 SSc patients, a correlation has been found between the degree of oesophageal manometric motor disturbances and evidence for ILD.3 In the present series, patients with severe oesophageal motor involvement (stage IV), compared with those without (stages I/II/III), more frequently exhibited ILD. Although our study does not offer direct evidence that aspiration occurred, it suggests that GER may be one of the contributing factors of ILD as our SSc patients with severe oesophageal motor dysfunction had higher prevalence of ILD. SSc-related oesophageal impairment may result in repeated micro-aspirations of acid content into the lungs, leading to histological ILD, because LES weakness results in a loss of the prime barrier against GER and absence of peristalsis leads to prolonged GER as a result of impaired oesophageal clearance.44 In this instance, because we have observed a relationship between the degree of oesophageal motor dysfunction and endoscopic mucosal abnormalities, we did not include a 24-h pH measurement as a direct proof of GER in the evaluation of SSc patients. Our findings are in accordance with Tobin et al.44 who observed that patients with biopsy-proved ILD had increased oesophageal acid exposure when compared with control subjects. Nevertheless, the relationship between oesophageal and lung manifestations may have also been because of a concomitant involvement of internal organs in the SSc process, resulting in ILD and fibrosis of oesophageal smooth muscle. However, in our series, we failed to find differences between SSc patients with severe oesophageal motor dysfunction and those without, with respect to median SSc duration, SSc subsets and extrapulmonary systemic complications of SSc. We therefore suggest that the observed correlation between the severity of oesophageal motor disturbances and higher prevalence of ILD may not be an expression of a more advanced SSc in our patients. Our findings underscore the usefulness of oesophageal manometry during evaluation of SSc in objectively defining the degree of oesophageal motor dysfunction. In turn, SSc patients with severe oesophageal impairment may require a closer monitoring of lung parameters (PFT and HRCT-scan). In addition, it is questionable whether a more aggressive therapy of GER in SSc patients with severe oesophageal motor involvement would improve or stop the course of ILD or prevent ILD onset.

Acknowledgement

  1. Top of page
  2. Summary
  3. Introduction
  4. Patients and methods
  5. Methods
  6. Results
  7. Discussion
  8. Acknowledgement
  9. References

The authors thank M. Richard Medeiros for his advice in editing the article.

No external funding was received for this study.

References

  1. Top of page
  2. Summary
  3. Introduction
  4. Patients and methods
  5. Methods
  6. Results
  7. Discussion
  8. Acknowledgement
  9. References
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