Acta Clinica Belgica International Journal of Clinical and Laboratory Medicine
ISSN: 1784-3286 (Print) 2295-3337 (Online) Journal homepage: http://www.tandfonline.com/loi/yacb20
Massive Eosinophilic Ascites: Differential Diagnosis Between Idiopathic Hypereosinophilic Syndrome and Eosinophilic Gastroenteritis I.A. Vandewiele, B.M. Maeyaert, E.J. Van Cutsem, K.R. Geboes & D.C. Knockaert To cite this article: I.A. Vandewiele, B.M. Maeyaert, E.J. Van Cutsem, K.R. Geboes & D.C. Knockaert (1991) Massive Eosinophilic Ascites: Differential Diagnosis Between Idiopathic Hypereosinophilic Syndrome and Eosinophilic Gastroenteritis, Acta Clinica Belgica, 46:1, 37-41, DOI: 10.1080/17843286.1991.11718139 To link to this article: http://dx.doi.org/10.1080/17843286.1991.11718139
Published online: 16 May 2016.
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Citing articles: 6 View citing articles
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Date: 10 August 2017, At: 05:31
37
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MASSIVE EOSINOPHILIC ASCITES : DIFFERENTIAL DIAGNOSIS BETWEEN IDIOPATHIC HYPEREOSINOPHILIC SYNDROME AND EOSINOPHILIC GASTROENTERITIS.
1
I.A. Vandewiele*, B.M. Maeyaert*, E.J. Van Cutsem*,K.R. Geboes** and D.C. Knockaert*
SUMMARY This paper describes a patient with massive eosinophilic ascites as presenting manifestation probably due to idiopathic hypereosinophilic syndrome. Eosinophilic ascites and stomach wall involvement were the first detected abnonnalities. The subsequent course was characterised by interstitial pulmonary disease and pleural and pericardia! effusion. Grand mal epilepsy and numbness of the left ann indicated central nervous system involvement. Treatment with corticosteroids resulted in complete remission. The differential diagnosis of eosinophilic gastroenteritis and idiopathic hypereosinophilic syndrome is discussed. Acta Clin Belg. 46, I : 37-41 INTRODUCTION The idiopathic hypereosinophilic syndrome (HES) is characterized by marked and persistent (more than 6 months) eosinophilia of unknown cause associated with diffuse organ infiltration and dysfunction (1). Virtually any organ system can be involved in HES but massive eosinophilic ascites as initial manifestation has not been previously described.
Departments of Internal Medicine* and Pathology**, University Hospital Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium. Reprints : D.C. Knockaert
In contrast, abdominal bloating and ascites has repeatedly been reported in the subserosal variant of eosinophilic gastroenteritis but in a recent series of 40 patients, there was no evidence of extraintestinal disease (2). We report on a patient presenting with massive ascites and eosinophilic stomach wall involvement in whom we eventually diagnosed HES because of multiple signs of extraintestinal organ involvement.
CASE REPORT A 65-year-old man was admitted because of rapidly increasing abdominal distention. Since more than 30 years he periodically took aminophyllines and corticosteroids because of infection induced bronchospasm. A search for an allergic base of his bronchospasm was negative 5 years earlier. At that time, the eosinophil count was normal (0.28 x 109/1), IgE level was 450 IU/ ml (nl< 120) and scratch tests as well rast tests were negative. He felt well until 7 weeks earlier when he noted a vague abdominal discomfort, decreased appetite, weight loss of8 kg and abdominal distention. Ultrasonography and CAT scan revealed ascites and also thickening and rigidity of the stomach wall. A barium examination of the stomach and gastroscopy confirmed thickened folds limited to the antral area and diminished expansion of the antrum. Endoscopic biopsies of stomach, duodenum and jejunum were reported Acra Clinica Belgica 46 .l ( 1991)
38
Downloaded by [University of Auckland Library] at 05:31 10 August 2017
to be nonnal. An exploratory laparotomy revealed an oedematous and thickened stomach wa11 and a nonnal liver.
