CASE CHALLENGE
A 38-Year-Old Woman with Abdominal Pain and Fever
Case
The case description for a Case Records of the Massachusetts General Hospital appears below. What is the diagnosis? What diagnostic test is most likely to be helpful? Cast your vote on the diagnosis and submit a comment about what diagnostic test is indicated. The correct diagnosis, along with the full description of the case and the procedures performed, will be published in the June 20, 2019, issue of the Journal.
A 38-year-old woman with a history of Crohn’s disease was admitted to the hospital because of abdominal pain and fever. Despite appropriate medical treatment, she had progressive worsening of clinical symptoms. What is the most likely diagnosis?
Presentation of Case
Dr. Erika J. Parisi (Medicine): A 38-year-old woman with a history of Crohn’s disease was admitted to this hospital because of abdominal pain and fever.
The patient had been in her usual state of health until 3 years before the current admission, when nausea, vomiting, diarrhea, and pain in the right upper quadrant developed. She was evaluated in the emergency department of another hospital. The right upper quadrant was tender on palpation, and the remainder of the physical examination was normal. Computed tomography (CT) of the abdomen and pelvis revealed mild dilatation and fecalization of the distal small intestine. A diagnosis of small-bowel obstruction was suspected, and the patient was admitted to the hospital for bowel rest and administration of intravenous fluids. On the second hospital day, all the symptoms had resolved, and the patient was discharged home with a presumed diagnosis of viral gastroenteritis.
One month later, the patient was evaluated in a gastroenterology clinic affiliated with a second hospital. She reported ongoing bloating, intermittent abdominal pain on the right side, and two or three bowel movements per day with loose stools without blood or mucus. She had no weight loss or fever. Physical examination was normal. CT with small-bowel enterography revealed mild circumferential wall thickening of the proximal ascending colon and distal ileum and prominent lymph nodes in the right lower quadrant. Colonoscopy was notable for erythematous, granular, hemorrhagic, ulcerated mucosa in the proximal ascending colon and cecum. Biopsy specimens of the ileum and ascending colon showed normal mucosa; a biopsy specimen of the cecum showed evidence of active chronic colitis that was consistent with Crohn’s disease. Mesalamine therapy was initiated, with a decrease in the bloating and abdominal pain but no change in the frequency of bowel movements.
Table 1.
Laboratory Data.
Figure 1. Colonoscopic Images.
Images obtained during colonoscopy performed 7 months before the current admission, before the initiation of anti–tumor necrosis factor therapy, show a normal terminal ileum (Panel A) and inflammation involving primarily the cecum, with a patulous ileocecal valve (Panel B).
Figure 2. Imaging Studies of the Abdomen.
Images obtained during magnetic resonance enterography performed 3 months before the current admission (Panels A and B) show thickening and hyperenhancement of the terminal ileum, cecum, and ascending colon. The coronal, T1-weighted, contrast-enhanced image (Panel A) shows prominent mesenteric lymph nodes (arrow), and the axial, T2-weighted, fat-suppressed image obtained at the level of the cecal pole (Panel B) shows marked pericolonic edema (arrow). In addition, CT of the abdomen and pelvis was performed at the time of the current presentation, after the administration of intravenous contrast material. A coronal, reformatted image (Panel C) shows persistent, contiguous thickening of the terminal ileum, cecum, and ascending colon (arrow), and an axial image obtained at the level of the cecum (Panel D) shows pericolonic stranding, edema, and a prominent mesenteric lymph node (arrow).
