Intestinal Angioedema Research Articles

A CASE REPORT OF HEREDITARY ANGIOEDEMA PRESENTING WITH INTESTINAL SYMPTOMS.

Hereditary angioedema (HAE) is the deciency or dysfunction of C1 esterase inhibitor.It may also occur due to increased activity of factor XII / estrogen levels or through an unidentied cause. It manifests the attacks of swelling involving the skin and /or mucosa or submucosa of different organs.The attacks may be a result of specic trigger or occur spontaneously.The intestinal angioedema is clinically presented with moderate or severe pain abdomen, associated with nausea, vomiting, diarrhoea and/or ascites. The 38 year old woman presents with abdominal pain diffuse associated with nausea, vomitings, lips and eyelids swelling.During this period, investigations were done which revealed a small amount of free intraperitoneal uid.Biochemical testing were performed which revealed C1 esterase inhibitor deciency, decreased C4 levels. She was then diagnosed as intestinal involvement with HAE. Late diagnosis is associated with high morbidity, therefore early recognition and investigation of HAE with involvement in intestinal patients with unexplained abdominal pain is important.

A Rare Case of ACE Inhibitor-Induced Intestinal Angioedema Presenting as a Delayed Complication in a Heart Transplant Recipient

<h3>Introduction</h3> ACE inhibitor-induced intestinal angioedema is a rare side effect of ACEi that presents with nonspecific signs and symptoms. Although the risk of occurrence is greater early in therapy, angioedema can present at any time and thus a high index of suspicion is needed to avoid delays in diagnosis, unnecessary testing, and considerable morbidity. <h3>Case Report</h3> Our patient is a 54-year-old male with past medical history of orthotopic heart transplantation 3 years prior for history of end-stage ischemic cardiomyopathy, heparin-induced thrombocytopenia, and hypertension who presented with 3 days of crampy abdominal pain rated 4 out of 10. He also had bloating and watery diarrhea. He had no fever, nausea or vomiting. The patient had rebound tenderness on physical examination. Labs were unremarkable. GI pathogen panel was negative including C. difficile. CT showed a moderate segment of distal ileum which demonstrated acute inflammatory changes including mucosal hyperemia and mural edema on a background of moderate ascites. GI were consulted and performed colonoscopy that revealed no significant abnormalities. The patient had been on lisinopril for 3 years to treat hypertension. This was subsequently stopped due to suspicion of intestinal angioedema. His symptoms resolved within 72 hours of discontinuation. <h3>Summary</h3> Isolated intestinal angioedema secondary to ACEi is a diagnosis of exclusion that necessitates awareness among physicians, particularly in vulnerable subgroups such as heart transplant patients. Symptoms classically subside within 24 to 48 hours after discontinuation of the ACEi.

S3021 Development of Isolated Intestinal Angioedema 10 Years After Initiation of Lisinopril

S3021 Development of Isolated Intestinal Angioedema 10 Years After Initiation of Lisinopril

Hypovolemic shock caused by intestinal angioedema induced by Angiotensin-Converting Enzyme inhibitors in postpartum period – A case series

Hypovolemic shock caused by intestinal angioedema induced by Angiotensin-Converting Enzyme inhibitors in postpartum period – A case series