MASSIVE EOSINOPHIL/C ASCJTES
in close association with ganglion cells. The ce11ular aggregate varied in size and showed a focal distribution. In some of the larger aggregates fibrillar eosinophilic material could be distinguished. In addition dense lymphoplasmocytic infiltrates could be observed around the submucosa vessels. The patient was discharged without further treatment. Because of persisting abdominal distention he was referred to our unit. On admission the patient appeared well. The abdomen was distended without co11ateral veins and there was no oedema nor other signs of cardiac failure. The erythrocyte sedimentation rate was 23 mm/h. The hematocrit was 0.43, the white-cell count was 6.2 x 109/l, with 60 % neutrophils, 24 % eosinophils and 16 % lympho-monocytes. Liver function tests and urine examination were nonnal.
Fig . I: Microphotograph ofthe stomach biopsy showing mucosa of the fundic gland type (m), muscularis mucosae (mm) and submucosa (s). A diffusely scattered inflammatory infiltrate is present in the muscu/aris mucosae and submucosa. In the latter it is especially concentrated around the vessels (arrows) . (H.E. X20).
Acta Clinica Be/gica 46.J (1991)
The IgE level was 1507 U/ml (nl < 120). Xrays of the thorax showed, in contrast to the nonnal picture seven weeks earlier, a bilateral pleural effusion and signs of interstitial Jung disease. Abdominal ultrasonography revealed ascites and a nonnal liver and spleen. A small (5 mm) pericardial effusion was detected but the myocardial contractility, the endocard and heart valves were nonnal. Lung function tests revealed a mixed restrictive and obstructive pattern: VC 2268 ml (59.5%), FEV 1 1332 ml (46.4 %), RV 2177 ml (90.5%), TLC 4445 ml (71.5%) DLCO 5.9 (70%), DLCONC 1.93 (112.3%). In the ascitic fluid 0.5 x 109 cells/I were detected with 66 % eosinophils, 16 % neutrophils, I % basophils, 12 % lymfocytes and 3 % monocytes. The LDH level was 206 U/l and the protein content 52.8 g/l. The pleural fluid contained 2.1 x 109 cells/I with 13 % neutrophils, 20 % eosinophils, 59 % lymphocytes, 3 % basophils and 5 % monocytes. The LDH level was 161 U/l, the protein content 40 g/l. Cultures, Ziehl-Nielsen staining and Lowenstein cultures of these fluids remained negative. Bone marrow eosinophilia was 24 %.
MASSIVE EOS/NOPHIL/C ASC/TES
Rectoscopy and barium examination of the small bowel were normal. The endo~copic biopsies of the rectum disclosed eosinophils in the submucosaJ layer.
Downloaded by [University of Auckland Library] at 05:31 10 August 2017
No parasites were detected in the stools nor in the small bowel aspirate. A search for toxocara and strongyloides antibodies remained negative.
1
During bronchoscopy the patient became extremely bronchospastic and high dose corticosteroid treatment (methylprednisolone 64 mg a day) was started urgently with quick amelioration of his pulmonary condition. Three days after starting corticosteroids the patient had a grand ma) seizure and experienced numbness of the left arm and dizziness. Cerebrospinal fluid analysis was entirely normal. Magnetic resonance imaging of the brain and electromyography revealed no abnormalities. Dyspnea progressively disappeared and there was a total regression of the ascites, the pleural effusion and the X ray signs of interstitial Jung disease. Lung function tests became normal: VC 3356 ml (88. I%) FEV 1 2273 ml (79.2 %), RV 3150 ml (130.9 %), TLC 6506 ml (I 04. 7 %), DLCO 8.41 ( 100 %), DLCO/ vc 1.68 (97.8 %). The patient was discharged with methylprednisolone 32 mg a day. At a follow up visit three months later, taking 24 mg methylprednisolone a day, the patient was free of complaints. The physical examination was entirely normal. The leucocyte count was 10.8 x 109/1, with 89 % neutrophils, I % eosinophils, 6 % lymphocytes and 4 % monocytes and the lgE level was 321 U/ ml. Chest x-rays and abdominal ultrasonography were normal. Eleven months after starting the corticosteroids the patient is taking 12 mg methylprednisone a day and remains symptom free.
