|Year : 2022 | Volume
| Issue : 4 | Page : 461-465
Eosinophilic gastroenteritis: A case series of a rare disease from an apex Tertiary Care Center in South India
Sandheep Janardhanan1, Allwin James1, Alagammai Palaniappan2, Jibin Lukose3, Mohan Narasimhan1, Ramesh Ardhanari4
1 Department of Medical and Surgical Gastroenterology, Meenakshi Mission Hospital and Research Centre, Madurai, Tamil Nadu, India
2 Department of Medical Gastroenterology, Meenakshi Mission Hospital and Research Centre, Madurai, Tamil Nadu, India
3 Department of Pathology, SCMS School of Science and Technology, Kochi, Kerala, India
4 Department of Gastroenterology and Medical, Meenakshi Mission Hospital and Research Centre, Madurai, Tamil Nadu, India
|Date of Submission||19-Jun-2021|
|Date of Decision||02-Dec-2021|
|Date of Acceptance||08-Apr-2022|
|Date of Web Publication||16-Nov-2022|
Meenakshi Mission Hospital and Research Centre, Melur Road, Madurai - 625 107, Tamil Nadu
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Eosinophilic gastroenteritis (EGE) is a rare disease with a myriad of presentations. In this case series of four patients from South India, we describe three classical manifestations of the disease (mucosal, muscular, and serosal). Two of them had obstructive jaundice as a presenting complaint due to duodenal obstruction, whereas one had massive upper gastrointestinal bleed. There are very few case series regarding this disease from India. Its presentation as hemetemesis and obstructive jaundice is also very rare,with only few such case reports reported till now.
| Abstract in French|| |
La gastro-entérite éosinophile (EGE) est une maladie rare avec une myriade de présentations. Dans ce cas, une série de quatre patients du sud de l'Inde, nous décrivons trois manifestations classiques de la maladie (muqueuse, musculaire et séreuse). Deux d'entre eux avaient une ictère obstructive comme plainte présentant en raison d'une obstruction duodénale, tandis que l'on avait un saignement gastro-intestinal supérieur massif. Il y a très peu de séries de cas concernant cette maladie de l'Inde. Sa présentation d'hématemèse et de jaunisse obstructive est également très rare, avec seulement quelques rapports de cas signalés jusqu'à présent.
Mots-clés: Atopie, examen de la moelle osseuse, gastro-entérite eosinophile
Keywords: Atopy, bone marrow examination, eosinophilic gastroenteritis
|How to cite this article:|
Janardhanan S, James A, Palaniappan A, Lukose J, Narasimhan M, Ardhanari R. Eosinophilic gastroenteritis: A case series of a rare disease from an apex Tertiary Care Center in South India. Ann Afr Med 2022;21:461-5
|How to cite this URL:|
Janardhanan S, James A, Palaniappan A, Lukose J, Narasimhan M, Ardhanari R. Eosinophilic gastroenteritis: A case series of a rare disease from an apex Tertiary Care Center in South India. Ann Afr Med [serial online] 2022 [cited 2022 Dec 7];21:461-5. Available from: https://www.annalsafrmed.org/text.asp?2022/21/4/461/361250
| Introduction|| |
Primary eosinophilic gastroenteritis refers to a rare group of diseases of the gastrointestinal (GI) tract, causing eosinophil infiltration of the bowel wall in the absence of other etiologies such as drugs, parasitic infestations, and neoplasms. The most common of these is eosinophilic gastroenteritis (EGE), first described by Kaijser in 1937. Klein described three variants of EGE with different clinical presentations, depending on the site of eosinophilic infiltration, namely mucosal, muscular, and serosal types. Mucosal type (40%–70%) presents with abdominal pain, vomiting, and diarrhea. Muscular EGE (10%–30%) causes signs and symptoms suggestive of luminal GI obstruction, whereas serosal, the rarest (10%–15%), causes eosinophilic ascites. In this case series from Meenakshi Mission Hospital and Research Centre, an apex tertiary care hospital and center of excellence in gastroenterology in Madurai, South India, we describe four different types and manifestations of EGE.
