Short Report
Acute eosinophilic ascites: An unusual form of an unusual case
Tropical Doctor 2015, Vol. 45(1) 39–41 ! The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0049475514553829 tdo.sagepub.com
Parul Kodan1, Meenakshi A Shetty2, MR Pavan3, Ahalya Kariappa4 and Chakrapani Mahabala5
Abstract Eosinophilic gastroenteritis (EGE) is an uncommon disease characterised by eosinophilic infiltration in the gastrointestinal tract. EGE may involve more than one layer of the gastrointestinal tract. Clinical features depend on the layer and location which is involved. We report an unusual case of eosinophilic ascites associated with antinuclear antibody positivity, which is an unusual variety of serosal form of EGE.
Keywords Eosinophilic gastroenteritis, ascites, ANA
Introduction Eosinophilic ascites (EA) is probably the most unusual and rare presentation of eosinophilic gastroenteritis (EGE) which is characterised by eosinophilic infiltration of any or all layers of the gut wall and may involve any segment of the gastrointestinal tract in the absence of known causes for eosinophilia. Peripheral eosinophilia may or may not be present. We report an unusual case of EA which is an unusual variety of the serosal form of EGE. We also found the patient to have antinuclear antibody (ANA) titer strongly positive, making this association all the more fascinating. The rarity of the case, its unusual rare associations and excellent response of our young patient to treatment, prompted us to report this case.
Case report A previously healthy 18-year-old young man presented with complaints of chronic diffuse severe abdominal pain, recurrent vomiting, loose stool and progressively increasing abdominal distension for the last 6 months. He had severe loss of appetite followed by significant weight loss. There was no history of atopy, allergy, food allergy or asthma in the past. None of his family members had similar abdominal symptoms. Several treatment modalities including antibiotics, antispasmodics, analgesics, antiemetic could not relieve his symptoms. The patient was referred to our centre in view of his chronic debilitating condition. On physical
examination, he was pale and malnourished but had no lymphadenopathy and pedal oedema. The abdomen was distended and tender diffusely with shifting dullness present. Other systemic examination was unremarkable. Haematological tests revealed mild anaemia and a peripheral smear showed peripheral eosinophilia. The Mantoux test was found to be negative. On abdominal ultrasonography, the liver was found to be of normal size and echogenicity, and all vessels were patent. There was a large amount of pelvic and abdominal ascites. Analysis of this fluid showed a low serum albumin-ascitic gradient (SAAG), 55% eosinophils and 45% lymphocytes. Standard bacterial and tuberculosis culture showed no growth. Parasitic infestation was ruled out by repeated negative stool examinations. HIV enzyme-linked immunosorbent 1 Resident, Department of Medicine, Kasturba Medical College, Manipal University, Mangalore, India 2 Associate Professor, Department Of Medicine, Kasturba Medical College, Manipal University, Mangalore, India 3 Associate Professor, Department Of Medicine, Kasturba Medical College, Manipal University, Mangalore, India 4 Senior Resident, Department Of Medicine, Kasturba Medical College, Manipal University, Mangalore, India 5 Professor, Department Of Medicine, Kasturba Medical College, Manipal University, Mangalore, India
Corresponding author: Chakrapani Mahabala, Department of Medicine, Kasturba Medical College, Mangalore, Manipal University, Light House Hill Road, Mangalore 575001, India. Email: [email protected]
Downloaded from tdo.sagepub.com at Bobst Library, New York University on April 18, 2016
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assay (ELISA) was negative. Bone marrow biopsy showed normal haematopoiesis with mild eosinophilia. He underwent oesophagogastroduodenoscopy, colonoscopy and diagnostic laparoscopy with biopsies all proving normal. ANA was found to be positive in a ratio of 1:100 with a pattern corresponding to antiSSA, B. No symptoms of joint pain, rash, proteinuria, mouth dryness or any feature corresponding to history of connective tissue disorders was present. Thus a diagnosis of serosal EGE with ANA positivity was made and he was started on a low dose steroid regimen, to which he responded well with symptoms resolving within 1 week. He was discharged on low dose steroid therapy with a tapering dose. On regular review, he remains asymptomatic.