DISCUSSION The HES comprises a variety of clinical syndromes and at least three different forms with
39
varying severity are described. Some patients only have hypereosinophilia with lung involvement and angio-oedema. Others present with or develop severe cardiac and central nervous system complications. A third group has eosinophilic cytogenic abnormalities and other features of a leukaemic disease and is considered as eosinophilic leukaemia (I). This heterogeneous picture reflects the absence of specific diagnostic characteristics. The three criteria required for the diagnosis of HES are I) persistent hypereosinophilia of more than 1500 eosinophils/mm3 for longer than 6 months or death before 6 months with signs and symptoms of HES syndrome 2) a lack of evidence for other causes of eosinophilia and 3) organ infiltration and dysfunction (3). Our case doesn't meet the first criterion, strictly spoken. However even without the urgent need for treatment we feel that further conservative approach would not have been possible in view of the massive ascites and the pleural and pericardial effusion. Our case belongs to the group of patients requiring treatment to prevent death from complications of HES. Although our patient had bronchospasm, atopy had been excluded as a cause of his bronchospasm five years before the present admission and his bronchospasm was diagnosed as exercise and infection induced. The evolving roentgenographic pattern consisting of fine reticulation could not be ascribed to alveolar hypoventilation secondary to the ascites. Asthma cannot explain the findings of massive eosinophilia in the gastro-intestinal tract nor the presence of a pericardial and pleural effusion. Pleural effusion may be seen in patients with tense ascites but pericardia! involvement is unlikely. HES has, to our know ledge, never been reported with eosinophilic ascites as presenting manifestation. Since our patient had no evidence of parasitic infection or neoplastic disease and never stayed in (sub)tropical countries the differential Acta Clinica Belgica 46.1 ( 1991)
40
Downloaded by [University of Auckland Library] at 05:31 10 August 2017
diagnosis in this presentation is chiefly limited to eosinophilic gastroenteritis. Eosinophilic gastroenteritis is characterized by peripheral eosinophilia and infiltration of the gastrointestinal tract by eosinophils which may lead to a wide array of symptoms. There is a slight preponderance of the condition among men (m:f =3:2) and the disease seems to have a bimodal age distribution with peaks in the third and sixth decade (2,4 ). HES is primarily a disease of middle age with male predominance (5) as in our case. Eosinophilic gastroenteritis typically involves the stomach and proximal small bowel but any area of the gastrointestinal tract may be involved (2). Eighty percent of the patients have symptoms for I to 25 years. Three major clinical patterns are described, depending on the depth of eosinophilic infiltration of the gastrointestinal wall (2). The most common pattern is the mucosal form with diarrhea as main manifestation. Patients with muscle layer invasion present with symptoms of gastric or small' bowel obstruction. Finally, a third group with subserosal eosinophilic infiltration may develop eosinophilic ascites (2,6). This manifestation is rare and until 1981 only 15 well documented case reports of eosinophilic gastroenteritis with ascites have been described (7). In a recent study 5 of 40 patients presented with abdominal bloating and ascites (2). Our patient had submucosal, muscular layer and subserosal eosinophilic infiltration. The endoscopic and roentgenologic studies were normal except for the stomach wall infiltration. In the endoscopic biopsies of the rectum eosinophils could also be found in the submucosa. Moreover the presence of pleural and pericardia( effusion, the signs of interstitial lung disease and the subsequent neurological symptoms strongly point to a multisystem involvement consistent with HES. These findings are not consistent with the diagnosis of eosinophilic gastroenteritis.
