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Atypical Presentation Of Crohn's Disease

Vol. 12 •Issue 9 • Page 16
Clinical Case Report

Atypical Presentation Of Crohn's Disease

A 25-year-old white man presents to the emergency department on April 15 with a history of worsening hematochezia, cramping and abdominal pain. He had been seen in the office four days previously, on April 11. At that time, the workup showed a hemorrhoid at 12 o'clock on digital rectal examination. The patient had heme-positive stools and was placed on Anusol-HC, Pepcid and Propulsid.

Today, he reports lethargy and weakness. In addition, the patient has had in excess of five bloody stools that he describes as bright red. He denies any melena and denies any fever or chills.

Medical and Family History

The patient's history is significant for beta-thalassemia minor; he has had no episodes of anemia. Otherwise his medical history is unremarkable; he denies hypertension, diabetes mellitus or coronary artery disease. He has had no positive tuberculin testing or blood transfusions.

His family history is significant in that his father died at age 34 of an unknown pathology. His grandfathers on both sides are positive for coronary artery disease, peripheral vascular disease and cerebrovascular accident, and he has a significant family history of hypertension.

The patient is married and is a student. He denies alcohol and tobacco abuse.

Review of Systems and Exam

Review of symptoms is generally unremarkable except for a few areas: He reports fatigue, lethargy and progressive weakness. He denies weight loss or weight gain and denies fever or chills. Although he denies trouble swallowing, heartburn, vomiting or indigestion, he indicates a change in bowel habits that has been progressive for the past several months, notable for a change in frequency—he reports often experiencing up to five bowel movements per day. Movements occasionally contain gross, bright-red blood. Movements are accompanied by bloating and gas. He also indicates a diffuse lower-abdominal pain.

The physical examination reveals a healthy-appearing man in mild distress. Positive bowel sounds occur at 34 per minute. The abdomen is soft and flat, with no palpable hepatomegaly or splenomegaly. Slight right-upper-quadrant tenderness is present. Murphy's sign is negative. Bilateral lower-quadrant tenderness is present that does not elicit true peritoneal signs. There is no rebound tenderness or guarding. The physical exam is otherwise unremarkable.

Operative Procedure

The patient was admitted. The next day, April 16, a colonoscopy was performed. The patient was found to have four distinct lesions of active colitis in the mid- to distal-transverse colon. Lesions included pseudopolyps, aphthous ulcers and fissures with purulent fluid.

Biopsies were obtained of all four regions. The colon, beyond the mid-transverse colon to the cecum, was entirely normal. The segment of the colon from the splenic flexure to the rectum was found to be normal, as well. No mucosal lesions were found. The scope was removed. The patient tolerated the procedure well and was transferred to the recovery room in stable condition.

Pathology

Specimen 1: Transverse colon. Nonspecific active acute and chronic colitis with focal micronecrosis.

Specimen 2: Transverse colon. Nonspecific active acute and chronic colitis with focal necrosis and ulceration.

Specimen 3: Transverse colon. Nonspecific active acute and chronic colitis with focal necrosis and ulceration. Ulcer base with focal involvement of medium-sized blood vessels.

Specimen 4: Transverse colon. Nonspecific active acute and chronic colitis with focal necrosis and ulceration.

The histology of all four specimens is similar and does not fit the usual histologic picture of ulcerative colitis or ischemic colitis. Location of the ulcers is unusual for the initial manifestation of Crohn's disease; however, the histology does not exclude the possibility. Similar ulcers may be seen with the use of nonsteroidal anti-inflammatory drugs. A careful drug history and stools for ova and parasites might be helpful in determining etiology of the ulcers.