Discussion EGE is an inflammatory disease of unknown aetiology, characterised by infiltration of the gastrointestinal tract by eosinophils, accompanied by varying abdominal symptoms and usually by peripheral blood eosinophilia.1 EA is a rare presentation of an unusual form of serosal EGE and data regarding its prevalence and the demographic distribution are scarce. It has been reported across all age groups. It was first described by Kaijser in 19372 and since then multiple cases have been reported. EGE is an uncommon condition.3,4 Klein expanded our horizon of the disease by classifying it according to predominance of eosinophilic infiltrate in different layers of the intestinal wall – the mucosa, muscle layer and subserosal layers.5 Mucosal involvement leads to protein-losing enteropathy, faecal blood loss and malabsorption. Muscularis involvement may lead to obstruction of the gastric outlet or small bowel. Subserosal involvement manifests as eosinophilic ascites. In many cases of EGE, especially the serosal form in which systemic eosinophilia and eosinophilic infiltration of gastrointestinal mucosa might be absent, the diagnosis may be hard to make and may require an extensive and invasive work-up.6 Most patients with EGE present with variable abdominal complaints such as abdominal pain, loss of appetite, nausea and vomiting. Children and adolescents may present with growth retardation and failure to thrive. Our patient also had chronic abdominal pain, vomiting and recurrent gastroenteritis and growth retardation. The variable presentation of the disease makes it important to have a high degree of suspicion to reach the diagnosis. Work-up for the diagnosis requires exclusion of parasitic infestation and malignancy, such as lymphomas, as a prerequisite. Most patients with EA present with features of atopy. Food allergy has been commonly linked to EGE, and although peripheral
eosinophilia is common, it is not essential for diagnosis. Talley et al., in their case series of 40 patients, showed normal eosinophils counts in 23% of cases.7 Our patient showed no evidence of atopy, allergy or family history of allergens. Peripheral eosinophilia which was not present initially was, interestingly, found later on and progressively increased with time until treatment was started. Our case is a similar rare form of EA where ascitic tap revealed predominant eosinophilia. Although we do not have a tissue diagnosis to prove EGE in our case, the literature supports the diagnosis with a few case reports where EA was the sole manifestation and where tissue diagnosis could not be made on histology as the mucosal biopsies may anyway be negative in cases of predominant serosal involvement.6 The mechanism of ascites in eosinophillic serositis is similar to ascites in carcinomatous peritonitis.8 Also in view of ANA positivity in our patient, we explored the literature and found a few rare case reports where connective tissue disorders were associated with EA.9,10 We also found a similar case report of a lady with EA with ANA positivity, who subsequently developed SLE.11 Our patient is being closely followed but has shown no symptoms of any connective tissue disorder; his very strong positivity to ANA is still an enigma. Management of this disease usually requires immunosuppression, most commonly with steroids. Patients with disabling symptoms can usually be effectively treated with corticosteroids. Occasionally, sodium cromolyn, ketotifen and/or elimination diets have been shown to be effective in the management of patients who have a significant history of allergic disorder. Surgical intervention may be required in patients with obstructive complications or refractory disease. Our patient also showed complete remission with low dose steroids.
Conclusion EA as a manifestation of EGE is rare and should be diagnosed only after excluding other causes like parasites, allergy and malignancy. Its response to steroids is good. Our case is an unusual presentation with enigmatic association of ANA positivity that has responded well to steroids. Declaration of conflicting interests None declared.
Funding This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.