1506 Poisoned by Indian Herbal Medicine: An Inconspicuous Cause for Abdominal Pain

INTRODUCTION: Symptoms of lead poisoning are nonspecific and include abdominal pain, constipation, irritability, weight loss, and anemia. Sources of exposure in adults are predominantly occupational (ex. batteries, paint, cable, or wires), but also include herbal remedies, or cookware. Here, we report a case of lead poisoning after oral intake of Ayurvedic supplements. CASE DESCRIPTION/METHODS: A 35 y/o man presented with 6 weeks of worsening diffuse abdominal pain and cramping, SOB, fatigue and nausea. Patient denied diarrhea, vomiting or weight loss. He traveled to India earlier this year. Examination was notable for severe abdominal tenderness and slight distention. Initial work up including CBC, CMP, UA and LFTs were notable for a normocytic anemia with a Hb 9.5 (baseline 16). Stool was brown and guaiac negative. Serum haptoglobin, LDH, B12 and iron studies were within normal limits. Abdominal CT and US were unremarkable. EGD showed no bleeding. Additional work-up such as GI PCR, Meckel scan, urine PBG, blood lead level (BLL) and C1-INH were ordered. However, Hb kept dropping without improvement in pain. On day 3 of hospitalization, he was started on laxatives for constipation and discharged after improvement in bowel movements and abdominal pain with outpatient follow up for his anemia. Lab results later revealed a BLL of 78.4 ug/dL, and a blood smear showed basophilic stippling of erythrocytes, consistent with lead poisoning. On further interview, patient noted taking Ayurvedic supplements obtained in India with last intake 3 weeks prior to admission. Patient stopped his herbal medication and was started on oral succimer and eventually switched to dimercaprol. Samples of the supplements were sent out for testing. DISCUSSION: Given that many differentials for abdominal pain and anemia were ruled out, less common causes were investigated, such as AIP, intestinal angioedema, and lead poisoning. Once absorbed, lead deposits in the blood, soft tissue and bone. The toxic effects of lead are due to oxidative stress caused by inactivation of anti-oxidant enzymes, which damages DNA, cell membranes and impairs DNA transcription. Common symptoms are microcytic anemia, abdominal colic, nausea, vomiting and neuropathy. Basic workup includes free erythrocyte protoporphyrin or zinc protoporphyrin, blood urea nitrogen, serum creatinine, and urinalysis for 5-Amino-laevulin acid. In this case elevated BLL and classic blood smear lead to the diagnosis of lead poisoning.

Lupus enteritis as the only active manifestation of systemic lupus erythematosus: A case report

BACKGROUNDLupus enteritis is a rare manifestation of systemic lupus erythematosus (SLE). Diagnosis of this condition is difficult, especially in the absence of other symptoms related to active SLE. We present the case of a 25-year-old female with lupus enteritis as the sole initial manifestation of active SLE.CASE SUMMARYA 25-year-old African American female presented to the Emergency Department complaining of diffuse abdominal pain, diarrhea, nausea, and vomiting for 2 days. Her past medical history was significant for seasonal allergies and family history was pertinent for discoid lupus in her father and SLE in a cousin. The patient’s vital signs on presentation were normal. Her physical exam was remarkable for significant lower abdominal tenderness without guarding or rigidity. A computed tomography of the abdomen and pelvis revealed marked circumferential wall thickening and edema of the proximal and mid small bowel predominantly involving the submucosa. Our main differential diagnoses were intestinal angioedema and mesenteric vein thrombosis. However, mesenteric vessels were patent, and laboratory testing for hereditary angioedema showed a normal C1 Esterase Inhibitor level and low C3 and C4 levels. Infectious work-up was negative. Autoimmune tests showed elevated anti-nuclear antibodies (ANA) (13.6), anti-Smith antibody, and anti-ribonucleoprotein (anti-RNP) antibody. The patient was diagnosed with SLE enteritis. She was maintained on bowel rest, given intravenous hydration, and started on methylprednisolone 60 mg IV daily. She had significant improvement in her abdominal pain, diarrhea, and emesis after 2 days of treatment. Steroids were tapered and maintained on Hydroxychloroquine with no relapses one year after presentation.CONCLUSIONThis case of lupus enteritis represents a rare manifestation of SLE. Diagnosis requires clinical suspicion, characteristic imaging and laboratory tests. Endoscopic appearance and biopsies usually yield non-specific findings. High dose steroids are the preferred treatment modality for moderate and severe cases.

