Original article—alimentary tract
Gluten-Free Diet and Steroid Treatment Are Effective Therapy for Most Patients With Collagenous Sprue

https://doi.org/10.1016/j.cgh.2009.12.023Get rights and content

Background & Aims

Collagenous sprue (CS) is characterized by the presence of a distinctive band of subepithelial collagen deposition in the small bowel. We evaluated the clinical characteristics, treatments, and outcomes of patients with CS.

Methods

Thirty patients with CS were identified at the 3 Mayo Clinic sites between 1993 and 2009. Clinical data from medical records were reviewed.

Results

The study cohort was 70% female (age range, 53–91 years). Most patients had severe diarrhea and weight loss. Hospitalization to treat dehydration was necessary in 16 (53%) patients. Associated immune-mediated diseases were noted in 70% of the patients; celiac disease was the most frequent. Other associated diseases were microscopic colitis, hypothyroidism, and autoimmune enteropathy. The median thickness of the layer of subepithelial collagen deposition in the small bowel was 29 μm (20–56.5 μm). Subepithelial collagen deposition in the colon or stomach was noted in 8 patients. A clinical response was observed in 24 (80%) patients after treatment with a combination of a gluten-free diet and immunosuppressive drugs. Histologic improvement was confirmed in 9 patients, with complete remission in 5. Two patients died (1 of complications of CS and 1 of another illness).

Conclusions

Most patients with CS are treated effectively with a combination of gluten-free diet and steroids. CS is often associated with collagen deposition or chronic inflammation in other segments of the gastrointestinal tract as well as other immune-mediated disorders.

Section snippets

Patients

All patients seen at Mayo Clinic sites in Rochester (n = 22), Jacksonville (n = 7), and Scottsdale (n = 5) from 1993–2009 with CS were identified. Medical records were reviewed, and only patients who met histologic and clinical criteria for CS were included.

Pathology Material Evaluation

Original pathology material was re-reviewed by a single pathologist (T.T.W.). Subepithelial collagen thickness was measured by using a micrometer-equipped Olympus DP71 digital camera (Olympus, Center Valley, PA). Two well-oriented fields

Patients

We included 30 patients (21 [70%] female) with a median age (range) at diagnosis of 72.5 years (53–91 years). Twenty-nine patients were white, and 1 was black. Symptoms had been present for a median (range) of 9 months (1–120 months) before the CS diagnosis. At presentation, all patients had diarrhea, and 97% experienced marked loss of weight (median loss, 27 pounds). Abrupt onset of diarrhea was noted in 8 patients. Transient acute renal failure caused by diarrhea occurred in 6 patients; none

Discussion

Collagenous sprue was first described in 1947 by Schein. 4 Since then, several more patients and small case series have been reported. 1, 5, 6, 8, 10, 27, 28, 29, 30, 31 Here we present the clinical characteristics, histologic findings, treatment, and prognosis of 30 patients with CS. This study represents the largest series of patients reported to date with the diagnosis of CS. Of clinical relevance, up to 80% of our patients had clinical response after treatment with a combination of

Acknowledgments

The authors thank Estela G. Staggs and Deanna Brogan for their assistance in collection of data and pathology material.

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    Conflicts of interest The authors declare no conflicts.

    Funding This article was supported by the National Institutes of Health (NIH), Ruth L. Kirschstein National Research Service Award/Training Grant in Gastrointestinal Allergy and Immunology (T32 AI-07047) (to A.R.T.), and NIH grant DK-57892 (to J.A.M.).

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