MASSIVE EOSINOPHILJC ASCJTES
was instituted at an early stage, so not allowing further functional and morphological deterioration to occur. Eosinophil-induced neuronal damage may be caused by different mechanisms and several neurotoxic eosinophil proteins have been described (8). We conclude that this case, presenting with massive eosinophilic ascites, was probably caused by HES and not by eosinophilic gastroenteritis
SAMENV ATIING Dit artikel beschrijft een patient met massieve eosinofiele ascites als presenterende manifestatie waarschijnlijk van het idiopathisch hypereosinofiel syndroom. Eosinofiele ascites en maagwand aantasting waren de eerst vastgestelde ziektetekenen. Het verder verloop was gekarakteriseerd door interstitieel longlijden, pleurale en pericardiale vochtuitstorting. Grand mal epilepsie en voosheid van de linker ann wezen op aantasting van het cent.raal zenuwstelsel. Behandeling met corticosteroi'den resulteerde in volledige remissie. De differentieel diagnose van eosinofiele gastroenteritis en het idiopathisch hypereosinofiel syndroom wordt besproken.
RESUME Cet article decrit l'histoire d ' un patient avec une ascite massive aeosinophiles comme premiere manifestation d'un syndrome hypereosinophilique idiopathique. L'ascite aeosinophiles et l'atteinte de la paroi gastrique furent Jes premieres anomalies detectees. L ·evolution ulterieure fut caracterisee par une atteinte interstitielle pulmonaire, un epanchement pleural et pericardique. Des crises d'epilepsie grand mal et des paresthesies du bras gauche temoignerent d'une atteinte du systeme nerveux central. Le traitement par corticosteroi'des induit une remission complete. Le diagnostic differentiel de la gastroenterite ll eosinophiles et du syndrome hypereosinophilique idiopathique sont discutes.
REFERENCES
The absence of abnormalities on electromyography and magnetic resonance imaging can be explained by the fact that treatment Acta Clinica Be/gica 46./ ( 1991)
I. Spry CJF. The hypereosi nophilic syndrome: clinical feature, laboratory findings and treatment. A/lergy.1982; 37: 539-51.
Downloaded by [University of Auckland Library] at 05:31 10 August 2017
MASSIVE EOSINOPH/L/C ASCITES
2. Talley NJ, Shorter RG, Phillips SF, Zinsmeister AR. Eosinophilic gastro-enteritis: a clinicopathological study of patients with disease of the mucosa, muscle layer and subserosal tissues. Gut.1990; 31: 54-8. 3. Fanci AS, Harley JB, Roberts WC et al. The idiopathic hypereosinophilic syndrome. Clinical, pathophysiologic and therapeutic considerations. Ann Intern Med. 1982; 97: 78-92. 4. Blackshaw AJ, Levison DA . Eos inophilic infiltrates of the gastrointestinal tract. J Clin Pathol. 1986; 39: I- 7. 5. Alfaham MA, Ferguson SD, Sihra Band Davies J.
41
The idiopathic hypereosinophilic syndrome. Arch Dis Childhood. 1987; 62: 601-13. 6. Spry CJF . Eosinophilic gastroenteritis. In : BouchierIAD, Allan RN, Hodgson HJF, Keighley MRB eds. Textbook ofGastroenterology. London : Saunders, 1984; 596-8. 7. Harmon WA, Helman CA. Eosinophilic gastroenteritis and ascites. J Clin Gastroentero/. 1981; 3: 371 8. Weaver OF, Heffemanman LP, Purdy RA, Ing VW. Eosinophil-induced neurotoxicity : axonal neuropathy, cerebral infarction and dementia. Neurology. 1988; 38: 144-6.
