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.

Zusammenfassung

Die chronische intestinale Pseudoobstruktion stellt sich oft als differenzialdiagnostisch schwierig einzuordnende und therapeutisch schlecht zu behandelnde Erkrankung dar. In diesem Fallbericht wird ein Patient mit erworbener schwerer intestinaler Pseudoobstruktion vorgestellt, bei welchem trotz umfangreicher Untersuchungen und probatorischer Prokinetikatherapie keine Besserung erreicht werden konnte, sodass letztlich eine subtotale Ileokolektomie durchgeführt wurde. Histologisch zeigte sich eine ausgedehnte Atrophie und Fibrose der Muscularis propria. Obwohl Serummarker für die Erkrankung wiederholt unauffällig waren, unterstützte eine Hautbiopsie die Diagnose einer Sklerodermie mit vorrangig intestinaler Beteiligung. Weder Steroide noch Methotrexat führten zu einer Besserung der ausgeprägten Symptomatik und trotz hochkalorischer parenteraler Ernährung kam es zu einer schweren Kachexie und schließlich zum Tod des Patienten. Aufgrund des häufig fehlenden Nachweises einer eindeutigen Krankheitsursache und schwieriger differenzialdiagnostischer Erwägungen ist von einem Mangel an Fallberichten auszugehen. Zum besseren Verständnis der chronischen Pseudoobstruktion und der zugrunde liegenden Ursachen ist eine umfängliche Erfassung dieser Fälle wünschenswert.

Key words

malassimilation - pseudo-obstruction - sclerodermia - systemic sclerosis - CIPO - chronic intestinal pseudo-obstruction

Schlüsselwörter

Malassimilation - Pseudoobstruktion - Sklerodermie - systemische Sklerose - CIPO - chronische intestinale Pseudoobstruktion

• References

• 1 Sjogren RW. Gastrointestinal motility disorders in scleroderma. Arthritis Rheum 1994; 37: 1265-1282
• 2 Connor FL, Di Lorenzo C. Chronic intestinal pseudo-obstruction: assessment and management. Gastroenterology 2006; 130 (02) S29-S36
• 3 Sutton DH, Harrell SP, Wo JM. Diagnosis and management of adult patients with chronic intestinal pseudoobstruction. Nutr Clin Pract 2006; 211: 16-22
• 4 Füger K, Barnert J, Höpfner W et al. Intestinal pseudoobstruction as a feature of myotonic muscular dystrophy. Z Gastroenterol 1995; 33: 534-538
• 5 Perez-Atayde AR, Fox V, Teitelbaum JE et al. Mitochondrial neurogastrointestinal encephalomyopathy: diagnosis by rectal biopsy. Am J Surg Pathol 1998; 22: 1141-1147
• 6 Cuillerier E, Coffin B, Potet F et al. Paraneoplastic intestinal pseudo-obstruction revealing small cell lung carcinoma: “the anti-Hu syndrome”. Gastroenterol Clin Biol 1998; 22: 346-348
• 7 Deguchi M, Shiraki K, Okano H et al. Primary localized amyloidosis of the small intestine presenting as an intestinal pseudo-obstruction: report of a case. Surg Today 2001; 31: 1091-1093
• 8 de Giorgio R, Volta U, Stanghellini V et al. Neurogenic chronic intestinal pseudo-obstruction: antineuronal antibody-mediated activation of autophagy via Fas. Gastroenterology 2008; 135: 601-609
• 9 Teixeira ARL, Nascimento RJ, Sturm NR. Evolution and pathology in chagas disease – a review. Mem Inst Oswaldo Cruz 2006; 101: 463-491
• 10 Cappell MS, Simon T. Colonic toxicity of administered medications and chemicals. Am J Gastroenterol 1993; 88: 1684-1699
• 11 Ebert EC. Gastric and enteric involvement in progressive systemic sclerosis. J Clin Gastroenterol 2008; 42: 5-12
• 12 Cacoub P, Benhamou Y, Barbet P et al. Systemic lupus erythematosus and chronic intestinal pseudoobstruction. J Rheumatol 1993; 20: 377-381
• 13 Yamazaki-Nakashimada MA, Rodriguez-Jurado R, Ortega-Salgado A et al. Intestinal pseudoobstruction associated with eosinophilic enteritis as the initial presentation of systemic lupus erythematosus in children. J Pediatr Gastroenterol Nutr 2009; 48: 482-486
• 14 Moore SW, Schneider JW, Kaschula ROC. Unusual variations of gastrointestinal smooth muscle abnormalities associated with chronic intestinal pseudo-obstruction. Pediatr Surg Int 2002; 18: 13-20
• 15 Durai R. Colonic pseudo-obstruction. Singapore Med J 2009; 50: 237-244
• 16 Sjogren RW. Gastrointestinal features of scleroderma. Curr Opin Rheumatol 1996; 8: 569-575
• 17 Parkman HP, Fass R, Foxx-Orenstein AE. Treatment of patients with diabetic gastroparesis. Gastroenterol Hepatol 2010; 6: 1-16
• 18 Fynne L, Worsøe J, Gregersen T et al. Gastrointestinal transit in patients with systemic sclerosis. Scand J Gastroenterol 2011; 46: 1187-1193
• 19 Schmeiser T, Saar P, Jin D et al. Profile of gastrointestinal involvement in patients with systemic sclerosis. Rheumatol Int 2011; 19 (Epub ahead of print)
• 20 Reinhart WH. From starving to life-threatening nutrition – the history of an intestinal pseudoobstruction. Ther Umsch 2006; 63: 757-761
• 21 Muñoz-Yagüe MT, Solís-Muñoz P, Salces I et al. Chronic intestinal pseudo-obstruction: a diagnosis to be considered. Rev Esp Enferm Dig 2009; 101: 336-342