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Eosinophilic enteritis with systemic lupus erythematosus.



Abstract: Gastrointestinal manifestations are common in systemic lupus erythematosus Systemic Lupus Erythematosus Definition

Systemic lupus erythematosus (also called lupus or SLE) is a disease where a person's immune system attacks and injures the body's own organs and tissues. Almost every system of the body can be affected by SLE.
 (SLE SLE systemic lupus erythematosus.

SLE
abbr.
systemic lupus erythematosus


Systemic lupus erythematosus (SLE) 
). Eosinophilic eosinophilic /eo·sin·o·phil·ic/ (-fil´ik)
1. readily stainable with eosin.

2. pertaining to eosinophils.

3. pertaining to or characterized by eosinophilia.
 enteritis enteritis (ĕn'tərī`tĭs), inflammation of the gastrointestinal tract. Acute enteritis is not usually serious except in infants and older people, in whom the accompanying diarrhea can cause dehydration through the loss of fluids.  is a rare disorder of uncertain cause that was recently reported for the first time in association with SLE. This report presents a second case of eosinophilic enteritis in a 47-year-old female patient with SLE. The patient presented with recurrent episodes of abdominal pain, nausea, vomiting, and diarrhea. Complete blood counts on occasion showed elevated eosinophil eosinophil /eo·sin·o·phil/ (e?o-sin´o-fil) a granular leukocyte having a nucleus with two lobes connected by a thread of chromatin, and cytoplasm containing coarse, round granules of uniform size.  counts. The patient underwent a comprehensive workup work·up
n. Abbr. w/u
A thorough medical examination for diagnostic purposes.
 over several weeks, culminating in a small bowel biopsy that showed eosinophil infiltration in the muscularis propria, establishing the diagnosis. The patient was treated with a prolonged taper of prednisone prednisone (prĕd`nĭsōn): see corticosteroid drug.  with successful resolution of symptoms.

Key Words: eosinophilic enteritis, systemic lupus erythematosus, peripheral eosinophilia eosinophilia /eo·sin·o·phil·ia/ (e?o-sin?o-fil´e-ah) abnormally increased eosinophils in the blood.

e·o·sin·o·phil·i·a
n.
An increase in the number of eosinophils in the blood.
 

**********

Primary eosinophilic gastroenteritis (PEG) is an uncommon disorder of unknown cause, characterized by abdominal symptoms of pain, vomiting, and diarrhea as the result of eosinophilic infiltration of the gastrointestinal tract. For a diagnosis of PEG, secondary causes such as parasitic infections and other diseases must be excluded (Table). Gastrointestinal symptoms in systemic lupus erythematosus (SLE) are common, and gastrointestinal manifestations directly attributable to lupus have been reported in 1 to 28% of patients. (1) Disease findings involving the small intestine in SLE include primary vasculitis Vasculitis Definition

Vasculitis refers to a varied group of disorders which all share a common underlying problem of inflammation of a blood vessel or blood vessels. The inflammation may affect any size blood vessel, anywhere in the body.
, bowel infarction, proteinlosing enteropathy enteropathy /en·ter·op·a·thy/ (en?ter-op´ah-the) any disease of the intestine.enteropath´ic

gluten enteropathy  celiac disease.


en·ter·op·a·thy
n.
, intestinal pseudo-obstruction, (2) fat malabsorption malabsorption /mal·ab·sorp·tion/ (mal?ab-sorp´shun) impaired intestinal absorption of nutrients.

mal·ab·sorp·tion
n.
Defective or inadequate absorption of nutrients from the intestinal tract.
, and infectious diarrhea. The association of PEG with SLE has been reported only once. (3) We describe the case of a patient having both conditions, which, to our knowledge, is only the second reported incidence of this unusual finding.

