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BMJ Case Rep. 2010; 2010: bcr05.2009.1918.
Published online Mar 26, 2010. doi:  10.1136/bcr.05.2009.1918
PMCID: PMC3028029
Rare disease
Obscure gastrointestinal bleeding as first symptom of eosinophilic jejunitis in a liver transplant recipient: diagnosis and treatment with single balloon enteroscopy
Riccardo Urgesi,1,2 Maria Elena Riccioni,1 Enrico C Nista,1 Raffaella Lionetti,3 Giuseppe Tisone,4 Pietro Familiari,1 Riccardo Ricci,5 Giorgio Pelecca,6 Mario Angelico,3 and Guido Costamagna1
1Catholic University, Rome, Endoscopic Unit, Largo A. Gemelli, 8, Roma, 00168, Italy
2Via Oderisi da Gubbio, 182, Roma, 00146, Italy
3Hepatology Unit, Tor Vergata University Rome, Viale Oxford, Rome, 00100, Italy
4Liver Transplantation Centre, Tor Vergata University Rome, Viale Oxford, Rome, 00100, Italy
5Division of Anatomic Pathology and Histology, Catholic University, Rome, Largo A, Gemelli, 8, Rome, 00168, Italy
6Gastroenterology Unit, Viterbo, Strada Sammartinese, Viterbo, 01100, Italy
Correspondence to Riccardo Urgesi, riurgesi/at/tin.it
The small bowel is only partially accessible to traditional endoscopic techniques. The recently introduced push-and-pull enteroscopy technique allows endoscopists to examine the small bowel in its entirety and enables them to take biopsy specimens and administer treatment. We report the case of a liver transplant recipient presenting with obscure gastrointestinal bleeding, whose diagnosis of eosinophilic enteritis was achieved following a single balloon enteroscopy examination. The patient was discharged 3 days after endoscopic treatment. Eosinophilic enteritis is still not a well known disease. The modality of treatment was suggested by our endoscopic experience and not from codified guidelines. The patient’s haemoglobin value was normal 12 months after treatment.
Background
We demonstrate the usefulness of a new procedure, single balloon enteroscopy (SBE), in the diagnosis and treatment of a rare disorder, eosinophilic enteritis, of which fewer than 300 cases have been reported since Kaiser first described it in 1937.
A 61-year-old woman underwent orthotopic liver transplantation 7 years previously for end stage liver disease secondary to hepatitis C virus (HCV) related cirrhosis. She was initially maintained on cyclosporine A. Two years after liver transplantation, because of recurrent hepatitis C and a liver biopsy already showing a staging score of 4 (according to the Ishak classification), she was administered pegylated interferon α2a, 180 μg weekly. Serum HCV RNA became undetectable after 3 months and remained negative for 9 months. At this time, however, treatment was withdrawn because of a pronounced flare up of liver enzymes (up to 700 IU/l). Liver biopsy showed a histological picture suggesting features of both acute and chronic rejection, with eosinophilic and lymphomononuclear infiltrates, granulomatous cholangiolitis and initial ductopenia. The patient was treated with multiple doses of prednisone, which was followed by improvement of serum biochemistry and sudden reappearance of serum HCV RNA. The patient was then started on ribavirin monotherapy, 400 mg/day, which was continued until 6 months ago, and maintenance immunosuppression was changed from cyclosporine A to tacrolimus.
