Abstract
Chronic intestinal pseudo-obstruction is a severe complication of systemic sclerosis. Inflammatory neuropathy and immunological alterations have a prominent role in the development of systemic sclerosis–related chronic intestinal pseudo-obstruction and immunomodulation might be beneficial in this context. An accidental observation of a patient with juvenile arthritis and a biopsy-proven diagnosis of autoimmune ganglionitis led us to experiment with a new approach to treat systemic sclerosis–related chronic intestinal pseudo-obstruction. In our arthritis patient, the severity and frequency of recurrent episodes of chronic intestinal pseudo-obstruction and aspiration pneumonia were reduced whenever steroids were used to treat arthritic flares, which dramatically improved with abatacept therapy. A systemic sclerosis patient presented typical chronic intestinal pseudo-obstruction features that were neither controlled by dietary interventions nor by prokinetics and were often complicated by acute episodes (5-year) requiring hospitalization. Increased food tolerance was observed whenever parenteral steroids were used during hospitalization. An adequate long-term control of symptoms was then obtained with the use of intramuscular methylprednisolone 20 mg/day; however, symptoms promptly recurred after tapering. Following this motivating example, immunomodulation with abatacept was started. Symptoms were then well controlled and steroids could be weaned off without further acute episodes of sub-occlusion. We postulate that inflammatory neuropathy resembling myenteric ganglionitis may be suspected in selected systemic sclerosis patients with chronic intestinal pseudo-obstruction features. Immunomodulation with drugs that act on T function and restore the regulatory/effector T cell balance may be beneficial in these subjects. The outcomes of four additional systemic sclerosis patients with severe and refractory symptoms of intestinal pseudo-obstruction successfully treated with abatacept are also presented.
Highlights
Chronic intestinal pseudo-obstruction (CIPO) is a life-threatening syndrome characterized by signs and symptoms of intestinal obstruction without evidence of mechanical lesions of the intestinal lumen.[1]
The pathophysiology of Systemic sclerosis (SSc)-related intestinal involvement is poorly understood, yet immunological aspects, as highlighted by the occurrence of alterations ranging from the presence of inflammatory infiltrates to the positivity to myenteric neuronal antibodies,[2,4] seem to play a prominent role in the development of this complication
Visceral neuropathy with inflammatory features is a cardinal feature of enteric dysmotility syndromes and of CIPO.[6]
Summary
Chronic intestinal pseudo-obstruction (CIPO) is a life-threatening syndrome characterized by signs and symptoms of intestinal obstruction without evidence of mechanical lesions of the intestinal lumen.[1]. SSc-related enteric involvement is usually severe and resistant to therapy; prokinetic drugs and dietary modification may be effective in patients with mild to moderate symptoms,[5] while the management of nonresponsive cases complicated by pseudo-obstruction is often challenging.[2] We report the case of an SSc patient with CIPO refractory to prokinetic and supportive measures that was successfully treated with abatacept (ABA).
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
