Collagenous sprue is not always associated with dismal outcomes: a clinicopathological study of 19 patients

Abstract

Collagenous sprue is associated with high morbidity; however, the etiology of this disorder is unclear. Data regarding the pathological and clinical manifestations of patients with collagenous sprue are also limited. We, thus, undertook this study to gain insight into the etiology, disease manifestations and outcomes of collagenous sprue. We searched our departmental database (1999)(2000)(2001)(2002)(2003)(2004)(2005)(2006)(2007)(2008) to identify cases of collagenous sprue and to obtain clinical and laboratory data. Small bowel histology, including thickness of subepithelial collagen, intra-epithelial lymphocyte phenotype and results of T-cell clonality assays were evaluated. Nineteen patients (15 women, 4 men, age 22-80 years, mean 57 years) were identified.

Collagenous sprue with associated features of refractory celiac disease

“…Indeed, there is recent evidence that these patients may be successfully treated (7), and the vast majority of patients with collagenous sprue (that sometimes display associated celiac disease) may have relatively good clinical outcomes (8).…”

A Rare Cause of Protein Losing Enteropathy: Collagenous Sprue

“…Histological alterations of intestinal villi may demonstrate subtotal or total atrophy. Patients die because of malnutrition, gastrointestinal system lymphomas, and infections [1,2,4]. Our patient died 5 months after being diagnosed because of an infection.…”

“…However, collagenous sprue, refractory celiac disease, refractory sprue, and unclassified sprue may demonstrate similar features. Collagenous sprue is also considered a severe complication of celiac disease in some literature [2].…”

“…Collagenous sprue is a clinicopathological entity with an unknown etiology. Its clinical features include progressive malabsorption, diarrhea, weight loss, unresponsiveness to treatment, and high mortality rates [2].…”

Refractory Celiac Disease: Epidemiology and Clinical Manifestations

“…Chronic inflammation could favor subepithelial accumulation of collagen and increase resistance to a GFD. In their series of 19 patients with collagenous sprue, Vakiani et al [18] observed that 17 had CD. Nine were primary or secondary resistant to a GFD at time of diagnosis of collagenous sprue, 8 with RCDI and 1 with RCDII.…”