In July 2004 the patient suffered of an episode of upper gastrointestinal (GI) bleeding of unknown origin at endoscopy, despite the presence of medium sized (F2) oesophageal varices. In the following 18 months she developed frequent diarrhoea, weight loss and severe anaemia, with faecal occult blood test repeatedly positive, reaching haemoglobin concentrations as low as 6.9 g/dl (normal range 13.6–17 g/dl), requiring multiple blood transfusions. Serology for cytomegalovirus (CMV) and Epstein–Barr virus (EBV) infections as well as focal culture and parasitological focal examinations were negative. At this stage maintenance immunosuppression was changed from tacrolimus to mycophenolate mofetil. An upper GI endoscopy confirmed the presence of oesophageal varices F2 and of a single sessile polyp in the stomach, 5 mm in diameter, which was removed. A colonoscopy and terminal ileoscopy were negative. Abdominal ultrasonography revealed slightly enlarged ileal walls (3.5 mm). The patient underwent multiple blood transfusions and intravenous infusion of iron and folates, resulting in only a transient improvement of the anaemia. Because of this clinical picture and a further episode of melena, a capsule endoscopy examination was performed in July 2006, which suggested the bleeding source to be in the jejunum; the recording showed, 17 minutes after starting (presumably in the proximal jejunum), a polypoid lesion, not bleeding at the moment of the exploration and, from the 25th to the 88th minute of recording, fresh blood in the absence of an identifiable mucosal lesion. The patient was therefore referred to undergo an SBE (Olympus, Tokyo, Japan), to confirm the diagnosis.
At admission to our hospital, the physical examination was negative and the haemoglobin concentration was 8.5 g/dl. Based on the results of the capsule endoscopy, the patient underwent push-and-pull enteroscopy (PPE) via the oral approach. The instrument consists of a high resolution videoendoscope 2 m long and a soft flexible overtube with one inflatable balloon attached to its distal end. Due to its extreme flexibility, the tip of the endoscope has a broad bending capability, which allows it to anchor to the small bowel wall without a balloon. Gripping the intestinal wall this way, the endoscope can be inserted further without forming redundant loops in the small bowel. In our patient, enteroscopic examination reached about 200 cm from the pylorus. On enteroscopy, gastric and duodenal mucosa appeared normal, while the small bowel had three polypoid lesions located in the jejunum, 5–15 mm in diameter. However, active bleeding was not observed from any of the polyps during the procedure. Only one polyp had ulcerations, which could account for the recent previous GI bleeding, thus requiring endoscopic treatment (fig 1). Treatment was achieved with an epinephrine injection and thermal coagulation with argon plasma coagulation (APC 2, Erbe, 40 W). No other lesions were detected in the length of small bowel that was explored. A biopsy sample taken from the hyperaemic mucosa of the polyps disclosed a pronounced eosinophilic infiltration (fig 2). Other specimens from the gastric antrum and duodenal bulb had no evidence of eosinophilic infiltration.
Figure 1
Figure 1
Endoscopic view of two different jejunal polyps (SBE-Olympus, Tokyo, Japan).
Figure 2
Figure 2
Jejunal mucosa showing intense eosinophilic infiltration (haematoxylin and eosin, (a) 200×; (b) 400×).
Outcome and follow-up
Because of the hepatic disease and of the transplant rejection, the patient was not administered steroid therapy but nonetheless, after polyp fulguration, her haemoglobin increased gradually within the first week, achieving 12 g/dl with no recurrence of bleeding during a 12 month follow-up period.
Small bowel is only partially accessible to traditional endoscopic techniques such as standard oesophagogastroduodenoscopy (OGD), colonoscopy, and push enteroscopy. This is the region where most of the undiagnosed lesions are located. The recently introduced PPE1 allows endoscopists to examine the small bowel in its entirety, enabling them to take biopsy specimens and administer treatment.25 We report the case of a liver transplant recipient presenting with obscure GI bleeding, whose diagnosis of eosinophilic enteritis was achieved following a PPE enteroscopy examination.6 We demonstrate the usefulness of this new procedure as an alternative to standard techniques in the diagnosis and treatment of this rare disorder, fewer than 300 cases of which have been reported.7,8 Two main aspects emerging from the present case report deserve to be discussed: (1) the diagnostic issues related to eosinophilic enteritis and their value in relation to the specific clinical context of liver transplantation; (2) the roles of capsule endoscopy and push enteroscopy in the management of the disease.