Acta Clinica Be/gica 46./ (1991)
ISSN: 1784-3286 (Print) 2295-3337 (Online) Journal homepage: http://www.tandfonline.com/loi/yacb20
Massive Eosinophilic Ascites: Differential Diagnosis Between Idiopathic Hypereosinophilic Syndrome and Eosinophilic Gastroenteritis I.A. Vandewiele, B.M. Maeyaert, E.J. Van Cutsem, K.R. Geboes & D.C. Knockaert To cite this article: I.A. Vandewiele, B.M. Maeyaert, E.J. Van Cutsem, K.R. Geboes & D.C. Knockaert (1991) Massive Eosinophilic Ascites: Differential Diagnosis Between Idiopathic Hypereosinophilic Syndrome and Eosinophilic Gastroenteritis, Acta Clinica Belgica, 46:1, 37-41, DOI: 10.1080/17843286.1991.11718139 To link to this article: http://dx.doi.org/10.1080/17843286.1991.11718139
Published online: 16 May 2016.
Submit your article to this journal
View related articles
Citing articles: 6 View citing articles
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=yacb20 Download by: [University of Auckland Library]
Date: 10 August 2017, At: 05:31
37
Downloaded by [University of Auckland Library] at 05:31 10 August 2017
MASSIVE EOSINOPHILIC ASCITES : DIFFERENTIAL DIAGNOSIS BETWEEN IDIOPATHIC HYPEREOSINOPHILIC SYNDROME AND EOSINOPHILIC GASTROENTERITIS.
1
I.A. Vandewiele*, B.M. Maeyaert*, E.J. Van Cutsem*,K.R. Geboes** and D.C. Knockaert*
SUMMARY This paper describes a patient with massive eosinophilic ascites as presenting manifestation probably due to idiopathic hypereosinophilic syndrome. Eosinophilic ascites and stomach wall involvement were the first detected abnonnalities. The subsequent course was characterised by interstitial pulmonary disease and pleural and pericardia! effusion. Grand mal epilepsy and numbness of the left ann indicated central nervous system involvement. Treatment with corticosteroids resulted in complete remission. The differential diagnosis of eosinophilic gastroenteritis and idiopathic hypereosinophilic syndrome is discussed. Acta Clin Belg. 46, I : 37-41 INTRODUCTION The idiopathic hypereosinophilic syndrome (HES) is characterized by marked and persistent (more than 6 months) eosinophilia of unknown cause associated with diffuse organ infiltration and dysfunction (1). Virtually any organ system can be involved in HES but massive eosinophilic ascites as initial manifestation has not been previously described.
Departments of Internal Medicine* and Pathology**, University Hospital Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium. Reprints : D.C. Knockaert
In contrast, abdominal bloating and ascites has repeatedly been reported in the subserosal variant of eosinophilic gastroenteritis but in a recent series of 40 patients, there was no evidence of extraintestinal disease (2). We report on a patient presenting with massive ascites and eosinophilic stomach wall involvement in whom we eventually diagnosed HES because of multiple signs of extraintestinal organ involvement.
CASE REPORT A 65-year-old man was admitted because of rapidly increasing abdominal distention. Since more than 30 years he periodically took aminophyllines and corticosteroids because of infection induced bronchospasm. A search for an allergic base of his bronchospasm was negative 5 years earlier. At that time, the eosinophil count was normal (0.28 x 109/1), IgE level was 450 IU/ ml (nl< 120) and scratch tests as well rast tests were negative. He felt well until 7 weeks earlier when he noted a vague abdominal discomfort, decreased appetite, weight loss of8 kg and abdominal distention. Ultrasonography and CAT scan revealed ascites and also thickening and rigidity of the stomach wall. A barium examination of the stomach and gastroscopy confirmed thickened folds limited to the antral area and diminished expansion of the antrum. Endoscopic biopsies of stomach, duodenum and jejunum were reported Acra Clinica Belgica 46 .l ( 1991)
38
Downloaded by [University of Auckland Library] at 05:31 10 August 2017
to be nonnal. An exploratory laparotomy revealed an oedematous and thickened stomach wa11 and a nonnal liver.
