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Background and objective: Collagenous sprue is a rare and severe small intestinal enteropathy characterized by a markedly thickened subepithelial collagen band, villous atrophy, crypt hyperplasia, and intraepithelial lymphocytosis. Clinically, patients present with chronic diarrhoea, malabsorption, protein-losing enteropathy, micronutrient deficiencies, and significant weight loss, often leading to cachexia. Although frequently associated with celiac disease, collagenous sprue typically demonstrates poor or absent response to a gluten-free diet [1]. Given its frequently refractory clinical course, therapeutic management often necessitates prolonged immunosuppressive treatment.

Method: We present a case of severe collagenous sprue achieving sustained clinical and histological remission under anti–tumor necrosis factor alpha (TNF-α) therapy with infliximab.

Result: A 50-year-old woman diagnosed with refractory celiac disease was referred to our in-patient treatment due to intractable watery diarrhoea and life-threatening cachexia. Duodenal biopsies revealed complete villous atrophy, crypt hyperplasia, pronounced intraepithelial lymphocytosis, and a subepithelial collagen band consistent with collagenous sprue. T-cell clonality analyses of biopsies showed no signs of mono-/oligoclonality. After nutritional stabilization and substitution of human immunoglobulin because of severe hypogammaglobulinemia as a result of dramatic enteric protein loss, therapy with infliximab was initiated, leading to marked clinical and endoscopic improvement, with eventual histological remission. After 3 years therapy was discontinued at the patient’s request, resulting in rapid clinical relapse characterized by recurrent diarrhoea and need for inpatient parenteral nutritional therapy. Subsequent endoscopic and histopathological assessment revealed disease recurrence, with subepithelial collagen band thickening up to 30.2 µm and complete villous atrophy (Marsh IIIc). Re-initiation of infliximab achieved rapid and sustained clinical and histological remission.

Summary: Collagenous sprue should be considered in patients with refractory celiac disease unresponsive to a gluten-free diet. Anti–TNF-α immunomodulatory therapy with infliximab can induce rapid and sustained remission, though relapse may occur after treatment cessation, highlighting the importance of ongoing surveillance and individualized long-term immunomodulatory therapy.

Literatur

[1] Rejeski J, Conway J, Zhou Y. Collagenous Sprue. Am J Med Sci. 2020 May;359(5):310-11. DOI: 10.1016/j.amjms.2019.12.008