Collagenous sprue across five decades (1970-2025): a systematic review.

Area of Science:

• Gastroenterology
• Pathology
• Internal Medicine

Background:

• Collagenous sprue (CS) is a rare sprue-like enteropathy characterized by villous atrophy and a subepithelial collagen band.
• Existing evidence on CS is fragmented, necessitating a comprehensive review.

Purpose of the Study:

• To systematically review and analyze patient-level data on the presentation, evaluation, management, and outcomes of adult collagenous sprue.
• To consolidate fragmented evidence to provide a clearer understanding of this rare condition.

Main Methods:

• A systematic scoping review was conducted, searching multiple databases (PubMed/MEDLINE, Embase, Web of Science, CINAHL, Scopus) up to December 1, 2025.
• Included were adult cases with biopsy-confirmed CS, defined by small-bowel villous atrophy and a subepithelial collagen band.
• A pooled, descriptive patient-level analysis was performed on data from 99 unique patients across 50 publications.

Main Results:

• The study identified 99 adult patients with CS, with a median age of 65 years and a female predominance (62%).
• Patients presented with prolonged symptoms (median 36 weeks), severe diarrhea, malnutrition, edema, and significant malabsorption markers.
• Common treatments included nutritional rehabilitation, gluten-free diet, medication withdrawal (especially angiotensin receptor blockers), and corticosteroids, leading to rapid symptom improvement in many.
• Histologic improvement was observed in 74% of cases with follow-up biopsies, but relapse occurred in 31% and mortality was 23%.

Conclusions:

• Collagenous sprue is a serious condition with high morbidity.
• Early diagnosis, medication review, nutritional support, and steroid therapy are crucial for management.
• Despite treatment, substantial rates of relapse and mortality persist, highlighting the need for ongoing research and improved therapeutic strategies.