Unique Presentation of Isolated Intestinal Angioedema Without ACE-I Involvement

1651_A Figure 1. Diffuse bowel wall thickening involving stomach, small bowel and colon with suggestion of mucosal enhancement and submucosal edema.1651_B Figure 2 No Caption available.Case reports of intestinal angioedema are well documented in the literature. However, the majority of these cases are due to angiotensin-converting enzyme inhibitor (ACE-I) induced angioedema. We present an unusual case of a patient who had isolated intestinal wall angioedema without taking ACE-I and normal C1 inhibitor levels. The inciting event was ingestion of a food allergen, which is not well described in the literature. The patient is a 63 year old female who presented for 1 day history of severe abdominal pain. The patient was in her usual state of health when she drank her daily smoothie. The only addition to her smoothie was parsley. Upon ingestion, she immediately developed severe cramping pain in her abdomen associated with nausea, vomiting, and watery diarrhea. She denied itching or rash. Upon presentation to the hospital, the patient was noted to be tachycardic and tachypneic. The patient admitted to having a similar reaction years ago to ingestion of kiwi fruit. Her physical exam demonstrated non distended abdomen with diffuse tenderness to palpation and normoactive bowel sounds. Her hospital course entailed a 1 day course of antibiotics and anti-emetics. Her diet was advanced and after a 3 day hospital stay was discharged home. Labs were notable for WBC of 18,000, lactate of 5.9. The remainder of the labs including blood cultures, stool analysis, ESR, C-ANCA, P-ANCA, C1-Inhibitor, complement levels were normal. Angioedema is one of the most common allergic disorders that results in hospitalization. Any hollow viscera are prone to this type of temporary swelling. The severity of presentation of intestinal angioedema can be varied, from mild colicky pain to severe vomiting and diarrhea. Most often the inciting agent is ACE- I. This reaction is well documented but still rare with only 21 cases described in the literature. We present a case of isolated intestinal angioedema following an ingested allergen. What is unique is that this sequela occurred in the absence of ACE-I use. Furthermore, the patient presented with isolated intestinal angioedema which is unreported in literature. No signs of systemic anaphylaxis or oral angioedema were noted which is typical in food allergies. Another unique finding was normal C1 inhibitor levels making hereditary angioedema unlikely. In this instance, the mechanism was most likely due to first time parsley ingestion causing IgE mediated mast cell degranulation releasing histamine leading to intestinal swelling.

Wolf in the Sheepʼs Clothing: Intestinal Angioedema Type III Mimicking Infectious Colitis

Hereditary Angioedema (HAE) type III is a less recognized condition characterized by recurrent attacks of severe swelling, most commonly involving the arms, legs, and lips.[1] We are highlighting a case of HAE type III presenting with unusual signs and symptoms of intestinal angioedema. A 48-year-old Caucasian female presented with nausea, vomiting, diarrhea and abdominal pain. She denied any fevers, chills, cough, urinary symptoms, recent travel, or sick contacts. Previously, she had multiple admissions for suspected infectious colitis but each time workup was inconclusive. On physical examination, she was afebrile, looked lethargic, had soft, non-distended abdomen without peritoneal signs. Chest, cardiovascular and neurovascular examination was insignificant. Laboratory results revealed white cell count of 17.0 k/UL with a normal platelet count. She had a serum potassium level of 3.2 mEq/dL. Her urinalysis, renal and liver function tests were within the normal limits. CT scan abdomen and pelvis revealed areas of mural thickening of the colon and distal ileum. Upon further exploration, it was found that she had few prior attacks of lips and tongue swelling. Testing for autoimmune and lymphoproliferative disorders was negative and ruled out Acquired Angioedema. Diagnosis of HAE type III was confirmed by genetic testing which revealed factor XII gene mutation and normal C4 and C1-esterase inhibitor protein levels. No antibiotics were given during this admission and use of antihistamine and steroids were futile as her episode subsided on day five with no subsequent recurrence of bouts of abdominal pain with supportive therapy. HAE type III can potentially cause life-threatening intestinal edema, presenting as vomiting, diarrhea, and abdominal pain.[2] In contrast to other two types, type III has two missense mutation of the factor XII gene, has normal C1INH levels and is exclusively observed in females.[3] Treatment of intestinal angioedema is supportive with bowel rest with no need for steroids and antihistamines, unlike other HAE types. It can sometimes disguise as gastroenteritis, acute abdomen or infectious colitis as in our case. Physicians, therefore, should have a high index of suspicion in recognizing this rare condition particularly in patients with repeated hospitalizations and a negative workup. More studies are needed to establish the guidelines for management of HAE type III induced intestinal angioedema given limited data.[1]1497_A Figure 1. CT A/P W CONTRAST SHOWING INTESTINAL ANGIOEDEMA1497_B Figure 2. CT A/P W CONTRAST SHOWING INTESTINAL ANGIOEDEMA

