A 39-year-old African American man presented to the Emergency Department (ED) with a one-week history of epigastric pain. He had a seven-year history of chronic arthritis in his hands, feet, and back but was otherwise healthy. He reported experiencing epigastric pain after dining out for his birthday a week prior to presentation. The pain was gnawing, non-radiating, non-positional, and constant, but there were no fevers, chills, nausea, vomiting, or diarrhea. All other individuals dining with him were asymptomatic. He medicated himself with naproxen but the pain worsened, prompting his visit to the ED.
The patient had emigrated from Cape Verde to the United States 20 years previously. He was a bodybuilder, had noticed recent weight loss as well as a poor appetite and had lost 30lbs in the three months before presentation. He lived with his wife and worked for a printing company, did not smoke tobacco or use illicit drugs, rarely drank alcohol and had no allergies. There was no family history of note. His only outpatient medication was naproxen as needed for arthritis.
On examination he was in extreme pain with a blood pressure of 112/70, pulse 111, respiratory rate 18, and oxygen saturation was 100% breathing room air. Abdominal examination revealed a flat abdomen, normoactive bowel sounds, no bruits, a soft and tender epigastrium, and no rebound tenderness, rigidity, or guarding. Carnett's and Murphy's signs were negative. There were no palpable masses, no hepatosplenomegaly, and no hernias. Rectal examination was normal. There was no palpable lymphadenopathy. There was no active synovitis, palpable swelling, or joint line tenderness on peripheral joint examination. Neurological examination was non-focal. Laboratory results revealed microcytic anemia (Hb 11.9 g/dL; normal 13.5-18 g/dL) and mild leukocytosis (Table 1). Contrast-enhanced computed tomography (CT) of the abdomen and pelvis showed extensive mesenteric lymphadenopathy (Fig. 1). He was admitted to the general medical floor due to his intractable abdominal pain and for further workup.
At that point, the differential diagnosis for this relatively young man with chronic joint pain, chronic weight loss, recent onset epigastric pain, and microcytic anemia included primary GI lymphoma, gastroduodenal tuberculosis (TB), and peptic ulcer disease. He was treated with morphine, pantoprazole, intravenous fluids, and kept nothing by mouth for an esophagogastroduodenoscopy (EGD) the following day. The EGD performed on admission day two was macroscopically normal; gastric and duodenal biopsies were taken. He resumed eating and the abdominal pain was controlled with acetaminophen. On admission day three, he underwent ultrasound-guided mesenteric lymph node biopsy. The same day, histopathological examination of the duodenal biopsy revealed Periodic Acid-Schiff (PAS)-positive macrophages. Whipple's disease was diagnosed, and the mesenteric lymph node biopsy was subsequently found to be congruent with a diagnosis of Whipple's disease, revealing PAS-positive macrophages (Fig. 2).
The patient received ceftriaxone (2 g i.v.) for two weeks and was prescribed trimethoprim-sulfamethoxazole (160 mg trimethoprim/800 mg sulfamethoxazole) for one year. At six months follow-up, abdominal pain and arthralgia had resolved.