MASSIVE EOSINOPHIL/C ASCJTES
in close association with ganglion cells. The ce11ular aggregate varied in size and showed a focal distribution. In some of the larger aggregates fibrillar eosinophilic material could be distinguished. In addition dense lymphoplasmocytic infiltrates could be observed around the submucosa vessels. The patient was discharged without further treatment. Because of persisting abdominal distention he was referred to our unit. On admission the patient appeared well. The abdomen was distended without co11ateral veins and there was no oedema nor other signs of cardiac failure. The erythrocyte sedimentation rate was 23 mm/h. The hematocrit was 0.43, the white-cell count was 6.2 x 109/l, with 60 % neutrophils, 24 % eosinophils and 16 % lympho-monocytes. Liver function tests and urine examination were nonnal.
Fig . I: Microphotograph ofthe stomach biopsy showing mucosa of the fundic gland type (m), muscularis mucosae (mm) and submucosa (s). A diffusely scattered inflammatory infiltrate is present in the muscu/aris mucosae and submucosa. In the latter it is especially concentrated around the vessels (arrows) . (H.E. X20).
Acta Clinica Be/gica 46.J (1991)
The IgE level was 1507 U/ml (nl < 120). Xrays of the thorax showed, in contrast to the nonnal picture seven weeks earlier, a bilateral pleural effusion and signs of interstitial Jung disease. Abdominal ultrasonography revealed ascites and a nonnal liver and spleen. A small (5 mm) pericardial effusion was detected but the myocardial contractility, the endocard and heart valves were nonnal. Lung function tests revealed a mixed restrictive and obstructive pattern: VC 2268 ml (59.5%), FEV 1 1332 ml (46.4 %), RV 2177 ml (90.5%), TLC 4445 ml (71.5%) DLCO 5.9 (70%), DLCONC 1.93 (112.3%). In the ascitic fluid 0.5 x 109 cells/I were detected with 66 % eosinophils, 16 % neutrophils, I % basophils, 12 % lymfocytes and 3 % monocytes. The LDH level was 206 U/l and the protein content 52.8 g/l. The pleural fluid contained 2.1 x 109 cells/I with 13 % neutrophils, 20 % eosinophils, 59 % lymphocytes, 3 % basophils and 5 % monocytes. The LDH level was 161 U/l, the protein content 40 g/l. Cultures, Ziehl-Nielsen staining and Lowenstein cultures of these fluids remained negative. Bone marrow eosinophilia was 24 %.
MASSIVE EOS/NOPHIL/C ASC/TES
Rectoscopy and barium examination of the small bowel were normal. The endo~copic biopsies of the rectum disclosed eosinophils in the submucosaJ layer.
Downloaded by [University of Auckland Library] at 05:31 10 August 2017
No parasites were detected in the stools nor in the small bowel aspirate. A search for toxocara and strongyloides antibodies remained negative.
1
During bronchoscopy the patient became extremely bronchospastic and high dose corticosteroid treatment (methylprednisolone 64 mg a day) was started urgently with quick amelioration of his pulmonary condition. Three days after starting corticosteroids the patient had a grand ma) seizure and experienced numbness of the left arm and dizziness. Cerebrospinal fluid analysis was entirely normal. Magnetic resonance imaging of the brain and electromyography revealed no abnormalities. Dyspnea progressively disappeared and there was a total regression of the ascites, the pleural effusion and the X ray signs of interstitial Jung disease. Lung function tests became normal: VC 3356 ml (88. I%) FEV 1 2273 ml (79.2 %), RV 3150 ml (130.9 %), TLC 6506 ml (I 04. 7 %), DLCO 8.41 ( 100 %), DLCO/ vc 1.68 (97.8 %). The patient was discharged with methylprednisolone 32 mg a day. At a follow up visit three months later, taking 24 mg methylprednisolone a day, the patient was free of complaints. The physical examination was entirely normal. The leucocyte count was 10.8 x 109/1, with 89 % neutrophils, I % eosinophils, 6 % lymphocytes and 4 % monocytes and the lgE level was 321 U/ ml. Chest x-rays and abdominal ultrasonography were normal. Eleven months after starting the corticosteroids the patient is taking 12 mg methylprednisone a day and remains symptom free.