Lupus Enteritis as the Only Manifestation of Active Systemic Lupus Erythematosus

Lupus enteritis is a rare manifestation of systemic lupus erythematosus (SLE). It is a difficult diagnosis to make, especially in the absence of other symptoms related to active SLE. We present a case of a 25 year old female who presented with lupus enteritis as the sole manifestation of active SLE. A 25 year old female presented to the ED with diffuse abdominal pain, diarrhea, nausea, and vomiting for 2 days. She had a medical history of seasonal allergies. Family history was significant for SLE in her cousin. Her vital signs were normal, and physical exam revealed lower abdominal tenderness. A CT of the abdomen showed marked circumferential wall thickening and edema of the proximal and mid small bowel loops predominantly involving the submucosa (Image 1). Our main differential diagnoses were intestinal angioedema and mesenteric vein thrombosis. The mesenteric vessels were patent and without evidence of thrombosis. Infectious workup was negative. Laboratory testing for hereditary angioedema showed a normal C1 Esterase Inhibitor level and low C3 and C4 levels. Autoimmune work-up showed elevated ANA (13.6), anti-Smith antibody (ab), and anti-RNP ab (Table 1). The patient was diagnosed with SLE enteritis. She was maintained on bowel rest, given intravenous (IV) hydration, and started on methylprednisolone 60 mg IV daily. She had significant improvement of all her symptoms and was discharged on prednisone 75 mg by mouth daily. In SLE, isolated gastrointestinal activity without other organ involvement is highly unusual. Lupus enteritis presents with non-specific symptoms, making the diagnosis difficult. Nevertheless, it is important to identify this disease manifestation as the mortality among patients with SLE with acute abdominal pain is approximately 11%. Lupus enteritis causes submucosal edema of the jejunum and ileum. The classic findings on CT scan include circumferential bowel wall thickening (“target sign”), dilation of intestinal segments, and engorgement of mesenteric vessels (“comb sign”). The CT scan in our case showed the “target sign”. Given her negative work up for hereditary angioedema and her family history of lupus, we decided to test for SLE. We did not perform an upper endoscopy because it often does not contribute to diagnosis. It's important to note that while most sources advocate pulse dose steroids, we were able to achieve symptomatic control with methylprednisolone 60 mg daily.2543_A Figure 1. Ab (antibody); ANA (anti-nuclear antibodies); dsDNA (double-stranded DNA); Anti-RNP (anti-ribonucleoprotein).2543_B Figure 2. Image 1. Marked circumferential wall thickening consistent with “target sign” involving multiple loops of proximal/mid small bowel. There is predominantly submucosal thickening/edema, more predominantly in the mid abdomen. The thickened small bowel loops are minimally dilated. However, there is no transition point. There is moderate volume of ascites.

Intestinal Angioedema: Case Report and Literature Review

Hereditary angioedema (HAE) is the deficiency or dysfunction of C1 esterase inhibitor (C1-INH). However, it may also occur due to either increased activity of factor XII / estrogen levels or through an unidentified cause. It manifests the attacks of swelling involving the skin and / or the mucosa / sub mucosa of different organs. The attacks may be the result of a specific trigger or occur spontaneously. The intestinal angioedema is clinically presented with moderate or severe abdominal pain, associated with nausea, vomiting, diarrhea and / or ascites, and interpreted as an “acute abdomen”. The treatment is into three distinct phases: treatment for acute attacks, short-term prevention, and long-term prophylaxis. The 26-year-old woman with food and medication allergy presents with thirteen-year history of recurrent abdominal pain diffuse and associated with diarrhea, nausea and hands, lips and eyelids swelling. During this period, she did several exams and six laparoscopies that only revealed a small amount of free intraperitoneal fluid. Biochemical testing performed at that time revealed the C1 esterase inhibitor, decreased protein level, and C4 level, and she was, then, diagnosed in adults with intestinal involvement with HAE. After the adequate treatment and prophylaxis, she evolved with reduction of the number of attacks. The late diagnosis is associated with high morbidity. Therefore, it is extremely important the recognition and investigation of HAE with involvement in intestinal patients with recurrent attacks of unexplained abdominal pain.