Downloaded from tdo.sagepub.com at Bobst Library, New York University on April 18, 2016
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References 1. Bouhmidi A, Lorente Poyatos R, Romero Cara P, et al. Eosinophilic enteritis as a rare cause of ascites. Gastroenterol Hepatol 2003; 26: 480–481. 2. Kaijser R. Zur Kenntnis der allergischen affektionen des verdauungskanals vom standput des chirurgen aus. Arch Klin Chir 1937; 188: 36–64. 3. Guajardo JR, Plotnick LM, Fende JM, Collins MH, Putnam PE and Rothenberg ME. Eosinophil-associated gastrointestinal disorders: a world-wide-web based registry. J Pediatr 2002; 141: 576–581. 4. Durieu I, Nove-Josserand R, Cathebras P, Durand DV, Rousset H and Levrat R. Eosinophilic ascites. 2 new case reports. Rev Med Interne 1992; 13: 446–448. 5. Klein NC, Hargrove RL, Sleisenger MH and Jeffries GH. Eosinophilic gastroenteritis. Medicine (Baltimore) 1970; 49: 299–319. 6. Hepburn IS, Sridhar S and Schade RR. Eosinophilic ascites, an unusual presentation of eosinophilic gastroenteritis: A case report and review. World J Gastrointest Pathophysiol 2010; 1: 166–170.
7. Talley NJ, Shorter RG, Phillips SF and Zinsmeister AR. Eosinophilic gastroenteritis: a clinicopathological study of patients with disease of the mucosa, muscle layer, and subserosal tissues. Gut 1990; 31: 54–58. 8. Kelly KJ. Eosinophilic gastroenteritis. J Pediatr Gastroenterol Nutr 2000; 30(Suppl): S28–35. 9. Barbie DA, Mangi AA and Lauwers GY. Eosinophilic gastroenteritis associated with systemic lupus erythematosus. J Clin Gastroenterol 2004; 38: 883–886. 10. DeSchryver-Kecskemeti K and Clouse RE. A previously unrecognized subgroup of ‘eosinophilic gastroenteritis’: Association with connective tissue diseases. Am J Surg Pathol 1984; 8: 171–180. 11. Asadi Gharabaghi M, Abdollahi P, Kalany M and Sotoudeh M. Systemic lupus erythematosus presenting with eosinophilic enteritis: a case report. J Med Case Rep 2011; 5: 235.
Downloaded from tdo.sagepub.com at Bobst Library, New York University on April 18, 2016
Acute eosinophilic ascites: An unusual form of an unusual case
Tropical Doctor 2015, Vol. 45(1) 39–41 ! The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0049475514553829 tdo.sagepub.com
Parul Kodan1, Meenakshi A Shetty2, MR Pavan3, Ahalya Kariappa4 and Chakrapani Mahabala5
Abstract Eosinophilic gastroenteritis (EGE) is an uncommon disease characterised by eosinophilic infiltration in the gastrointestinal tract. EGE may involve more than one layer of the gastrointestinal tract. Clinical features depend on the layer and location which is involved. We report an unusual case of eosinophilic ascites associated with antinuclear antibody positivity, which is an unusual variety of serosal form of EGE.
Keywords Eosinophilic gastroenteritis, ascites, ANA
Introduction Eosinophilic ascites (EA) is probably the most unusual and rare presentation of eosinophilic gastroenteritis (EGE) which is characterised by eosinophilic infiltration of any or all layers of the gut wall and may involve any segment of the gastrointestinal tract in the absence of known causes for eosinophilia. Peripheral eosinophilia may or may not be present. We report an unusual case of EA which is an unusual variety of the serosal form of EGE. We also found the patient to have antinuclear antibody (ANA) titer strongly positive, making this association all the more fascinating. The rarity of the case, its unusual rare associations and excellent response of our young patient to treatment, prompted us to report this case.