Case Report

The patient is a 47-year-old female with SLE initially diagnosed in 1993 with manifestations of arthritis, malar rash, lymphopenia, antinuclear antinuclear /an·ti·nu·cle·ar/ (-noo´kle-ar) destructive to or reactive with components of the cell nucleus.  and antismooth muscle antibodies. Her SLE had been well controlled with 200 mg hydroxychloroquine twice each day and 5 to 10 mg prednisone taken daily for years. She initially presented to our hospital in August 2004 with complaints of abdominal pain, nausea, vomiting, and diarrhea. She described intermittent episodes of similar symptoms occurring sporadically and resolving in 3 to 4 days for a few weeks before admission. She had no history of exposure to diarrheal illness or parasites and no history of foreign travel. The intensity and duration of symptoms worsened with each episode until she was hospitalized for further workup and treatment. On initial examination by the rheumatology service, the patient was a mildly obese woman in some distress due to abdominal discomfort. The vital signs were unremarkable. She had no evidence of active SLE with normal findings on cardiopulmonary, joint, and skin examination. The initial abdominal examination revealed a diffusely tender abdomen with no involuntary guarding or rebound tenderness. Rectal examination was unremarkable, with a negative test for occult blood in stool.

Laboratory tests showed an erythrocyte sedimentation rate Erythrocyte Sedimentation Rate Definition

The erythrocyte sedimentation rate (ESR), or sedimentation rate (sed rate), is a measure of the settling of red blood cells in a tube of blood during one hour.
 (Westergren method) of 16 mm/h (normal, 0 to 20), and a C-reactive protein level of 0.4 mg/dL (normal, 0 to 0.8). Serum complete blood count showed an absolute lymphocyte count of 1,100/[mm.sup.3] (normal, 1,300 to 4,400). C3 complement was 50 mg/dL (normal, 86 to 154) and C4 complement was 5.2 mg/dL (normal, 20 to 59). Basic metabolic profile was significant for a serum potassium level of 2.5 mmol/L. Liver enzyme tests and urinalyses were within normal limits. Anti-dsDNA antibodies were not detected. She was given an increased dose of prednisone, and her symptoms seemed to improve. Despite improvement with steroids, there was disagreement among consultants as to the cause of her symptoms.

Gastrointestinal symptoms recurred and waxed and waned in sevetity over many months. She had a weight loss of 20 pounds during this time. She ultimately had an extensive inpatient and outpatient evaluation. Multiple stool tests for fecal leukocytes, ova ova (o´vah) plural of ovum.
Ova
Eggs.

Mentioned in: Stool O & P Test


ova

plural of ovum.
 and parasites, occult blood, Clostridium difficile toxin, and stool cultures were negative. Complete blood counts revealed elevated eosinophil counts of 500 to 700/cmm (normal, 0 to 400). Abdominal radiographs were normal. CT imaging of the abdomen was performed more than once with inconsistent findings. The initial CT of the abdomen revealed diffusely thickened and edematous e·dem·a·tous
adj.
Marked by edema.
 loops of small bowel involving most of the ileum ileum: see intestine.
ileum

Final and longest segment of the small intestine. It is the site of absorption of vitamin B12 (see vitamin B complex) and reabsorption of about 90% of conjugated bile salts.
. A subsequent CT of the abdomen showed resolution of the small bowel thickening but showed circumferential thickening of the colon wall from the mid transverse colon through the rectum. A third CT of the abdomen showed thickening of both the small and large bowels. During one admission, the patient underwent an esophagogastroduodenoscopy and colonoscopy with biopsy specimens that showed no pathologic findings. A gastric emptying study showed delayed gastric emptying. A magnetic resonance angiogram an·gi·o·gram
n.
An angiographic x-ray of blood vessels used in diagnosing pathological conditions of the cardiovascular system.//An x-ray of one or more blood vessels produced by angiography and used in diagnosing pathology in the cardiovascular
 of the abdominal vessels showed normal large vessels, but the small vessels could not be properly evaluated with this technique. The patient eventually underwent an exploratory laparoscopy laparoscopy
 or peritoneoscopy

Procedure for inspecting the abdominal cavity using a laparoscope; also surgery requiring use of a laparoscope. Laparoscopes use fibre-optic lights and small video cameras to show tissues and organs on a monitor.
 with an excisional biopsy of the small bowel that showed significant infiltration of the muscularis propria by eosinophils Eosinophils
A leukocyte with coarse, round granules present.