ACE inhibitor‐associated intestinal angioedema in orthotopic heart transplantation

Angiotensin‐converting enzyme inhibitor induced angioedema commonly involves the head and neck area. We report a case of angiotensin‐converting enzyme inhibitor induced intestinal angioedema in a heart transplant recipient on mTOR immunosuppression. A 36‐year‐old Caucasian woman with history of heart transplantation on sirolimus, tacrolimus and prednisone presented to the Emergency Department with abdominal pain, one day following lisinopril initiation. A computer tomography scan demonstrated diffuse bowel wall thickening consistent with pancolitis and edema. She was subsequently diagnosed with angiotensin‐converting enzyme inhibitor induced angioedema. Patients on mTOR immunosuppression are at higher risk for this potentially life‐threatening side effect. Knowledge of this interaction is critical for providers prescribing mTOR agents.

Isolated Angiotensin Converting Enzyme (ACE) Inhibitor Induced Small Bowel Angioedema After 10 Years of Oral Lisinopril Therapy

A 65-year-old male presented to the emergency room for one episode of “coffee colored” emesis and nausea 5 hours prior to admission. He has a history of Vitiligo and hypertension for which he has been taking Lisinopril for 10 years. He has had occasional abdominal pain over the last few years for which he has been placed on protonix. He has no prior surgical history and is otherwise healthy. On physical exam he had hypoactive bowel sounds, minimal generalized tenderness with no guarding or rebound. Laboratory studies revealed leukocytosis (18.6 k/uL) with neutrophilia (83%). An abdominal computed tomography (CT) scan revealed segments of edema and elongation of loops of small bowel with associated wall thickening consistent with the diagnosis. Isolated ACE Inhibitor angioedema of the small intestine is a rare phenomenon, with limited case representation in the literature. In the time period 1982-2010 there have been only 27 individual case reports documenting ACE inhibitor associated small bowel angioedema. Upon further review, in the period between 2011-2016 there were 17 additional case reports in the literature. The majority of cases reported were associated with Lisinopril (35) and Enalapril (4). In a retrospective study conducted on 20 patients from two separate facilities from 1996 through 2012 with clinical consistent with ACE inhibitor angioedema of the small bowel, 19/20 were taking Lisinopril, and one with Enalapril2. The average patient was a middle aged female presenting with acute abdominal symptoms including nausea, vomiting and abdominal pain with a mild leukocytosis. The diagnosis was made by CT of the abdomen and pelvis with characteristic finings of isolated small bowel wall thickening, edema, and straightening with abdominal ascites present1,2,3. 1 Vedra A. Augenstein, MD B. Todd Heniford, MD Ronald F. Sing, DO Intestinal Angioedema Induced by Angiotensin-Converting Enzyme Inhibitors: An Underrecognized Cause of Abdominal Pain? The Journal of the American Osteopathic Association March 2013 | Vol 113 | No. 3 2 Christopher D. Scheirey, Francis J Scholz, Michael J Shortsleeve, Douglas S. Katz. Angiotensin-Convertin Eznyme Inhibitor-Induced Small Boel Anioedema: Clinical and Imaging Findins in 20 Patients. American Journal of Roentgenology. 2011;197: 393-398. 10.2214/AJR.10.4451 3.Campbell T, Peckler B, Hackstadt RD, Payor A. ACE inhibitor induced angioedema of the bowel. Case Report Med. 2010;2010:1-4.Figure 1

Acquired Small Intestinal Angioedema Presenting as a Paraneoplastic Phenomenon Related to Lymphoma

