A 61-Year-Old Man With Crampy Abdominal Pain
A 61-year-old white man presents to the emergency department with a 1-week history of diffuse "crampy" abdominal pain. For the past 2 months, he has had diarrhea and has been passing blood with every bowel movement, either on the stool surface or separately from the stool. Recently, he has had 10-15 loose stools per day, with associated urgency and tenesmus. He has lost 4 lb in the past week. He feels lightheaded upon standing and is experiencing fatigue and mild shortness of breath. He takes atorvastatin for hypercholesteremia and acetaminophen for intermittent back and knee pain. He is allergic to penicillin. He has no history of gastrointestinal tract disease; screening colonoscopy performed 2 years ago was unremarkable. His father died of myocardial infarction when he was in his 60s, and his mother died of breast cancer when she was in her 40s. He has no siblings. He is retired from his work as an accountant and lives with his wife. He has not travelled recently. He has a 25-pack-year smoking history but has not smoked for approximately 10 years. He drinks alcohol on social occasions and on weekends, and he denies illicit drug abuse.
On physical examination, the patient is a pale, diaphoretic man in some distress due to abdominal pain. His oral temperature is 101.5°F (38.6°C), blood pressure is 110/62 mm Hg, pulse is 107 beats/min, and respiratory rate is 24 breaths/min. His body mass index is 29.7 kg/m
2. Examination of his head and neck is unremarkable aside from pale mucosal membranes. Lung auscultation reveals normal breath sounds, with no crackles or rhonchi. His heart rate is tachycardic, and his heart rhythm is regular. There are no murmurs or extra heart sounds. His abdomen is distended and firm, with diffuse rebound tenderness that seems worse in the lower left quadrant. Bowel sounds are diminished. No hepatosplenomegaly, ascites, or palpable masses are appreciated. Digital rectal examination demonstrates normal rectal tone, "velvety" rectal mucosa, and bright red blood on withdrawal of the examining finger. There is pitting edema of the legs and feet.
Laboratory analysis includes a complete blood count (CBC), which demonstrates anemia (hemoglobin concentration of 6.7 g/dL [67 g/L]), leukocytosis (leukocyte count of 14.2 x 10
3 cells/μL [14.2 x 10
9 cells/L]), and thrombocytosis (platelet count of 540 x 10
3 cells/µL [540 x 10
9 cells/L]). Additional findings include elevations in the erythrocyte sedimentation rate (78 mm/h) and C-reactive protein level (114 mg/L). His albumin level is low, at 2.0 g/dL (20 g/L). The basic metabolic panel is unremarkable. Repeat stool cultures and samples for ova and parasites are negative. Flexible sigmoidoscopy shows diffusely erythematous and friable colonic mucosa with large ulcerated patches, abundant yellow-white exudate, and oozing blood. These findings extend in a proximal and continuous fashion from the rectum to at least the middle of the sigmoid colon. Tissue biopsy samples sent for histopathologic examination reveal lymphoplasmacytic inflammation along the base of the crypts; neutrophils that infiltrate the crypt epithelium and collect in the depths of the colonic crypts to form "crypt abscesses"; and focal chronic reactive changes, including crypt branching, gland atrophy, and goblet-cell mucin depletion. No granulomas are identified.
What is the most likely diagnosis?
Hint: Note the frequent passage of bloody loose stools for more than 2 months in the setting of fever, tachycardia, and anemia.
The diagnosis of fulminant ulcerative colitis, an idiopathic form of inflammatory bowel disease, was initially suspected on the basis of the patient's history, physical examination, and laboratory test results; endoscopic and biopsy findings confirmed the diagnosis. The rectal bleeding was bright red, indicating that the source of the bleeding was most likely the lower gastrointestinal tract. The concurrent presence of crampy abdominal pain, diarrhea, tenesmus, and fever suggested that an inflammatory process had damaged the colonic mucosa. The unremarkable colonoscopy findings 2 years before presentation rendered a neoplasm unlikely. Although not impossible, it is rare for colon cancer to present with diarrhea. Most compelling was the continuity and character of the mucosal damage identified on endoscopy and the histopathologic findings, which were diagnostic of ulcerative colitis. Symptom duration suggested a chronic inflammatory process and infectious causes of colitis had been ruled out.