Case report A previously healthy 18-year-old young man presented with complaints of chronic diffuse severe abdominal pain, recurrent vomiting, loose stool and progressively increasing abdominal distension for the last 6 months. He had severe loss of appetite followed by significant weight loss. There was no history of atopy, allergy, food allergy or asthma in the past. None of his family members had similar abdominal symptoms. Several treatment modalities including antibiotics, antispasmodics, analgesics, antiemetic could not relieve his symptoms. The patient was referred to our centre in view of his chronic debilitating condition. On physical
examination, he was pale and malnourished but had no lymphadenopathy and pedal oedema. The abdomen was distended and tender diffusely with shifting dullness present. Other systemic examination was unremarkable. Haematological tests revealed mild anaemia and a peripheral smear showed peripheral eosinophilia. The Mantoux test was found to be negative. On abdominal ultrasonography, the liver was found to be of normal size and echogenicity, and all vessels were patent. There was a large amount of pelvic and abdominal ascites. Analysis of this fluid showed a low serum albumin-ascitic gradient (SAAG), 55% eosinophils and 45% lymphocytes. Standard bacterial and tuberculosis culture showed no growth. Parasitic infestation was ruled out by repeated negative stool examinations. HIV enzyme-linked immunosorbent 1 Resident, Department of Medicine, Kasturba Medical College, Manipal University, Mangalore, India 2 Associate Professor, Department Of Medicine, Kasturba Medical College, Manipal University, Mangalore, India 3 Associate Professor, Department Of Medicine, Kasturba Medical College, Manipal University, Mangalore, India 4 Senior Resident, Department Of Medicine, Kasturba Medical College, Manipal University, Mangalore, India 5 Professor, Department Of Medicine, Kasturba Medical College, Manipal University, Mangalore, India
Corresponding author: Chakrapani Mahabala, Department of Medicine, Kasturba Medical College, Mangalore, Manipal University, Light House Hill Road, Mangalore 575001, India. Email: [email protected]
Downloaded from tdo.sagepub.com at Bobst Library, New York University on April 18, 2016
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Tropical Doctor 45(1)
assay (ELISA) was negative. Bone marrow biopsy showed normal haematopoiesis with mild eosinophilia. He underwent oesophagogastroduodenoscopy, colonoscopy and diagnostic laparoscopy with biopsies all proving normal. ANA was found to be positive in a ratio of 1:100 with a pattern corresponding to antiSSA, B. No symptoms of joint pain, rash, proteinuria, mouth dryness or any feature corresponding to history of connective tissue disorders was present. Thus a diagnosis of serosal EGE with ANA positivity was made and he was started on a low dose steroid regimen, to which he responded well with symptoms resolving within 1 week. He was discharged on low dose steroid therapy with a tapering dose. On regular review, he remains asymptomatic.
Discussion EGE is an inflammatory disease of unknown aetiology, characterised by infiltration of the gastrointestinal tract by eosinophils, accompanied by varying abdominal symptoms and usually by peripheral blood eosinophilia.1 EA is a rare presentation of an unusual form of serosal EGE and data regarding its prevalence and the demographic distribution are scarce. It has been reported across all age groups. It was first described by Kaijser in 19372 and since then multiple cases have been reported. EGE is an uncommon condition.3,4 Klein expanded our horizon of the disease by classifying it according to predominance of eosinophilic infiltrate in different layers of the intestinal wall – the mucosa, muscle layer and subserosal layers.5 Mucosal involvement leads to protein-losing enteropathy, faecal blood loss and malabsorption. Muscularis involvement may lead to obstruction of the gastric outlet or small bowel. Subserosal involvement manifests as eosinophilic ascites. In many cases of EGE, especially the serosal form in which systemic eosinophilia and eosinophilic infiltration of gastrointestinal mucosa might be absent, the diagnosis may be hard to make and may require an extensive and invasive work-up.6 Most patients with EGE present with variable abdominal complaints such as abdominal pain, loss of appetite, nausea and vomiting. Children and adolescents may present with growth retardation and failure to thrive. Our patient also had chronic abdominal pain, vomiting and recurrent gastroenteritis and growth retardation. The variable presentation of the disease makes it important to have a high degree of suspicion to reach the diagnosis. Work-up for the diagnosis requires exclusion of parasitic infestation and malignancy, such as lymphomas, as a prerequisite. Most patients with EA present with features of atopy. Food allergy has been commonly linked to EGE, and although peripheral
