Case Reports in Immunology / 2015 / Article / Tab 3

Case Report

Hereditary Angioedema and Gastrointestinal Complications: An Extensive Review of the Literature

Table 3

Clinical presentation of HAE abdominal attacks in comparison to other gastrointestinal disorders.

DisorderSign/symptomLaboratory dataDistinguishing features

HAE abdominal attacksNausea, vomiting, diarrhea, crampy abdominal painType 1 HAE: low C4 and C1 inhibitor level/activity, normal C1q level [3]
Type 2 HAE: normal C1 inhibitor level, low activity, normal C1q level [3]
History of HAE, colonoscopy: massive segmental mucosal edema [13]
CT abdomen: intestinal edema, ascites, dilated loops
Attacks usually begin in childhood, but they can occur at any age [34]

Acute diverticulitisAcute LLQ abdominal pain and tenderness, fever, anorexia, nausea, vomiting, constipation or loose stoolsMild to moderate leukocytosisLLQ palpable abdominal mass
CT abdomen: evidence of colonic diverticula, wall thickening, pericolic fat infiltration, abscess formation or extraluminal air or contrast. At age of 40 less than 5% are affected, at age of 60 that number is 30%, and by age of 80 50–65% of adults are affected [40]

Acute appendicitisPeriumbilical abdominal pain that migrates to RLQ with noted rebound tenderness, anorexia, nausea, vomiting, feverLeukocytosis with neutrophils >70%, elevated levels of CRP, SAA, ProCT [41]Peak incidence occurs at age 10–19 years [42],
Alvarado Score >7 meets criteria for surgical appendectomy
CT abdomen: enlarged appendix diameter >6 mm, appendiceal wall >2 mm thick, inflammatory compression of adjoining adipose tissue, RLQ abscess formation, calcified appendicolith [43]

Small bowel obstruction (SBO)Diffuse abdominal pain, colicky with waxing/waning characteristic, nausea, vomiting, abdominal distention and tenderness, hyperactive or hypoactive bowel sounds, feculent emesis
Peritonitis should be suspected when rigidity, rebound tenderness, or guarding presents [44]
Leukocytosis, hemoconcentration, electrolyte imbalanceMost common in adults with history of abdominal surgery raising suspicion for peritoneal adhesions (75% cases); the second most common cause is hernias
Plain film of abdomen displays air-fluid levels, small bowel distention and paucity of air in rectal vault [44]
Passage of stool and flatus do not rule out SBO [44]
CT abdomen with contrast is diagnostic method of choice

PancreatitisAcute onset of abdominal pain, located in epigastrium with radiation to back, nausea and vomiting, low grade fever, tachypnea, epigastric tenderness to palpationLeukocytosis, hemoconcentration with elevated hematocrit, elevated serum amylase and lipaseMost common occurrence in childhood between ages of 15 and 19 years
History of gallstones or alcohol abuse
CT abdomen with IV contrast is recommended when suspecting pancreatic necrosis, worsening response to therapy, or questionable diagnosis [44]

Inflammatory bowel disease-ulcerative colitis (UC) and Crohn’s disease (CD) UC: bloody diarrhea, with symptoms of urgency and tenesmus [45]
CD: chronic or nocturnal abdominal pain, diarrhea
Weight loss, fever; rectal bleeding may or may not be present; extraintestinal manifestations including inflammation of eyes, skin, or joints [46]
Elevated acute phase reactants CRP, ESR
UC: p-ANCA positive
CD: ASCA positive, p-ANCA negative
Both: most frequently diagnosed in the second decade of life. Stool examination to rule out infectious etiology
UC: disease limited to colon. Sigmoidoscopy or colonoscopy: loss of vascular pattern, friability and ulceration
Biopsy: crypt atrophy, increase presence of lymphocytes and plasma cells at crypt bases [45]
CD: primarily involving distal ileum, though any part of alimentary tract may be involved in a transmural inflammatory pattern
Endoscopy: deep serpiginous ulcers and “cobblestone” appearance
Biopsy: granulomas noted on specimen [47]

Intussusception Abdominal pain, nausea, vomiting, diarrhea, hematochezia [48]Similar to bowel obstruction: leukocytosis, hemoconcentration, electrolyte imbalancePeak age at presentation is 4–8 months
History of tumor or prior abdominal surgery
CT abdomen: “target sign” indicative of intussusception [49]

Celiac diseaseAbdominal discomfort, weight loss, diarrhea, increased flatusIron and folate deficiency, steatorrhea, hypoalbuminemia, hypocalcemia, elevated serum transaminases May manifest as early as childhood after introduction of gluten in diet
Positive serologic testing serum IgA anti-tissue transglutaminase and IgA anti-endomysial antibody have sensitivities of 80–95% and specificities of 95–99%. Mucosal intestinal biopsy showing blunted villi, hyperplastic crypts with increased number of mitotic figures [50]

HAE: Hereditary Angioedema, LLQ: left lower quadrant, RLQ: right lower quadrant, CRP: C-reactive protein, ESR: Erythrocyte Sedimentation Rate, SAA: Serum Amyloid A, ProCT: serum procalcitonin, p-ANCA: perinuclear antineutrophil cytoplasmic antibodies, and ASCA: anti-Saccharomyces cerevisiae.

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