Ulcerative colitis is estimated to respectively have an incidence of 7.3 and prevalence of 116 per 100,000 people in the United States.
[1] The peak age at onset is 15-25 years, with a second peak at 40-60 years.
[2] Individuals of Ashkenazi Jewish or Scandinavian descent are more often affected, with men and women experiencing a similar disease incidence. Smoking seems to play a protective role against the development of ulcerative colitis, and it has been proposed that the second incidence peak in part represents patients who stopped smoking at a later age.
[3] Most research suggests that the etiology and pathogenesis of ulcerative colitis are multifactorial, with a combination of genetic susceptibility, bacterial antigens, and alteration of mucosal immunity responsible for the development of the disease.
[4]
Histopathologically, ulcerative colitis is characterized by lymphoplasmacytic inflammation of the mucosa and submucosa, with scattered neutrophils in the lamina propria (Figure 1). The neutrophils typically injure the crypt epithelium and may collect in the base of the crypts, forming crypt abscesses (Figure 2). Within weeks of disease onset, features of crypt architectural distortion (such as crypt branching and shortening) develop; these chronic changes are a nonspecific regenerative response to previous injury. Deep granulomas and transmural inflammation, 2 hallmarks of Crohn's disease, do not occur.
[5]
Classically, ulcerative colitis is insidious in onset. Affected patients present with frequent passage of bloody, loose stools and tenesmus. The intensity of the symptoms generally correlates with the extent of anatomic involvement, allowing classification of disease as mild, moderate, or severe/fulminant. The majority of patients have mild, indolent disease limited to the rectum and sigmoid colon that is characterized by diarrhea, intermittent rectal bleeding, and tenesmus. The physical examination is often normal aside from bright red blood within the rectum. Other patients present with systemic symptoms, including more frequent bowel movements, crampy abdominal pain, decreased bowel sounds, high-grade fever, tachycardia, anemia, orthostatic hypotension, and weight loss. Extraintestinal manifestations, such as acute arthropathy, episcleritis, erythema nodosum, and pyoderma gangrenosum may also arise. Fewer than 10% of patients with ulcerative colitis initially present with fulminant disease, with older individuals represented in greater numbers. Fulminant ulcerative colitis is more abrupt in onset and is usually characterized by extensive colonic involvement ("pancolitis"), with rectal bleeding that may be extensive enough to necessitate blood transfusion. Abdominal distention and tenderness to palpation with signs of peritoneal inflammation (eg, rebound tenderness) may be observed in these patients. Of greatest concern in patients with fulminant disease is the prospect of massive hemorrhage, toxic megacolon, or bowel perforation. Immediate hospitalization is often necessary in these patients.
The differential diagnosis of bright red blood in the rectum associated with diarrhea includes infectious colitis, ischemic colitis, and nonsteroidal anti-inflammatory drug enteropathy. Other causes of colonic bleeding, such as diverticular disease, can present with loose stools, because blood is a cathartic. Stool studies (eg, fecal leukocytes, culture, ova, and parasites) and a
Clostridium difficile toxin screen should be considered to eliminate the possibility of infectious colitis. A complete blood cell count should be obtained to evaluate for anemia or leukocytosis. Low albumin levels signify poor nutritional status and protein-losing enteropathy. A basic metabolic panel may demonstrate electrolyte abnormalities in the setting of severe prolonged diarrhea. Inflammatory markers, such as the erythrocyte sedimentation rate and C-reactive protein level, are typically elevated in patients with inflammatory bowel disease. Radiographic studies may be used as an adjunct in diagnosing complications of ulcerative colitis (eg, plain films can establish the presence of toxic megacolon). In the setting of acute flares, other radiologic studies, such as computed tomography (CT), may be done to evaluate for an alternative diagnosis. Common CT findings in ulcerative colitis include thickening of the colonic wall, pericolonic fat stranding, and a "target" appearance of the rectum. Most important in confirming the diagnosis of ulcerative colitis is flexible sigmoidoscopy with biopsy; colonoscopy may also be used in some settings, but it is associated with a higher risk for perforation and is contraindicated in cases of suspected toxic megacolon. Typical endoscopic findings in ulcerative colitis patients include diffuse erythema; edema; friability; and granularity of the mucosa, with loss of the normal vascular pattern. Ulceration with exudate and pseudopolyps are frequently identified as well. These findings invariably begin in the rectum and extend proximally in a continuous manner, up to and including the cecum, in cases of pancolitis.