DISCUSSION The HES comprises a variety of clinical syndromes and at least three different forms with
39
varying severity are described. Some patients only have hypereosinophilia with lung involvement and angio-oedema. Others present with or develop severe cardiac and central nervous system complications. A third group has eosinophilic cytogenic abnormalities and other features of a leukaemic disease and is considered as eosinophilic leukaemia (I). This heterogeneous picture reflects the absence of specific diagnostic characteristics. The three criteria required for the diagnosis of HES are I) persistent hypereosinophilia of more than 1500 eosinophils/mm3 for longer than 6 months or death before 6 months with signs and symptoms of HES syndrome 2) a lack of evidence for other causes of eosinophilia and 3) organ infiltration and dysfunction (3). Our case doesn't meet the first criterion, strictly spoken. However even without the urgent need for treatment we feel that further conservative approach would not have been possible in view of the massive ascites and the pleural and pericardial effusion. Our case belongs to the group of patients requiring treatment to prevent death from complications of HES. Although our patient had bronchospasm, atopy had been excluded as a cause of his bronchospasm five years before the present admission and his bronchospasm was diagnosed as exercise and infection induced. The evolving roentgenographic pattern consisting of fine reticulation could not be ascribed to alveolar hypoventilation secondary to the ascites. Asthma cannot explain the findings of massive eosinophilia in the gastro-intestinal tract nor the presence of a pericardial and pleural effusion. Pleural effusion may be seen in patients with tense ascites but pericardia! involvement is unlikely. HES has, to our know ledge, never been reported with eosinophilic ascites as presenting manifestation. Since our patient had no evidence of parasitic infection or neoplastic disease and never stayed in (sub)tropical countries the differential Acta Clinica Belgica 46.1 ( 1991)
40
Downloaded by [University of Auckland Library] at 05:31 10 August 2017
diagnosis in this presentation is chiefly limited to eosinophilic gastroenteritis. Eosinophilic gastroenteritis is characterized by peripheral eosinophilia and infiltration of the gastrointestinal tract by eosinophils which may lead to a wide array of symptoms. There is a slight preponderance of the condition among men (m:f =3:2) and the disease seems to have a bimodal age distribution with peaks in the third and sixth decade (2,4 ). HES is primarily a disease of middle age with male predominance (5) as in our case. Eosinophilic gastroenteritis typically involves the stomach and proximal small bowel but any area of the gastrointestinal tract may be involved (2). Eighty percent of the patients have symptoms for I to 25 years. Three major clinical patterns are described, depending on the depth of eosinophilic infiltration of the gastrointestinal wall (2). The most common pattern is the mucosal form with diarrhea as main manifestation. Patients with muscle layer invasion present with symptoms of gastric or small' bowel obstruction. Finally, a third group with subserosal eosinophilic infiltration may develop eosinophilic ascites (2,6). This manifestation is rare and until 1981 only 15 well documented case reports of eosinophilic gastroenteritis with ascites have been described (7). In a recent study 5 of 40 patients presented with abdominal bloating and ascites (2). Our patient had submucosal, muscular layer and subserosal eosinophilic infiltration. The endoscopic and roentgenologic studies were normal except for the stomach wall infiltration. In the endoscopic biopsies of the rectum eosinophils could also be found in the submucosa. Moreover the presence of pleural and pericardia( effusion, the signs of interstitial lung disease and the subsequent neurological symptoms strongly point to a multisystem involvement consistent with HES. These findings are not consistent with the diagnosis of eosinophilic gastroenteritis.