Seven months before the current admission, the patient was evaluated in a gastroenterology clinic affiliated with this hospital. She reported postprandial bloating and epigastric pain, as well as two or three bowel movements per day with hard stools and associated straining. Physical examination was normal. The fecal calprotectin level was 243 μg per gram (reference range, <50). An interferon-γ release assay for Mycobacterium tuberculosis was negative. The blood lipase level was normal, as were the results of liver-function tests. Other laboratory test results are shown in Table 1. Results of esophagogastroduodenoscopy were normal, but gastric biopsy specimens showed evidence of Helicobacter pylori gastritis. Colonoscopy (Figure 1) revealed shallow ulcerations, edema, and friability in the cecum and a segment of the ascending colon. Findings on examination of a biopsy specimen of the cecum were interpreted as consistent with severe active colitis, with marked ulceration but without dysplasia or granulomas; immunohistochemical staining for cytomegalovirus was negative. The H. pylori gastritis was treated with lansoprazole, amoxicillin, and clarithromycin, with near resolution of the bloating and upper abdominal pain. Five months before the current admission, mesalamine was discontinued and adalimumab was initiated for the treatment of Crohn’s disease. Three months before the current admission, bloating and epigastric pain recurred; stools contained blood and mucus, and bowel movements increased in frequency to three or four times per day. Two months before the current admission, the patient was again evaluated in the gastroenterology clinic affiliated with this hospital. Physical examination was normal. The fecal calprotectin level was 2144 μg per gram. The adalimumab level and antiadalimumab antibody titer were interpreted as low. Additional imaging studies were obtained.
Dr. Aileen O’Shea: Magnetic resonance enterography of the abdomen and pelvis (Figure 2), performed after the administration of intravenous contrast material, revealed thickening and edema of the ascending colon, cecum, and terminal ileum, with surrounding changes consistent with inflammation and marked contrast enhancement. There was no evidence of bowel obstruction.
Dr. Parisi: The frequency of the adalimumab injections was increased, and prednisone therapy was initiated. However, 7 weeks before the current admission, nausea and vomiting developed, abdominal pain worsened, and the patient was admitted to this hospital. Physical examination was notable for pain on palpation of the epigastric area and tachycardia that resolved with the administration of intravenous fluids. The blood lipase level was normal, as were the results of liver-function tests. Other laboratory test results are shown in Table 1. The fecal calprotectin level was more than 3000 μg per gram. Results of esophagogastroduodenoscopy were normal, and biopsy specimens showed resolution of the previous gastritis. Colonoscopy revealed severe inflammation with deep ulcerations in the right side of the colon, extending from the area 1 cm proximal to the ileocecal valve to the hepatic flexure. Findings on examination of a biopsy specimen obtained from the right side of the colon were interpreted as consistent with severe active colitis; immunohistochemical staining for cytomegalovirus was negative. Treatment with intravenous glucocorticoids was initiated, with resolution of the bloody bowel movements. Adalimumab was discontinued, and infliximab was initiated. Epigastric pain decreased with the initiation of a bland diet, and on the seventh hospital day, the patient was discharged home with a plan to complete a 14-day course of empirical ciprofloxacin and metronidazole and a 6-week tapering course of oral prednisone.
During the last week of the prednisone course, fever and diaphoresis developed and abdominal pain worsened. The patient presented to the emergency department of this hospital for evaluation. She reported 6 days of increased bloating and 1 day of sharp abdominal pain on the right side; she rated the pain at 9 on a scale of 0 to 10, with 10 indicating the most severe pain. She reported that her stools did not contain blood or mucus and had not changed in consistency and her bowel movements had not changed in frequency. There was no joint pain, rash, or eye redness or pain. Other history included Clostridium difficile colitis (which had been treated with oral vancomycin 29 months before presentation), polycystic ovarian syndrome, dysmenorrhea, infertility (which had led to in vitro fertilization), and piriformis syndrome. Surgical history included appendectomy 20 years before presentation, right inguinal hernia repair 2 years before presentation, and right knee arthroscopic chondroplasty 3 months before presentation. There were no known drug allergies. Medications included infliximab, pantoprazole, prednisone, pregabalin, and sucralfate. Family history included coronary artery disease in her mother and diabetes and hypertension in her father. The patient did not smoke tobacco, drink alcohol, or use illicit drugs. She had moved to the United States from India 13 years before presentation, and she lived with her husband and son in New England.