Aim: To describe a case of acquired small intestinal angioedema presenting as a paraneoplastic phenomenon related to lymphoma. Case report: A 36 yo female presented with acute nondescript, upper abdominal pain. The pain was variably exacerbated by food intake and tensing her abdominal musculature. She was taking no medications. Physical exam revealed mild epigastric tenderness. Laboratory evaluation revealed pancytopenia, an elevated PT INR = 1.5 and a PTT =46.2 s (N=26.8-39.5). A CT scan suggested edema at the root of the mesentery and splenomegaly. The etiology of her pain was initially unclear and the gastroenterologist who evaluated the patient believed that the illness was unlikely to be related to a primary gastrointestinal disorder and the probability of a preeminent hematologic process was suggested. While a hematology consultation was sought, her pain escalated and a repeat CT showed the evolution of segmental jejunal mural thickening with submucosal edema (see Figure 1). The hematologist was immediately suspicious of the possibility of lymphoma with acquired angioedema of the small bowel. A bone marrow biopsy revealed involvement with B-cell lymphoma. A blood C4 level was 8 mg/dl (N= 9-36) and a blood C1 esterase inhibitor level was 12 mg/dl (N= 21-39). The patient was transferred to a tertiary center specializing in hematologic/oncologic care. She underwent left axillary lymph node biopsy, and a diagnosis of marginal zone lymphoma was confirmed. She was treated with rituximab and after 2 years, she remains in remission. Angioedema ordinarily involves the skin, respiratory and/or gastrointestinal tract. Since acquired angioedema related to C1 esterase deficiency and lymphoproliferative disease was initially reported nearly 40 years ago, the disorder has been associated with a variety of hematologic processes. When associated with a hematologic disease, angioedema can on rare occasion be associated with a normal C1 esterase level and auto antibodies which cause inactivation and qualitative dysfunction of this protein.Figure 1Conclusion: From the gastroenterological perspective, it should be noted that angioedema can affect multiple organs simultaneously, but it can involve the gastrointestinal tract alone, as in this case. When acquired angioedema occurs and is not associated with exposure to drugs and toxins, the scenario should raise the specter of an underlying hematologic disorder to avoid an unnecessary delay in diagnosis.

Isolated Intestinal Angioedema in the Emergency Department

BackgroundAngioedema is a condition that can cause cutaneous and mucosal edema of practically any part of the body. Isolated edema of the intestines is a rather rare manifestation, but it can cause important morbidity. Hereditary angioedema as well as certain medications can give rise to intestinal angioedema. We have seen a rise in frequency of intestinal angioedema since the advent of angiotensin-converting enzyme inhibitors. Ultrasound of the abdomen is an inexpensive, safe, and easy tool that can help in the differential diagnosis. Case ReportWe describe the case of a 25-year-old woman who presented with acute abdominal pain. She was diagnosed with intestinal angioedema due to hereditary angioedema type I. Why Should an Emergency Physician Be Aware of This?Misdiagnosis can lead to administering the wrong treatment or even unnecessary surgical interventions. Intestinal angioedema is best treated with purified C1-inhibitor, icatibant, or ecallantide. Fresh frozen plasma is to be avoided because it carries the risk of worsening the symptoms.

Recurrent Isolated Intestinal Angioedema Associated With Splenic Marginal Zone Lymphoma

