University of Kansas School of Medicine Wichita, KS, United States
Marisa-Nicole Zayat, BS1, Zeina-Christina Zayat, BS2, Estephan Zayat, MD2 1University of Kansas Medical Center, Kansas City, KS; 2University of Kansas School of Medicine, Wichita, KS
Introduction: Celiac disease is the most common autoimmune enteropathy affecting one in a hundred individuals worldwide. Diagnosis relies on serologic tests and small bowel biopsy. Treatment of celiac disease focuses on a lifelong gluten-free diet. Dreaded but rare complications include lymphoma and collagenous sprue.
Case Description/Methods: We present a 70-year-old female patient with a history of well-controlled celiac disease on a strict gluten-free diet for years who complained of new-onset fluctuating, crampy abdominal pain. The pain was associated with a change in bowel movements and relieved with defecation. She noted that the pain sometimes worsened after eating but denied any change in appetite or weight. She denied nausea, vomiting, watery diarrhea, hematochezia, and melena. Screening blood tests, including celiac serologies, were unremarkable. She described a sensation of incomplete evacuation, and a diagnosis of irritable bowel syndrome was suspected.
She did not improve with dicyclomine and underwent endoscopy, which revealed flattened atrophic small bowel mucosa. Biopsies demonstrated total villous blunting, increased intraepithelial lymphocytes, crypt hyperplasia, chronic inflammation, and patchy thickening of the subepithelial collagen band. A diagnosis of collagenous sprue was made. CT enterography was negative.
Soon thereafter, she developed progressive weight loss. She was given a short course of steroids and a low dose of azathioprine due to her TPMT showing intermediate activity with genotype 3A. Treatment was complicated by leukopenia, necessitating maintenance with very low-dose azathioprine. Her weight stabilized, and her abdominal symptoms improved.
Follow-up endoscopy revealed improvement in endoscopic and microscopic findings. She continued to be stable on azathioprine and a strict-gluten free diet.
Discussion: Our case highlights the need to re-evaluate the patient with repeat endoscopy when “the pieces don’t fit anymore.” Specifically, as in our patient, the need to thoroughly investigate new symptoms in patients with well-controlled celiac disease. The treatment of collagenous sprue is not well established. More studies are needed to evaluate azathioprine in this setting.
Figure: Biopsy findings of collagenous sprue before treatment at (A) low and (B) high magnification and after treatment at (C) low and (D) high magnification.
Disclosures: Marisa-Nicole Zayat indicated no relevant financial relationships. Zeina-Christina Zayat indicated no relevant financial relationships. Estephan Zayat indicated no relevant financial relationships.
Marisa-Nicole Zayat, BS1, Zeina-Christina Zayat, BS2, Estephan Zayat, MD2. P0960 - “The Pieces Don’t Fit Anymore”: An Unusual Presentation of Collagenous Sprue, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.
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