On physical examination, the temperature was 38.5°C, the pulse 118 beats per minute, the blood pressure 125/78 mm Hg, the respiratory rate 16 breaths per minute, and the oxygen saturation 99% while the patient was breathing ambient air. The weight was 57.5 kg, the height 157 cm, and the body-mass index (the weight in kilograms divided by the square of the height in meters) 23.2. The abdomen was nondistended, with normal bowel sounds. There was tenderness on palpation of the right lower, right upper, and left upper quadrants, without rebound or guarding. The anus appeared normal; there were two nonbleeding external hemorrhoids. The patient had no joint swelling, rash, or oral lesions. The remainder of the physical examination was normal. The blood lactic acid level was normal, as were the results of liver-function tests and urinalysis. The fecal calprotectin level was 1372 μg per gram. Other laboratory test results are shown in Table 1. Blood cultures were obtained. Examination of a stool specimen for the C. difficile toxin was negative.
Dr. O’Shea: A chest radiograph showed no consolidation or pulmonary edema. CT of the abdomen and pelvis (Figure 2), performed after the administration of intravenous contrast material, revealed diffuse thickening and enhancement of the ascending colon, cecum, and terminal ileum, with surrounding changes consistent with inflammation, minimal free peritoneal fluid, and multiple enlarged mesenteric lymph nodes (measuring ≤9 mm) in the right lower quadrant.
Dr. Parisi: Intravenous fluids were administered, tachycardia resolved, and the patient was admitted to this hospital. Glucocorticoids and piperacillin in combination with tazobactam were administered intravenously, with a minimal decrease in the bloating and abdominal pain. On the fifth hospital day, right hemicolectomy was performed, and a diagnosis was made.
Question
What is the diagnosis? Cast your vote. What diagnostic test is most likely to be helpful? Submit a comment about this case and about what diagnostic test is indicated.
Poll
What is the most likely diagnosis in this case?
Amebic colitis.11%
Chronic campylobacter infection.8%
Crohn's disease.23%
Intestinal Mycobacterium tuberculosis infection.33%
Gastrointestinal Behcet's disease.11%
Gastrointestinal sarcoidosis.11%
A 38-Year-Old Woman with Abdominal Pain and Fever
Case
The case description for a Case Records of the Massachusetts General Hospital appears below. What is the diagnosis? What diagnostic test is most likely to be helpful? Cast your vote on the diagnosis and submit a comment about what diagnostic test is indicated. The correct diagnosis, along with the full description of the case and the procedures performed, will be published in the June 20, 2019, issue of the Journal.
A 38-year-old woman with a history of Crohn’s disease was admitted to the hospital because of abdominal pain and fever. Despite appropriate medical treatment, she had progressive worsening of clinical symptoms. What is the most likely diagnosis?
Presentation of Case
Dr. Erika J. Parisi (Medicine): A 38-year-old woman with a history of Crohn’s disease was admitted to this hospital because of abdominal pain and fever.
The patient had been in her usual state of health until 3 years before the current admission, when nausea, vomiting, diarrhea, and pain in the right upper quadrant developed. She was evaluated in the emergency department of another hospital. The right upper quadrant was tender on palpation, and the remainder of the physical examination was normal. Computed tomography (CT) of the abdomen and pelvis revealed mild dilatation and fecalization of the distal small intestine. A diagnosis of small-bowel obstruction was suspected, and the patient was admitted to the hospital for bowel rest and administration of intravenous fluids. On the second hospital day, all the symptoms had resolved, and the patient was discharged home with a presumed diagnosis of viral gastroenteritis.
One month later, the patient was evaluated in a gastroenterology clinic affiliated with a second hospital. She reported ongoing bloating, intermittent abdominal pain on the right side, and two or three bowel movements per day with loose stools without blood or mucus. She had no weight loss or fever. Physical examination was normal. CT with small-bowel enterography revealed mild circumferential wall thickening of the proximal ascending colon and distal ileum and prominent lymph nodes in the right lower quadrant. Colonoscopy was notable for erythematous, granular, hemorrhagic, ulcerated mucosa in the proximal ascending colon and cecum. Biopsy specimens of the ileum and ascending colon showed normal mucosa; a biopsy specimen of the cecum showed evidence of active chronic colitis that was consistent with Crohn’s disease. Mesalamine therapy was initiated, with a decrease in the bloating and abdominal pain but no change in the frequency of bowel movements.