| Case Reports|| |
A 34-year-old male presented to us with colicky epigastric pain, nonbilious vomiting, and loose stools, associated with weight loss of more than 5 kg over 3 months. There was no history of fever, cough, atopy, or drug intake. General and systemic examination was normal except for mild epigastric tenderness. His baseline parameters showed persistent peripheral eosinophilia high eosinophils in differential count and peripheral smear, high absolute eosinophil count (AEC)-3798/mm3 (normal, <500/mm3), and high serum immunoglobulin IgE levels (1160 IU/ml) (normal, 150–300 IU/ml). Stool tests for parasites were negative for ova, cyst, and larvae. After admission, he was treated symptomatically with adequate hydration, proton-pump inhibitors, and antiemetics. Abdominal imaging revealed GI wall thickening on ultrasonogram (USG), confirmed with a contrast-enhanced computed tomography (CECT) abdomen. We proceeded with an upper GI scopy (EGD), which showed pangastritis and duodenitis [Figure 1]. Histopathological examination of biopsies showed high eosinophilic count in stomach mucosa, duodenum, and jejunum (30–35/high-power field [HPF]) [Figure 2] and [Figure 3], while colonoscopic biopsies were normal. Bone marrow biopsy showed hypercellularity with eosinophilia (25%). Cytogenetic analysis such as fluorescent in situ hybridization for platelet-derived growth factor-alpha, platelet-derived growth factor-beta, fibroblast growth factor receptor; Janus kinase 2 mutation and Philadelphia chromosome studies were negative. A six-food elimination diet (SFED) which is the most frequently employed dietary therapy in patients with EGE was tried (typically, trials the exclusion of wheat, milk, egg, nuts, soy, fish, and shellfish). Moreover, he was initiated on 1 mg/kg prednisolone for 4 weeks and tapered over 2 months, along with antihistaminics and supportive medications. His symptoms got alleviated and AEC became normal (400/mm3). He was continuously followed up for a period of 2 years and is still asymptomatic on his last visit.
|Figure 1: Esophagogastroduodenoscopy (EGD) showing gastritis in case No. 1|
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|Figure 2: High-power view of duodenum (×40) showing eosinophilic infiltration-in case No. 1|
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|Figure 3: High-power view (×40) of stomach mucosa showing eosniophilic infiltration in case No. 4|
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A 71-year-old male presented with insidious onset of abdominal pain, vomiting, and yellowish discoloration of urine and eyes associated with cholestatic symptoms and loss of weight and appetite of 3-month duration. He had intrahepatic biliary radicle and common bile duct dilatation. On ultrasonography and was referred as a case of periampullary malignancy. General and systemic examination was within normal limits, except for mild icterus. Baseline investigations showed normal counts with moderate eosinophilia (AEC-970/mm3). Liver function tests showed hyperbilirubinemia (bilirubin total 4.7 direct-2.4); alanine transaminase, 412 IU/L (normal range, 5–40 IU/L); alkaline phosphatase, 178 IU/L (normal range, 30–115 IU/L); and albumin, 3.4 g/dL (normal range, 3.0–3.8 g/dL). Viral hepatitis screening (including hepatitis B and C) was negative. Chest and abdominal X-rays were normal. Abdominal computed tomography revealed an ampullary growth. Endoscopic ultrasonography revealed that the common bile and pancreatic ducts were dilated [Figure 4]. Upper GI endoscopy done using a duodenoscope showed edematous gastroduodenal mucosa [Figure 5], and gastric and ampullary biopsies were taken. Histology showed eosinophilic infiltration of the mucosa and submucosa. He was started on SFED and tapering dose of oral prednisolone (40 mg/d). He had marked clinical improvement within a week [Figure 6]. He became asymptomatic,LFTs stabilized and eosinophil count stabilized(4%),over a three week period. He was discharged on a tapering steroid cover over a period of 3 months. He was asymptomatic for long time and was regularly followed up in outpatient department, till 1 month before, when he had the same presentation and after stabilization and documenting tissue eosinophilia, he was initiated on steroid therapy. He is now on maintenance dosage of prednisolone.
|Figure 4: Endoscopic ultrasound of ampullary lesion showing ampullary thickening in case No. 2|
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|Figure 5: Duodenoscopic image showing ampullary lesion pretreatment in case No. 2|
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|Figure 6: Duodenoscopic image showing normal ampulla posttreatment in case No. 2|
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A 47-year-old male, who had a history of occasional mild asthma treated with a short-acting bronchodilator and inhaled corticosteroids experienced nausea, and lassitude of 3-week duration, with abdominal fullness and weight gain of around 5 kg. He was treated by his family physician and after detection of ascites on imaging was treated with paracentesis, diuretics, and supportive measures, which did not yield much results. There was no history of transfusions, atopies, rash, food allergies, or any drugs. There was no history of liver, heart, kidney, or thyroid disease. He had massive ascites without any hepatosplenomegaly or lymphadenopathy. Laboratory data showed a normocytic normochromic blood picture with severe eosinophilia (50% eosinophils and 20,100 AEC). Repeated negative stool studies were normal and did not show any parasites. On abdominal USG, the liver was found to be of normal size and echogenicity. There was abdominal ascites. Diagnostic paracentesis showed fluid with protein level of 4.7 g/dL, albumin of 2.5 g/dL, and white blood cell of 1600/mL with significant eosinophilia (72%). Ascitic fluid analysis for bacterial culture, malignant cytology, and polymerase chain reaction for tuberculosis were negative. Bone marrow biopsy showed normal hematopoiesis with significant eosinophilia (20%–25%) and normal cytogenetics. EGD showed significant gastric mucosal edema. He was empirically treated with SFED, prednisolone 40 mg once daily, and tapered off over 3 months, with rapid resolution of eosinophilia and symptoms on follow-up 3 months later (AEC, 289/mm3). However, he did have two more bouts of the same presentation later during the course after weaning off steroids over the last 3 years and hence had to be put on maintenance low-dose steroids and is now on periodic follow-up.