eosinophilia is common, it is not essential for diagnosis. Talley et al., in their case series of 40 patients, showed normal eosinophils counts in 23% of cases.7 Our patient showed no evidence of atopy, allergy or family history of allergens. Peripheral eosinophilia which was not present initially was, interestingly, found later on and progressively increased with time until treatment was started. Our case is a similar rare form of EA where ascitic tap revealed predominant eosinophilia. Although we do not have a tissue diagnosis to prove EGE in our case, the literature supports the diagnosis with a few case reports where EA was the sole manifestation and where tissue diagnosis could not be made on histology as the mucosal biopsies may anyway be negative in cases of predominant serosal involvement.6 The mechanism of ascites in eosinophillic serositis is similar to ascites in carcinomatous peritonitis.8 Also in view of ANA positivity in our patient, we explored the literature and found a few rare case reports where connective tissue disorders were associated with EA.9,10 We also found a similar case report of a lady with EA with ANA positivity, who subsequently developed SLE.11 Our patient is being closely followed but has shown no symptoms of any connective tissue disorder; his very strong positivity to ANA is still an enigma. Management of this disease usually requires immunosuppression, most commonly with steroids. Patients with disabling symptoms can usually be effectively treated with corticosteroids. Occasionally, sodium cromolyn, ketotifen and/or elimination diets have been shown to be effective in the management of patients who have a significant history of allergic disorder. Surgical intervention may be required in patients with obstructive complications or refractory disease. Our patient also showed complete remission with low dose steroids.
Conclusion EA as a manifestation of EGE is rare and should be diagnosed only after excluding other causes like parasites, allergy and malignancy. Its response to steroids is good. Our case is an unusual presentation with enigmatic association of ANA positivity that has responded well to steroids. Declaration of conflicting interests None declared.
Funding This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.
Downloaded from tdo.sagepub.com at Bobst Library, New York University on April 18, 2016
Kodan et al.
41
References 1. Bouhmidi A, Lorente Poyatos R, Romero Cara P, et al. Eosinophilic enteritis as a rare cause of ascites. Gastroenterol Hepatol 2003; 26: 480–481. 2. Kaijser R. Zur Kenntnis der allergischen affektionen des verdauungskanals vom standput des chirurgen aus. Arch Klin Chir 1937; 188: 36–64. 3. Guajardo JR, Plotnick LM, Fende JM, Collins MH, Putnam PE and Rothenberg ME. Eosinophil-associated gastrointestinal disorders: a world-wide-web based registry. J Pediatr 2002; 141: 576–581. 4. Durieu I, Nove-Josserand R, Cathebras P, Durand DV, Rousset H and Levrat R. Eosinophilic ascites. 2 new case reports. Rev Med Interne 1992; 13: 446–448. 5. Klein NC, Hargrove RL, Sleisenger MH and Jeffries GH. Eosinophilic gastroenteritis. Medicine (Baltimore) 1970; 49: 299–319. 6. Hepburn IS, Sridhar S and Schade RR. Eosinophilic ascites, an unusual presentation of eosinophilic gastroenteritis: A case report and review. World J Gastrointest Pathophysiol 2010; 1: 166–170.
7. Talley NJ, Shorter RG, Phillips SF and Zinsmeister AR. Eosinophilic gastroenteritis: a clinicopathological study of patients with disease of the mucosa, muscle layer, and subserosal tissues. Gut 1990; 31: 54–58. 8. Kelly KJ. Eosinophilic gastroenteritis. J Pediatr Gastroenterol Nutr 2000; 30(Suppl): S28–35. 9. Barbie DA, Mangi AA and Lauwers GY. Eosinophilic gastroenteritis associated with systemic lupus erythematosus. J Clin Gastroenterol 2004; 38: 883–886. 10. DeSchryver-Kecskemeti K and Clouse RE. A previously unrecognized subgroup of ‘eosinophilic gastroenteritis’: Association with connective tissue diseases. Am J Surg Pathol 1984; 8: 171–180. 11. Asadi Gharabaghi M, Abdollahi P, Kalany M and Sotoudeh M. Systemic lupus erythematosus presenting with eosinophilic enteritis: a case report. J Med Case Rep 2011; 5: 235.
Downloaded from tdo.sagepub.com at Bobst Library, New York University on April 18, 2016