Table 1.
Laboratory Data.
Figure 1. Colonoscopic Images.
Images obtained during colonoscopy performed 7 months before the current admission, before the initiation of anti–tumor necrosis factor therapy, show a normal terminal ileum (Panel A) and inflammation involving primarily the cecum, with a patulous ileocecal valve (Panel B).
Figure 2. Imaging Studies of the Abdomen.
Images obtained during magnetic resonance enterography performed 3 months before the current admission (Panels A and B) show thickening and hyperenhancement of the terminal ileum, cecum, and ascending colon. The coronal, T1-weighted, contrast-enhanced image (Panel A) shows prominent mesenteric lymph nodes (arrow), and the axial, T2-weighted, fat-suppressed image obtained at the level of the cecal pole (Panel B) shows marked pericolonic edema (arrow). In addition, CT of the abdomen and pelvis was performed at the time of the current presentation, after the administration of intravenous contrast material. A coronal, reformatted image (Panel C) shows persistent, contiguous thickening of the terminal ileum, cecum, and ascending colon (arrow), and an axial image obtained at the level of the cecum (Panel D) shows pericolonic stranding, edema, and a prominent mesenteric lymph node (arrow).
Seven months before the current admission, the patient was evaluated in a gastroenterology clinic affiliated with this hospital. She reported postprandial bloating and epigastric pain, as well as two or three bowel movements per day with hard stools and associated straining. Physical examination was normal. The fecal calprotectin level was 243 μg per gram (reference range, <50). An interferon-γ release assay for Mycobacterium tuberculosis was negative. The blood lipase level was normal, as were the results of liver-function tests. Other laboratory test results are shown in Table 1. Results of esophagogastroduodenoscopy were normal, but gastric biopsy specimens showed evidence of Helicobacter pylori gastritis. Colonoscopy (Figure 1) revealed shallow ulcerations, edema, and friability in the cecum and a segment of the ascending colon. Findings on examination of a biopsy specimen of the cecum were interpreted as consistent with severe active colitis, with marked ulceration but without dysplasia or granulomas; immunohistochemical staining for cytomegalovirus was negative. The H. pylori gastritis was treated with lansoprazole, amoxicillin, and clarithromycin, with near resolution of the bloating and upper abdominal pain. Five months before the current admission, mesalamine was discontinued and adalimumab was initiated for the treatment of Crohn’s disease. Three months before the current admission, bloating and epigastric pain recurred; stools contained blood and mucus, and bowel movements increased in frequency to three or four times per day. Two months before the current admission, the patient was again evaluated in the gastroenterology clinic affiliated with this hospital. Physical examination was normal. The fecal calprotectin level was 2144 μg per gram. The adalimumab level and antiadalimumab antibody titer were interpreted as low. Additional imaging studies were obtained.
Dr. Aileen O’Shea: Magnetic resonance enterography of the abdomen and pelvis (Figure 2), performed after the administration of intravenous contrast material, revealed thickening and edema of the ascending colon, cecum, and terminal ileum, with surrounding changes consistent with inflammation and marked contrast enhancement. There was no evidence of bowel obstruction.