Mentioned in: Histiocytosis X

eosinophils
 accompanied by degenerative changes of the smooth muscle. Neither thrombosis nor vasculitis were identified. (Figure) The diagnosis of primary eosinophilic gastroenteritis (muscularis subtype) was made on the basis of clinical, laboratory, and histopathologic findings. The patient was started on 40 mg prednisone daily, and the dose was tapered over a few weeks. Eight weeks later, the patient reported no symptoms and was stable, taking 5 mg prednisone daily. The serum eosinophil count at follow-up was 200/cmm. Hydroxychloroquine was continued because of its benefit as a long-term maintenance drug for systemic lupus erythematosus. (4)

Discussion

Primary eosinophilic enteritis is a rare disorder of unknown cause that affects the gastrointestinal tract. Diagnosis must be made in the absence of known causes of eosinophilia such as malignancy, drug reactions, and other secondary causes (Table). (5) It is considered to be part of the spectrum of eosinophilic gastrointestinal inflammatory disorders that include eosinophilic esophagitis, gastroenteritis gastroenteritis: see enteritis.
gastroenteritis

Acute infectious syndrome of the stomach lining and intestines. Symptoms include diarrhea, vomiting, and abdominal cramps.
, and colitis.

Primary eosinophilic gastroenteritis is subdivided into the atopic atopic /atop·ic/ (a-top´ik) (ah-top´ik)
1. ectopic.

2. pertaining to atopy; allergic.


atopic

1. displaced; ectopic.

2. pertaining to atopy.
, nonatopic, and familial variants. Histologically, three subtypes have been classified on the basis of tissue involvement and include mucosal, muscularis, and serosal forms. (5) The typical symptoms include abdominal pain, nausea, vomiting, and diarrhea. Peripheral eosinophilia is seen less than 50% of the time. (5) The mucosal form is the most common variant and is characterized by severe abdominal symptoms that may be associated with iron-deficiency anemia, malabsorption, protein-losing enteropathy, and failure to thrive Failure to Thrive Definition

Failure to thrive (FTT) is used to describe a delay in a child's growth or development. It is usually applied to infants and children up to two years of age who do not gain or maintain weight as they should.
. The muscularis form usually shows infiltration of the muscularis layer, which can lead to thickening of the bowel wall. The serosal form is the least common variant and may be associated with eosinophilic ascites Ascites Definition

Ascites is an abnormal accumulation of fluid in the abdomen.
Description

Rapidly developing (acute) ascites can occur as a complication of trauma, perforated ulcer, appendicitis, or inflammation of the colon or other
 and peripheral eosinophilia. (6) Eosinophilic gastroenteritis has also been associated with hypereosinophilic syndrome, which is characterized by peripheral eosinophilia of at least 1,500 cells/[mm.sup.2] and infiltration of organs such as lung, liver, skin, and gastrointestinal tract that occur in the absence of known causes of eosinophilia such as parasites.

The first case of eosinophilic gastroenteritis associated with systemic lupus erythematosus was recently reported in a patient with idiopathic thrombocytopenic purpura Idiopathic Thrombocytopenic Purpura Definition

Idiopathic thrombocytopenic purpura, or ITP, is a bleeding disorder caused by an abnormally low level of platelets in the patient's blood.
, who was simultaneously diagnosed with eosinophilic gastroenteritis and SLE. (3) An association with other connective diseases including scleroderma scleroderma
 or progressive systemic sclerosis

Chronic disease that hardens the skin and fixes it to underlying structures. Swelling and collagen buildup lead to loss of elasticity. The cause is unknown.
 and idiopathic inflammatory myopathy myopathy /my·op·a·thy/ (mi-op´ah-the) any disease of muscle.myopath´ic

centronuclear myopathy  myotubular m.
 has been reported. (7,8) The incidence of primary eosinophilic gastroenteritis is unknown, but eosinophilic gastrointestinal disorders have been reported with increasing frequency, especially in children. (9) The cause of these disorders is not well understood, but allergic mechanisms have been implicated in some patients, although supporting evidence is not present in all.

Eosinophils are normal constituents of the gastrointestinal tract. They are mainly found in the lamina propria and have a primary role in host defense against helminthic hel·min·thic
adj.
1. Of or relating to worms, especially parasitic worms.

