Raising Awareness about Chronic Intestinal Pseudo-Obstruction (CIPO): A Case Report Showing CIPO as Initial Manifestation of Atypical Seronegative Systemic Sclerosis

Abstract

Only few case studies address pseudo-obstruction, a disorder - which often frustrates clinicians and patients due to an unclear diagnosis and limited therapeutic options. Thus, the aim of this paper is to investigate a relevant case concerning a patient presenting with symptoms of acquired chronic intestinal pseudo-obstruction (CIPO). After one year of extensive diagnostic tests and unsuccessful treatment with prokinetics, the patient underwent a subtotal ileocolectomy. The histology of the intestinal specimen revealed continuous atrophy and fibrosis mainly within the circular, inner muscle layer of muscularis propria of the ileum and colon. Even though serum markers were lacking, a subsequent skin biopsy showed signs of scleroderma supporting an initial diagnosis of intestinal involvement in systemic sclerosis. Despite treatment with steroids and methotrexate, the increasingly emaciated patient died. In conclusion, there is a bias against the publishing of pseudo-obstruction studies, in particular, due to the obscure underlying causes. To raise awareness of this problem, we call for clinicians to systematically generate comprehensive data about patients presenting these symptoms.

Chronic Idiopathic Intestinal Pseudo-Obstruction: A Working Diagnosis

“…4 Octreotide has been used with much success in CIP secondary to scleroderma. Gastrointestinal transit studies have demonstrated that, while octreotide has been proven to slow intestinal transit by inhibiting intermittent, low-amplitude contractions, it enhances short burst, high amplitude contractions.…”

Chronic intestinal pseudo‐obstruction in adults: A practical guide to identify patient subgroups that are suitable for more specific treatments

“…56 Systemic sclerosis is suggested by skin involvement, Raynaud's phenomenon, microvascular abnormalities on capillaroscopy, and the presence of anti-topoisomerase I (SCL 70) antibodies. 57,58 TA B L E 2 Tests and investigations according to the suspected diagnosis in adult patients presenting with chronic intestinal pseudoobstruction (CIPO).…”

“…Systemic sclerosis is suggested by skin involvement, Raynaud's phenomenon, microvascular abnormalities on capillaroscopy, and the presence of anti‐topoisomerase I (SCL 70) antibodies 57,58 …”

Could Chronic Idiopatic Intestinal Pseudo-Obstruction Be Related to Viral Infections?

“…In fact, the extrinsic autonomic nervous system can be affected both centrally (for example, Parkinson's disease, vascular stroke, encephalitis, neoplasms and diseases affecting the encephalic autonomous centers) and peripherally (for example, diabetic neuropathy, or further neuropathies involving the enteric nervous system such as Hirschsprung Disease and Chagas Disease) [2,9]. This also includes paraneoplastic syndromes [10][11][12], immune-mediated [13,14] and collagen diseases, and viral infections. Furthermore, radiation enteritis, collagenosis, jejunal diverticulosis, and Ehlers-Danlos syndrome could cause neuronal and myogenic dysfunction [2].…”