Dr. Parisi: The frequency of the adalimumab injections was increased, and prednisone therapy was initiated. However, 7 weeks before the current admission, nausea and vomiting developed, abdominal pain worsened, and the patient was admitted to this hospital. Physical examination was notable for pain on palpation of the epigastric area and tachycardia that resolved with the administration of intravenous fluids. The blood lipase level was normal, as were the results of liver-function tests. Other laboratory test results are shown in Table 1. The fecal calprotectin level was more than 3000 μg per gram. Results of esophagogastroduodenoscopy were normal, and biopsy specimens showed resolution of the previous gastritis. Colonoscopy revealed severe inflammation with deep ulcerations in the right side of the colon, extending from the area 1 cm proximal to the ileocecal valve to the hepatic flexure. Findings on examination of a biopsy specimen obtained from the right side of the colon were interpreted as consistent with severe active colitis; immunohistochemical staining for cytomegalovirus was negative. Treatment with intravenous glucocorticoids was initiated, with resolution of the bloody bowel movements. Adalimumab was discontinued, and infliximab was initiated. Epigastric pain decreased with the initiation of a bland diet, and on the seventh hospital day, the patient was discharged home with a plan to complete a 14-day course of empirical ciprofloxacin and metronidazole and a 6-week tapering course of oral prednisone.
During the last week of the prednisone course, fever and diaphoresis developed and abdominal pain worsened. The patient presented to the emergency department of this hospital for evaluation. She reported 6 days of increased bloating and 1 day of sharp abdominal pain on the right side; she rated the pain at 9 on a scale of 0 to 10, with 10 indicating the most severe pain. She reported that her stools did not contain blood or mucus and had not changed in consistency and her bowel movements had not changed in frequency. There was no joint pain, rash, or eye redness or pain. Other history included Clostridium difficile colitis (which had been treated with oral vancomycin 29 months before presentation), polycystic ovarian syndrome, dysmenorrhea, infertility (which had led to in vitro fertilization), and piriformis syndrome. Surgical history included appendectomy 20 years before presentation, right inguinal hernia repair 2 years before presentation, and right knee arthroscopic chondroplasty 3 months before presentation. There were no known drug allergies. Medications included infliximab, pantoprazole, prednisone, pregabalin, and sucralfate. Family history included coronary artery disease in her mother and diabetes and hypertension in her father. The patient did not smoke tobacco, drink alcohol, or use illicit drugs. She had moved to the United States from India 13 years before presentation, and she lived with her husband and son in New England.
On physical examination, the temperature was 38.5°C, the pulse 118 beats per minute, the blood pressure 125/78 mm Hg, the respiratory rate 16 breaths per minute, and the oxygen saturation 99% while the patient was breathing ambient air. The weight was 57.5 kg, the height 157 cm, and the body-mass index (the weight in kilograms divided by the square of the height in meters) 23.2. The abdomen was nondistended, with normal bowel sounds. There was tenderness on palpation of the right lower, right upper, and left upper quadrants, without rebound or guarding. The anus appeared normal; there were two nonbleeding external hemorrhoids. The patient had no joint swelling, rash, or oral lesions. The remainder of the physical examination was normal. The blood lactic acid level was normal, as were the results of liver-function tests and urinalysis. The fecal calprotectin level was 1372 μg per gram. Other laboratory test results are shown in Table 1. Blood cultures were obtained. Examination of a stool specimen for the C. difficile toxin was negative.
Dr. O’Shea: A chest radiograph showed no consolidation or pulmonary edema. CT of the abdomen and pelvis (Figure 2), performed after the administration of intravenous contrast material, revealed diffuse thickening and enhancement of the ascending colon, cecum, and terminal ileum, with surrounding changes consistent with inflammation, minimal free peritoneal fluid, and multiple enlarged mesenteric lymph nodes (measuring ≤9 mm) in the right lower quadrant.
Dr. Parisi: Intravenous fluids were administered, tachycardia resolved, and the patient was admitted to this hospital. Glucocorticoids and piperacillin in combination with tazobactam were administered intravenously, with a minimal decrease in the bloating and abdominal pain. On the fifth hospital day, right hemicolectomy was performed, and a diagnosis was made.
Question
What is the diagnosis? Cast your vote. What diagnostic test is most likely to be helpful? Submit a comment about this case and about what diagnostic test is indicated.
Poll
What is the most likely diagnosis in this case?
Amebic colitis.11%
Chronic campylobacter infection.8%
Crohn's disease.23%
Intestinal Mycobacterium tuberculosis infection.33%
Gastrointestinal Behcet's disease.11%
Gastrointestinal sarcoidosis.11%
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