MASSIVE EOSINOPHILJC ASCJTES
was instituted at an early stage, so not allowing further functional and morphological deterioration to occur. Eosinophil-induced neuronal damage may be caused by different mechanisms and several neurotoxic eosinophil proteins have been described (8). We conclude that this case, presenting with massive eosinophilic ascites, was probably caused by HES and not by eosinophilic gastroenteritis
SAMENV ATIING Dit artikel beschrijft een patient met massieve eosinofiele ascites als presenterende manifestatie waarschijnlijk van het idiopathisch hypereosinofiel syndroom. Eosinofiele ascites en maagwand aantasting waren de eerst vastgestelde ziektetekenen. Het verder verloop was gekarakteriseerd door interstitieel longlijden, pleurale en pericardiale vochtuitstorting. Grand mal epilepsie en voosheid van de linker ann wezen op aantasting van het cent.raal zenuwstelsel. Behandeling met corticosteroi'den resulteerde in volledige remissie. De differentieel diagnose van eosinofiele gastroenteritis en het idiopathisch hypereosinofiel syndroom wordt besproken.
RESUME Cet article decrit l'histoire d ' un patient avec une ascite massive aeosinophiles comme premiere manifestation d'un syndrome hypereosinophilique idiopathique. L'ascite aeosinophiles et l'atteinte de la paroi gastrique furent Jes premieres anomalies detectees. L ·evolution ulterieure fut caracterisee par une atteinte interstitielle pulmonaire, un epanchement pleural et pericardique. Des crises d'epilepsie grand mal et des paresthesies du bras gauche temoignerent d'une atteinte du systeme nerveux central. Le traitement par corticosteroi'des induit une remission complete. Le diagnostic differentiel de la gastroenterite ll eosinophiles et du syndrome hypereosinophilique idiopathique sont discutes.
REFERENCES
The absence of abnormalities on electromyography and magnetic resonance imaging can be explained by the fact that treatment Acta Clinica Be/gica 46./ ( 1991)
I. Spry CJF. The hypereosi nophilic syndrome: clinical feature, laboratory findings and treatment. A/lergy.1982; 37: 539-51.
Downloaded by [University of Auckland Library] at 05:31 10 August 2017
MASSIVE EOSINOPH/L/C ASCITES
2. Talley NJ, Shorter RG, Phillips SF, Zinsmeister AR. Eosinophilic gastro-enteritis: a clinicopathological study of patients with disease of the mucosa, muscle layer and subserosal tissues. Gut.1990; 31: 54-8. 3. Fanci AS, Harley JB, Roberts WC et al. The idiopathic hypereosinophilic syndrome. Clinical, pathophysiologic and therapeutic considerations. Ann Intern Med. 1982; 97: 78-92. 4. Blackshaw AJ, Levison DA . Eos inophilic infiltrates of the gastrointestinal tract. J Clin Pathol. 1986; 39: I- 7. 5. Alfaham MA, Ferguson SD, Sihra Band Davies J.
41
The idiopathic hypereosinophilic syndrome. Arch Dis Childhood. 1987; 62: 601-13. 6. Spry CJF . Eosinophilic gastroenteritis. In : BouchierIAD, Allan RN, Hodgson HJF, Keighley MRB eds. Textbook ofGastroenterology. London : Saunders, 1984; 596-8. 7. Harmon WA, Helman CA. Eosinophilic gastroenteritis and ascites. J Clin Gastroentero/. 1981; 3: 371 8. Weaver OF, Heffemanman LP, Purdy RA, Ing VW. Eosinophil-induced neurotoxicity : axonal neuropathy, cerebral infarction and dementia. Neurology. 1988; 38: 144-6.
Acta Clinica Be/gica 46./ (1991)