Diagnosis

Differential diagnosis includes colon disease; ulcerative colitis; ischemic colitis; enteric pathogens (amebiasis, tuberculosis, Yersinia, Campylobacter, Gonorrhea, Clostridium difficile toxin, Shigella, Salmonella, lymphogranuloma venereum (LGV) and non-LGV Chlamydia, fungi); malignancies such as lymphoma; adenocarcinoma; caustic enemas (e.g., hydrogen peroxide); small-bowel disease; eosinophilic gastroenteritis; and various vascular pathologies such as superior mesenteric venous thrombosis, collagen vascular disease, solitary rectal ulcer syndrome (localized colitis cystica profunda). Also considered in the differential diagnosis are drug-related causes (e.g., from NSAIDs, oral contraceptives, gold, pancreatic enzyme supplements in cystic fibrosis) and immune-mediated causes such as congenital immunodeficiency, X-linked agammaglobulinemia, common variable immunodeficiency, glycogen storage disease type Ib, chronic granulomatous disease, Turner's syndrome and Hermansky-Pudlak syndrome.

The patient eventually was diagnosed with Crohn's disease of the transverse colon, along with lower gastrointestinal bleed.

Treatment and Management

The patient was transfused with two units of A-positive whole blood on April 16. He was placed on prednisone 60 mg per day for three days, followed by 40 mg per day for three days, followed by 20 mg per day for three days. He was also placed on sulfasalazine 500 mg by mouth four times a day and iron ferrous gluconate 325 mg by mouth twice a day.

Upon discharge on April 17 the patient remained asymptomatic. Less than four months later, on August 8, the patient presented intermittent hematochezia and abdominal cramping to a different physician.

A full gastrointestinal workup, including an esophagogastroduodenoscopy (EGD), small bowel series and colonoscopy, was performed at that time. The EGD and small bowel series were negative; however, the colonoscopy revealed an acute exacerbation of Crohn's disease, the cause of which was suspected to be inadequate initial steroid therapy. The patient was subsequently placed on prednisone for one year and mesalamine for chronic therapy.

An Atypical Presentation

In this case, the patient only exhibited a few of the traditional signs, symptoms and clinical findings associated with Crohn's disease. The patient presented with an acute GI bleed, which is seen in only 20% of patients with Crohn's disease. In this case, the disease's onset was acute and severe, which sharply contrasts to the typical insidious onset of Crohn's. Additionally, the patient did not experience weight loss traditionally associated with Crohn's disease. The histology of all four specimens taken at the time of colonoscopy was similar and did not fit the usual histologic picture of ulcerative colitis or ischemic colitis. The location of the ulcers is unusual for the initial manifestation of Crohn's disease.

Crohn's Disease

Crohn's disease is an inflammatory bowel disease marked by patchy areas of full-thickness inflammation anywhere in the gastrointestinal tract, from mouth to anus. It frequently involves the terminal ileum of the small intestine or the proximal large intestine and may be responsible for abdominal pain, diarrhea, malabsorption, fistula formation between the intestines and other organs and bloody stools. The clinical course of Crohn's disease is often difficult to predict. Some patients experience low morbidity rates, while others experience high morbidity rates and require surgery for relief of symptoms. Active Crohn's disease can be managed in several ways: pharmacologic therapy, surgical therapy (bowel resection) or a combination of the two. Crohn's disease is an idiopathic disease with no known cure; therefore, treatment involves management of symptoms the disease manifests.

Crohn's disease is mediated by T lymphocytes, which arise in genetically susceptible individuals as a result of a breakdown in the regulatory constraints on mucosal immune responses to enteric bacteria. Regulation of immune reactivity to enteric antigens has improved understanding of the pathophysiologic mechanisms of Crohn's disease and has expanded therapeutic options for patients with the disorder. Disease heterogeneity is probable, with various underlying defects associated with a similar pathophysiologic outcome. Although most conventional drug treatments are directed at modification of host response, therapeutic manipulation of the enteric flora is becoming a realistic option.

David Robert DeWalch is a surgical PA at East Texas Vascular Associates in Tyler, Texas. Todd C. Frank is a professional writer and editor in Houston.

In July-August's "Clinical Case Report" on an MRSA pituitary abscess complicating posttraumatic pituitary apoplexy, the accompanying computed tomography images and their captions did not match. For a corrected version of the article and its figures, go to www.ADVANCEweb.com/pa.




     

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