[6]
In most cases, ulcerative colitis can be managed in the outpatient setting. Treatment is designed to achieve and maintain disease remission. Topical therapy and 5-aminosalicylic acid (ASA) agents are the first-line means of treating ulcerative colitis, with steroids for acute flares and immunomodulators as maintenance therapy in severe refractory disease. Fulminant ulcerative colitis requires careful attention to the development of toxic megacolon. Parenteral corticosteroids; rehydration; bowel rest; nutritional supplementation; anticoagulation with low-dose heparin to prevent venous thrombosis, which is common in patients with ulcerative colitis; and monitoring of hemoglobin values (with transfusion sometimes required) are recommended. If remission is achieved, a maintenance regimen consisting of immunomodulators (such as cyclosporine, 6-mercaptopurine, or azathioprine) and/or 5-ASA should be initiated. In patients with fulminant ulcerative colitis that is refractory to first-line therapy, infusion of a biologic agent (such as infliximab) or colectomy may be necessary.
[7]
The patient in this case was immediately admitted to the hospital because of concerns that he was at risk for massive hemorrhage, toxic megacolon, or bowel perforation. Three units of cross-matched blood were transfused, and he was prescribed bowel rest, peripheral hyperalimentation, and prednisolone. Shortly thereafter, 5-ASA was added to his therapeutic regimen. His symptoms resolved gradually, and he was discharged to home. Over the next few years, he experienced several disease flares, and the benefits and risks of immunomodulators and biologic agents versus colectomy were reviewed with him during a particularly severe flare. After considerable reflection, he decided in favor of colectomy; the patient tolerated the operation well. Gross examination of the colectomy specimen demonstrated involvement of the entire colon from cecum to anus. Multiple irregular ulcers with an associated yellow-white exudate were scattered throughout the colon, and diffuse pseudopolyps (Figure 3) were present. Findings consistent with Crohn's disease (eg, fissures, fistulas, perianal involvement, "creeping fat," and segmental disease) were not identified. As expected, the procedure resulted in complete remission of the patient's symptoms. After ileal pouch anal anastomosis surgery, most patients can expect to have 4-6 loose bowel movements per day.
Q1. You are caring for a patient with abdominal pain, fever, and hematochezia and are concerned that the patient has ulcerative colitis. Which finding is most consistent with a diagnosis of ulcerative colitis?
| | |
|
| Pseudopolyps | Correct Answer |  41% |
| Segmental disease ("skip lesions") | | 15% |
| Perianal involvement | | 12% |
| Transmural inflammation | | 25% |
| Deep granulomas | | 7% |
Pseudopolyps are a common alteration of the colonic mucosa in patients with long-standing active ulcerative colitis; they are raised areas of inflamed, regenerative tissue arising in a background of ulceration. When present in significant numbers, pseudopolyps can cause cobblestoning of the mucosa that does not regress, even with treatment. Segmental disease ("skip lesions"), perianal involvement, transmural inflammation, and granulomas are all manifestations of Crohn's disease, another form of inflammatory bowel disease.
Q2. You are caring for a patient with known ulcerative colitis. Which of the following clinical manifestations is more worrisome for severe or fulminant ulcerative colitis?
| | | |
| Hematochezia | | 31% |
| Diarrhea | | 6% |
| Sacroiliitis | | 7% |
| High-grade fever | Correct Answer | 50% |
| Tenesmus |
| 7% |
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