With regard to the first point, based on the clinical manifestations and gut wall involvement, three types of eosinophilic enteritis have been defined: mucosal disease, muscle disease, and serosal disease.7 The differential diagnosis requires distinction from Whipple’s disease, amyloidosis, lymphangiectasia,7 and Crohn’s disease.8 When eosinophilic infiltration is profound, resulting in rigid loops, it may mimic lymphoma and small bowel adenocarcinoma.9 According to Talley7 a diagnosis of eosinophilic GI disorder can be made when the following four criteria are fulfilled: (1) presence of GI symptoms; (2) demonstration of eosinophilic infiltration of the GI tract; (3) absence of eosinophilic involvement of other organs; and (4) absence of parasitic infection. All these criteria were fulfilled by the patient described in the present report, with the only exception of a concomitant eosinophilic liver involvement in the setting of a chronic allograft rejection. To our knowledge, the occurrence of eosinophilic enteritis has not yet been described in liver transplant recipients. It may be of significance that our patient received interferon α treatment because of recurrent post-transplant hepatitis C before the development of symptoms, later attributed to eosinophilic enteritis. Notably, eosinophilic enteritis has been already reported only in a single non–transplanted patient receiving interferon α therapy for hepatitis C,10 which suggests, however, that interferon induced immune stimulation may be capable of triggering an eosinophilic reaction. This matter is further complicated in liver transplant recipients by the need for maintenance immunosuppression to control cellular rejection. Our patient had histological evidence of an acute rejection episode (which determined peg-interferon withdrawal), as well as of initial chronic rejection, suggesting that the localised eosinophilic enteritis could have been an epiphenomenon of a more extended, systemic reaction. This hypothesis is reinforced by the fact that GI symptoms (diarrhoea and bleeding) worsened after May 2005, when the patient was switched from tacrolimus to mycophenolate treatment—in other words, when she was moved to a lower degree of maintenance immunosuppression. This case report therefore suggests that the development of unexplained anaemia and occult or overt GI bleeding of unknown origin in a under-immunosuppressed liver transplant recipient may indicate the presence of jejunal eosinophilic enteritis.
With regard to the second point, this case report emphasises the value of capsule endoscopy and of push enteroscopy in the diagnostic work-up and clinical management of this rare condition. In fact, radiographic changes associated with eosinophilic enteritis are variable and usually non-specific.7,9 Capsule endoscopy, a non-invasive modality used to evaluate disorders of the small bowel, has already been used to evaluate intestinal complications caused by eosinophilic enteritis,11 yet its use is limited due to the inability to obtain biopsy specimens. PPE allows specimen withdrawal, but it only spans the proximal jejunum.12 Newly introduced double balloon enteroscopy (DBE)14,13 and SBE display higher diagnostic yield and greater therapeutic possibilities compared to other diagnostic modalities. In the present case, SBE proved to be a useful technique for histological confirmation of the diagnosis, with no associated complications. A prompt diagnosis of this disorder avoids unnecessary laparoscopy and unnecessary treatments. To our knowledge, this is the first case of SBE diagnosis and treatment of eosinophilic enteritis presenting with bleeding arising from polypoid lesions of the small bowel.
In conclusion, eosinophilic enteritis is a rare disorder and can be difficult to diagnose, especially when accompanied with intestinal bleeding. We found SBE a useful tool for the timely and prompt diagnosis and treatment of this disorder.
Learning points
  • Small bowel is only partially accessible to traditional endoscopic techniques.
  • The recently introduced push-and-pull enteroscopy allows endoscopists to examine the small bowel in its entirety, and enables them to take biopsy specimens and administer treatment.
  • We demonstrate the usefulness of this new procedure in the diagnosis and treatment of a rare disorder, eosinophilic enteritis.
Footnotes
Competing interests: None.
Patient consent: Patient/guardian consent was obtained for publication.
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