A 61-year-old woman with history of IgM monoclonal gammopathy presented for evaluation of episodic acute, non-radiating peri-umbilical and epigastric abdominal pain. Each episode last for 4-5 days, and symptoms only were relieved with opioid medications. Initial abdominal CT scan revealed an enlarged spleen (18cm). Laboratory evaluation showed elevated transaminases (AST 53 U/L, ALT 64 U/L) with normal alkaline phosphatase and bilirubin with negative AMA, ASMA, and LKM1 antibodies. Complement C3 level was normal, and C4 was decreased to less than 10 mg/dl during the pain episode. There was no evidence of Budd-Chiari syndrome or portal hypertension on abdominal Doppler or upper endoscopy. Almost two years after the first presentation, she was noted to have recurrent abdominal pain prompting abdominal ultrasound, which showed ascites with a spontaneous decline in ascites volume over 5 days. This ascetic fluid was not sampled by paracentesis due to scant amounts. CT imaging findings confirmed the presence of small amount of ascites and marked bowel wall thickening during two separate acute abdominal pain episodes. Liver biopsy demonstrated bridging fibrosis. In light of her slowly increasing IgM gammopathy, persistent splenomegaly and progressive colicky abdominal pain, peripheral blood flow cytometry and bone marrow biopsy were performed. Findings supported a diagnosis of splenic marginal zone lymphoma. She received 8 cycles of chemotherapy (rituximab, cyclophosphamide, vincristine and prednisone). Abdominal pain episodes continued while she was on chemotherapy, but the final episode occurred 3 weeks after completing chemotherapy. C1-esterase function and C1q were subsequently found to be normal after resolution of the pain, but the patient was noted to have C4 complement level of less than 3 mg/dl. In the presence of bowel wall thickening, ascites and low C4 complement level during pain episodes, we suspected acquired angioedema (AAE) in the setting of splenic marginal zone lymphoma as the cause for her symptoms. This entity has previously been reported. The patient denies a family history of angioedema and has never experienced cutaneous symptoms. Our suspicion for intestinal AAE was confirmed after she remained asymptomatic following lymphoma treatment. AAE is a rare condition manifesting with recurrent episodes of angioedema and may accompany an underlying lymphoproliferative disorder. Gastrointestinal attack is reported in less than 50% of patients with AAE. Clinical awareness of this entity as a cause for acute abdominal pain may improve the quality of care for these patients.

A Case of Eosinophilic Gastroenteritis Initially Misdiagnosed as Idiopathic Intestinal Angioedema

A Case of Eosinophilic Gastroenteritis Initially Misdiagnosed as Idiopathic Intestinal Angioedema

Isolated Intestinal Involvement of Angioedema Induced by ACE Inhibitor

Purpose: 58-year-old obese African-American woman with metabolic syndrome presents to our hospital with progressively worsening acute on chronic non-radiating periumbilical pain with abdominal distension, nausea and non-bloody emesis, which has progressed over the past six months. Physical exam was notable for an uncomfortable afebrile black woman, normotensive, with tachycardia and tachypnea. She had no peripheral rash or edema, no mucosal edema, and had clear lung fields on auscultation. Her abdomen had no surgical scars, was obese and distended with hypoactive and high pitched bowel sounds, and was diffusely tender with guarding and rebound tenderness. Rectal exam revealed brown guaiac negative stool in an empty rectal vault. Laboratory investigation revealed a normal complete blood count, comprehensive metabolic panel, amylase, lipase, and slightly elevated lactate at 4.2 mmol/L. Contrast enhanced CT abdomen was revealed contiguous distal jejunal to ileal small bowel enhancement and thickening with associated small bowel dilation of the affected areas without a transition point of obstruction. In light of her presentation and risk factors for vascular disease, a CT angiogram was performed 6 hours after her initial CT abdomen to evaluate for mesenteric ischemia. Remarkably, this exam revealed complete resolution of bowel wall enhancement, thickening, and dilation, along with patent mesenteric arteries. During this time, patient's clinical symptoms also completely resolved. Upon reviewing patient's history, patient started having symptoms after initiation of lisinopril approximately 9 months prior to presentation. C1-esterase, C1q, and C3 complement levels were subsequently found to be normal. Our suspicion for ACE inhibitor induced intestinal angioedema was confirmed after patient remained asymptomatic with discontinuation of lisinopril. ACE inhibitor induced angioedema typically involves the extremities, face, and upper airways. It is caused by quantitative or qualitative deficiency of C1-esterase protein, which inhibits vasodilators such as bradykinin. Concomitant gastrointestinal involvement is common; however, it is rarely the sole manifestation of this disease. This disease carries a high mortality if untreated and may even lead to unnecessary surgical exploration if misdiagnosed. As such, clinical awareness of this entity as a cause of acute abdomen is paramount in providing the best care for these patients. Conclusion: Isolated intestinal involvement of ACE-inhibitor induced angioedema can also occur without typical features of facial or extremity swelling. Knowledge of such manifestation can improve patient outcomes.