An 8-year-old female presented with abdominal pain and vomiting for 3 days, followed by hematemesis and melena of 1-day duration. There was no history of peptic ulcer disease, nonsteroid anti-inflammatory drugs intake, liver disease, drug intake, atopy, or food allergies. Blood investigations revealed eosinophilic leukocytosis with moderate eosinophilia (24%, AEC 4200/mm3). A CECT abdomen showed ileal and jejunal thickening and interloop ascites. Endoscopies revealed erythematous gastroduodenitis and mild terminal ileitis, and segmental biopsies were positive for EGE [Figure 7]. Ascitic fluid analysis showed low serum ascitic albumin gradient high protein ascites, with an eosinophilic infiltrate (70%). A bone marrow aspiration and biopsy was performed, the bone marrow biopsy showed normocellular picture with raised eosinophils, and the molecular cytogenetics study was negative. She was diagnosed to have EGE and treated with SFED, tapering dose of steroids and antihistaminics, and supportive measures. She became completely asymptomatic, with a normal eosinophil count (AEC, 231) on follow-up. Later, she developed similar symptoms after 1 year, had to be put on low-dose maintenance dose of steroids, and was asymptomatic till last visit three months ago.
|Figure 7: Esophagogastroduodenoscopy (EGD) showing duodenitis in case No. 4|
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| Discussion|| |
EGE is an uncommon disease of GI tract with varying symptoms depending on the site of involvement. In the first case, there were eosinophilic infiltrates in the proximal small bowel mucosa (mucosal type), whereas the second had duodenal ampullary wall eosinophilic infiltration, causing biliary obstruction (muscular type) and mimicked periampullary malignancy. The third one was a pure serosal type without any mucosal involvement causing refractory ascites, whereas the fourth one had features suggestive of mucosal and serosal involvement. Although the cause of ascites could be multifactorial, we could more or less definitely conclude that EGE was the cause in both ascites cases. This could be explained by the fact that ascitic fluid analysis of both ascites cases showed significantly high eosinophil levels, which is specific for EGE. Furthermore, other causes such as protein-losing enteropathy could be ruled out because of repeated stool tests and negative mucosal biopsies. The diagnosis of mucosal EG is typically confirmed by endoscopic biopsies, which reveal ≥20–25 eosinophils per HPF on microscopic examination.
A unique World Wide Web database for eosinophilic GI disorders reveals that EGE is strongly associated with atopy in 80% of cases; however, such a history was absent in all but one case. Drugs such as azathioprine, gemfibrozil, and carbamazepine can cause eosinophilic infiltration and such a history was negative. Helicobacter pylori infestation was absent, ruling out another cause of eosinophilia. Intestinal parasitic infestation is also associated with eosinophilia; however, repeated sampling for parasitological stool examination was negative, and serial mucosal biopsies showed absent parasites. Hematological causes of eosinophilia were ruled out after bone marrow sampling by an expert hematologist. Although a thorough allergic history was taken in all of our cases and extrinsic allergens were excluded, we did not do any allergy testing. This was because of the logistical difficulties, and current guidelines do not warranty, conducting such a test. A review of the literature on treatment for EGE showed that the first step is a dietary restriction as an association was evident between food allergy and EGE. However, the results of elimination diets are often poor. The next step is oral steroids, which are the mainstay of treatment. Oral prednisolone may be used at the onset at a dosage of 20–40 mg/day for 6–8 weeks with around 90% response. Budesonide has been used in some select cases, with reasonably good efficacy and less systemic toxicity, even though it is not widely used as prednisolone. Mast cell stabilizers and drugs such as montelukast, an antagonist of the leukotriene receptor, are also useful as steroid-sparing agents. Few studies showed the effectiveness of anti-interleukin monoclonal antibody mepolizumab.
Venkataraman et al. described the first case series of EGE in India. The serosal form, though rare, can cause refractory ascites and can be fatal due to hypereosinophilia as described by Agah et al. Our case series has some unique features. Only handful of obstructive jaundice cases have been caused by EGE and ours was one. Most of them had underwent needless invasive procedures, before realizing the root cause of the problem, Amita et al. and Shetty and Shetty, have published case series about obstructive presentations of EGE in this regard., The resemblance of muscular EGE to malignancy is so notorious that a term tumefactive eosinophilia of the GI tract” was coined. The incidence of upper GI bleed is also an exceedingly rare phenomenon. Chen et al. described a case, in which an endoscopic biopsy leads to exacerbation of bleed causing a hematoma and refractory bleed.
To conclude, EGE is a rare disease, which can be deceptively confused with other pathologies and requires a high index of suspicion and meticulous workup to diagnose and treat. The need for follow-up and maintenance medications is high as relapses are quite common.
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Conflicts of interest
There are no conflicts of interest.
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