2. Tending to expel worms.

n.
See anthelmintic.
 parasites. However, eosinophilic infiltration of the deeper layers is only seen in the eosinophilic gastrointestinal disorders, both primary and secondary. (10) The expansion and tissue distribution of eosinophils is primarily regulated by IL-5 and eotaxin 1, among other cytokines. Eosinophils also contain granules Granules
Small packets of reactive chemicals stored within cells.

Mentioned in: Allergic Rhinitis, Allergies
 that are composed of toxic cationic cationic

having qualities dependent on having free cations available.


cationic detergents
are wetting agents that disrupt or damage cell membranes, denature proteins and inactivate enzymes.
 proteins such as major basic protein and eosinophil cationic protein. These cytokines, along with extracellular deposition of eosinophil granule granule, in astronomy: see photosphere.  constituents, have been demonstrated in patients with eosinophilic gastroenteritis. (6,11) However, the triggering events for this inflammatory cascade are not fully understood.

The diagnosis of eosinophilic gastroenteritis is often difficult. Symptoms are nonspecific, and peripheral eosinophilia is often not present. Endoscopic appearance of the gastrointestinal tract is often normal. As a result, the diagnosis is often delayed, as in our patient. One study reported a mean of 4 years of symptoms before the diagnosis was established. (12) Findings that support the diagnosis of PEG include (1) the presence of increased numbers of eosinophils in biopsy specimens with infiltration of the eosinophils into abnormal regions such as the intraepithelial, superficial mucosal, and intestinal crypt regions; (2) the presence of extracellular eosinophilic staining constituents; (3) the lack of involvement of other organs; and (4) the exclusion of other causes of eosinophilia. Food allergy and peripheral eosinophilia are not required for the diagnosis. (5)

Treatment of primary eosinophilic gastroenteritis depends on the subtype. In the allergic subtype, dietary modification, including elimination of foods implicated by skin prick testing or RAST, and institution of amino acid-based elemental diets might be beneficial in some patients. (13) Corticosteroids are often effective in patients refractory to dietary changes. Although there are no clear guidelines regarding dosage, prednisone (20 to 40 mg/d), followed by a 2- to 3-month taper, has been reported to have rapid symptom resolution in 90% of patients. (14) Mast cells have been found in increased levels in patients with eosinophilic gastroenteritis. As a result, mast cell stabilizers such as cromolyn and ketotifen have been used with mixed results. Montelukast montelukast /mon·te·lu·kast/ (mon?te-loo´kast) a leukotriene antagonist used as the sodium salt in prophylaxis and chronic treatment of asthma.

mon·te·lu·kast
n.
 has also been used with mixed results. New drugs such as anti-IL-5 mepolizumab and the tyrosine kinase inhibitor Noun 1. tyrosine kinase inhibitor - a drug used in cases of chronic myeloid leukemia
medicament, medication, medicinal drug, medicine - (medicine) something that treats or prevents or alleviates the symptoms of disease
 imatinib have shown promise for use in eosinophilic gastrointestinal disorders. (5)

[FIGURE OMITTED]

This is the second known case of eosinophilic gastroenteritis associated with SLE reported in the literature. It is impossible to tell if there is a pathologic relation between PEG and SLE, based on these two cases. This case also illustrates the point that gastrointestinal symptoms in patients with SLE should not be attributed to lupus-related pathology without a workup that excludes other unrelated gastrointestinal disease.

References

1. Hallegua D, Wallace D. Gastrointestinal manifestations of systemiclupus erythematosus. Curr Opin Rheumatol 2000;12:379-385.

2. Nguyen H, Khanna N. Intestinal pseudo-obstruction as a presenting manifestation of systemic lupus erythematosus: case report and review of the literature. South Med J 2004;97:186-189.

3. Barbie DA, Mangi AA, Lauwers GY. Eosinophilic gastroenteritis associated with systemic lupus erythematosus. J Clin Gastroenterol 2004;38:883-886.