Differential Diagnostic Aspects Are Lacking

Acute abdomen requires emergency treatment, including emergency diagnostic evaluation. In the introduction of their review article, the authors say that “While […] around 1% of all patients with acute abdomen have AMI [acute mesenteric ischemia], AMI is the cause of acute abdomen in up to 10% of patients aged over 70”, without explaining differential diagnostic aspects. Intestinal angioedema with massive mucosal swelling presents with similar symptoms; this seems to be neither more common nor rarer than AMI. Intestinal angioedema can have allergic triggers but can also be caused by taking angiotensin converting enzyme inhibitors and angiotensin II receptor blockers (many cases have been documented in the literature) or be provoked by non-steroidal anti-inflammatory drugs. An individual case of intestinal angioedema has been described in a patient after taking a calcium antagonist (1). Hereditary angioedema (HAE, a defect in C1-INH synthesis), angioedema with normal concentrations of C1-esterase-inhibitor but functional insufficiency, and angioedema based on acquired C1-esterase-inhibitor deficiency (acquired angioedema, AA) as well as idiopathic angioedema should also be included in the differential diagnostic evaluation. While a patient’s medical history can provide an early indication of angioedema due to medication, diet, or hereditary causes and thus prevent unnecessary laparotomy, hereditary angioedema and other forms need to be confirmed using specific laboratory-based examination. In case of clinical suspicion, this should include immunological determination of C1-INH, functional determination of C1-INH, measurement of C1, C2, C4, CH50, and, if required, measurement of autoantibodies against C1-INH (2). Adding intestinal angioedema as a differential diagnosis to the well-known range of causes for acute abdomen might be worth mentioning, in order to clearly differentiate from vascular-ischemic causes.

Unnecessary surgery for acute abdomen secondary to angiotensin-converting enzyme inhibitor use

Acute abdominal pain is the reason for 5% to 10% of all emergency department visits. In 1 in every 9 patients, operated on for an acute abdomen, laparotomy is negative. In a minority of patients, the acute abdomen is caused by side effects of medication. We present a case of unnecessary abdominal surgery in a patient with acute abdominal pain caused by intestinal angioedema (AE), which was eventually due to angiotensin-converting enzyme inhibitor (ACE-i) use. We hope that this case report increases awareness of this underdiagnosed side effect. Emergency department physicians, surgeons, internists, and family physicians should always consider ACE-i in the differential diagnosis of unexplained abdominal pain. Since early withdrawal of the medication causing intestinal AE can prevent further complications and, in some cases, needless surgery, we propose an altered version of the known diagnostic algorithm, in which ACE-i and nonsteroidal anti-inflammatory drugs–induced AE is excluded at an early stage.

Many faces of angioedema

Angioedema of the intestinal tract is an infrequent but well-described cause of abdominal pain that can occur because of inherited, acquired, allergic, or drug-induced causes. Hereditary angioedema (HAE) is a genetic disorder that causes recurrent attacks of severe edema of various body parts, including the intestinal tract. Moderate to severe abdominal pain occurs in 43-93% of such attacks due to intestinal edema. Laryngeal edema is a potentially life-threatening manifestation. Failure to recognize and diagnose HAE or other causes of intestinal angioedema can lead to years of delay in diagnosis, and in the case of HAE, often to unnecessary abdominal surgeries. Recognizing the typical history of recurrent attacks of abdominal pain, oropharyngeal/laryngeal angioedema or cutaneous angioedema, family history of similar symptoms, association of attacks with stress or menses, and exacerbation of attacks after administration of estrogens or angiotensin-converting enzyme inhibitors will increase diagnostic accuracy. Interdisciplinary treatment is often necessary after the diagnosis of HAE, first with acute management in the emergency room or the intensive care unit, followed by either drug prophylaxis against future attacks using a C1-esterase inhibitor concentrate or attenuated androgens and discontinuation of medications known to trigger attacks. Newer drugs approved for treatment of acute attacks may have future roles in the prevention of attacks if further studies support their efficacy. Gastroenterologists in particular should maintain a high index of suspicion for the possibility of HAE or other causes of intestinal angioedema in patients with a history of recurrent abdominal pain.