Objective <p>Gastrointestinal involvement is almost universal in patients with systemic sclerosis (SSc). Enteric dysmotility, at its most severe, can present with pseudo-obstruction. In this study, we aimed to quantify the prevalence of SSc pseudo-obstruction and evaluate risk factors for its development. In a subgroup of patients requiring admission to hospital for acute episodes of pseudo-obstruction, we evaluated the clinical course and treatments administered.</p> Methods <p>Using data from 1969 Australian Scleroderma Cohort Study (ASCS) participants, we performed multivariable logistic regression analysis to identify SSc-specific risk factors for pseudo-obstruction. Descriptive statistics were used to examine the clinical course of patients admitted with pseudo-obstruction at a single ASCS centre.</p> Results <p>Pseudo-obstruction occurred uncommonly, affecting 70 (3.56%) ASCS participants. Records of 14 participants with a total of 39 admissions for acute pseudo-obstruction were identified. Pseudo-obstruction was associated with longer disease duration (OR 1.03, <i>p</i> = 0.03), bowel dysmotility (OR 4.51, <i>p</i> &lt; 0.01), small intestinal bacterial overgrowth (OR 2.81, 95% CI (1.00–1.05), <i>p</i> &lt; 0.01), and gastric antral vascular ectasia (OR 2.52, 95% CI 1.28–4.94, <i>p</i> &lt; 0.01). Severe diarrhoea, as measured by the UCLA Gastrointestinal 2.0 questionnaire, was the only clinical symptom significantly associated with episodes of pseudo-obstruction (OR 3.39, 95% CI 1.56–7.38, <i>p</i> &lt; 0.01). Opioids were more commonly prescribed in patients with pseudo-obstruction but were not significantly associated with pseudo-obstruction in multivariable analysis (OR 1.24, 95% CI 0.62–2.48, <i>p</i> = 0.54). Patients with a history of pseudo-obstruction were more likely to require enteral (4.29% vs. 0.21%, <i>p</i> &lt; 0.01) or parenteral nutrition (7.14% vs. 0.16%, <i>p</i> &lt; 0.01).</p> Conclusion <p>Pseudo-obstruction is associated with other severe gastrointestinal manifestations and is associated with malnutrition in SSc patients. Future studies are required to assess the impact of treatment of SSc-associated enteric dysmotility to prevent progression to pseudo-obstruction.</p> <p><Table Float="No" ID="Taba"> <tgroup cols="2"> <colspec align="left" colname="c1" colnum="1" /> <colspec align="left" colname="c2" colnum="2" /> <tbody> <row> <entry align="left" nameend="c2" namest="c1"> <p>Key Points</p> <p>• Pseudo-obstruction is an uncommon manifestation of systemic sclerosis but frequently recurs and is associated with increased mortality.</p> <p>• Severe diarrhoea and long disease duration are associated with an increased risk of pseudo-obstruction.</p> <p>• Pseudo-obstruction occurs more commonly in patients with severe enteric dysmotility and gastric antral vascular ectasia (GAVE).</p> </entry> </row> </tbody> </tgroup> </Table></p>

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Prevalence, clinical characteristics, and hospital course of systemic sclerosis-associated pseudo-obstruction

  • Laura Ross,
  • Lyman Lin,
  • Dylan Hansen,
  • Alannah Quinlivan,
  • Wendy Stevens,
  • Susanna Proudman,
  • Jennifer Walker,
  • Joanne Sahhar,
  • Gene-Siew Ngian,
  • Lauren Host,
  • Mandana Nikpour,
  • Chamara Basnayake

摘要

Objective

Gastrointestinal involvement is almost universal in patients with systemic sclerosis (SSc). Enteric dysmotility, at its most severe, can present with pseudo-obstruction. In this study, we aimed to quantify the prevalence of SSc pseudo-obstruction and evaluate risk factors for its development. In a subgroup of patients requiring admission to hospital for acute episodes of pseudo-obstruction, we evaluated the clinical course and treatments administered.

Methods

Using data from 1969 Australian Scleroderma Cohort Study (ASCS) participants, we performed multivariable logistic regression analysis to identify SSc-specific risk factors for pseudo-obstruction. Descriptive statistics were used to examine the clinical course of patients admitted with pseudo-obstruction at a single ASCS centre.

Results

Pseudo-obstruction occurred uncommonly, affecting 70 (3.56%) ASCS participants. Records of 14 participants with a total of 39 admissions for acute pseudo-obstruction were identified. Pseudo-obstruction was associated with longer disease duration (OR 1.03, p = 0.03), bowel dysmotility (OR 4.51, p < 0.01), small intestinal bacterial overgrowth (OR 2.81, 95% CI (1.00–1.05), p < 0.01), and gastric antral vascular ectasia (OR 2.52, 95% CI 1.28–4.94, p < 0.01). Severe diarrhoea, as measured by the UCLA Gastrointestinal 2.0 questionnaire, was the only clinical symptom significantly associated with episodes of pseudo-obstruction (OR 3.39, 95% CI 1.56–7.38, p < 0.01). Opioids were more commonly prescribed in patients with pseudo-obstruction but were not significantly associated with pseudo-obstruction in multivariable analysis (OR 1.24, 95% CI 0.62–2.48, p = 0.54). Patients with a history of pseudo-obstruction were more likely to require enteral (4.29% vs. 0.21%, p < 0.01) or parenteral nutrition (7.14% vs. 0.16%, p < 0.01).

Conclusion

Pseudo-obstruction is associated with other severe gastrointestinal manifestations and is associated with malnutrition in SSc patients. Future studies are required to assess the impact of treatment of SSc-associated enteric dysmotility to prevent progression to pseudo-obstruction.

Key Points

• Pseudo-obstruction is an uncommon manifestation of systemic sclerosis but frequently recurs and is associated with increased mortality.

• Severe diarrhoea and long disease duration are associated with an increased risk of pseudo-obstruction.

• Pseudo-obstruction occurs more commonly in patients with severe enteric dysmotility and gastric antral vascular ectasia (GAVE).