4. Petri MA. Systemic lupus erythematosus: New management strategies. J Musculoskel Med 2005;22:108-116.

5. Rothenberg ME. Eosinophilic gastrointestinal disorders. J Allergy Clin Immunol 2004;113:11-28.

6. Talley NJ, Shorter RG. Phillips SF, et al. Eosinophilic gastroenteritis: A clinicopathological study of patients with disease of the mucosa, muscle layer and subserosal tissues. Gut 1990;31:54-58.

7. DeSchryver-Kecskemeti K, Clouse RE. A previously unrecognized subgroup of 'eosinophilic gastroenteritis': Association with connective tissue diseases. Am J Surg Pathol 1984;8:171-180.

8. Buchman AL, Wolf D, Gramlich T. Eosinophilic gastrojejunitis associated with connective tissue disease. South Med J 1996;89:327-330.

9. Bates Bates   , Katherine Lee 1859-1929.

American educator and writer best known for her poem "America the Beautiful," written in 1893 and revised in 1904 and 1911.
 B. 'Explosion' of eosinophilic esophagitis in children. Pediatr News 2000;34:4

10. Rothenberg ME, Mishra A, Brandt EB, et al. Gastrointestinal eosinophils. Immunol Rev 2001;179:139-155.

11. Talley NJ, Kephart GM, McGovern TW, et al. Deposition of eosinophil granule major basic protein in eosinophilic gastroenteritis and celiac disease. Gastroenterology 1992;103:137-145.

12. Guajardo JR, Plotnick LM, Fende JM, et al. Eosinophil-associated gastrointestinal disorders: a world-wide-web based registry. J Pediatr 2002;141:576-581.

13. Justinich C, Katz A, Gurbindo C, et al. Elemental diet improves steroid-dependent eosinophilic gastroenteritis and reverses growth failure. J Pediatr Gastroenterol Nutr 1996;23:81-85.

14. Fenoglio LM, Benedetti V, Rossi CM, et al. Eosinophilic gastroenteritis with ascites: A case report and review of the literature. Dig Dis Sci 2003;48:1013-1020.
When we remember we are all mad, the mysteries disappear and life stands
explained.
--Mark Twain


Prashanth R. Sunkureddi, MD, Nguyen Luu, MD, Shu-Yuan Xiao, MD, Wendell W. Tang, MD, and Bruce A. Baethge, MD

From the Division of Rheumatology, the Department of Internal Medicine, and the Department of Pathology, University of Texas Medical Branch "UTMB" redirects here. For other system schools, see University of Texas System.
The University of Texas Medical Branch (UTMB) is a component of the University of Texas System located in Galveston, Texas, about 50 miles (80 km) southeast of downtown Houston.
, Galveston, TX. Email: prsunkur@utmb.edu

Reprint requests to Dr. Bruce A. Baethge, UTMB, 301 University Boulevard, Galveston, TX 77551-1165.

Accepted May 10, 2005.

RELATED ARTICLE: Key Points

* Eosinophilic enteritis is a rare disorder of unknown cause characterized by general abdominal symptoms of pain, nausea, vomiting, and diarrhea.

* This report describes a case of eosinophilic enteritis in association with systemic lupus erythematosus.

* Peripheral eosinophilia is often present but is not necessary for the diagnosis.

* The diagnosis is made by intestinal biopsy and the demonstration of eosinophils in the intestinal layers.

* Gastrointestinal complications are common in systemic lupus erythematosus, and workup should include consideration of eosinophilic enteritis.
Table. Classification of eosinophil-associated gastroenteritis (5)

Primary (mucosa, muscularis, serosal forms)
  Atopic
  Nonatopic
  Familial
Secondary
  Eosinophilic disorders
Hypereosinophilic syndrome
  Noneosinophilic disorders
    Celiac disease
    Connective tissue disease
    Iatrogenic
    Infection
    Inflammatory bowel disease
    Vasculitis (Churg-Strauss)
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Copyright 2005, Gale Group. All rights reserved. Gale Group is a Thomson Corporation Company.

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Title Annotation:Case Report
Author:Baethge, Bruce A.
Publication:Southern Medical Journal
Geographic Code:1USA
Date:Oct 1, 2005
Words:2172
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