Mucosal Healing and Risk for Lymphoproliferative Malignancy in Celiac Disease: A Population-Based Cohort Study

• Published in: Annals of internal medicine Vol. 159; no. 3; pp. 169 - 175
• Main Authors: LEBWOHL, Benjamin, GRANATH, Fredrik, EKBOM, Anders, SMEDBY, Karin E, MURRAY, Joseph A, NEUGUT, Alfred I, GREEN, Peter H. R, LUDVIGSSON, Jonas F
• Format: Journal Article
• Language: English
• Published: Philadelphia, PA American College of Physicians 06-08-2013
• Subjects: Adolescent; Adult; Atrophy; Biological and medical sciences; Biopsy; Celiac disease; Celiac Disease - complications; Celiac Disease - pathology; Child; Child, Preschool; Female; General aspects; Hematologic and hematopoietic diseases; Humans; Infant; Internal medicine; Intestinal Mucosa - pathology; Intestine, Small - pathology; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphoma - complications; Lymphoma - epidemiology; Lymphoma, B-Cell - epidemiology; Lymphoma, T-Cell - epidemiology; Male; Medical sciences; Medicin och hälsovetenskap; Middle Aged; Multiple tumors. Solid tumors. Tumors in childhood (general aspects); Risk assessment; Risk Factors; Sweden - epidemiology; Tumors; Young Adult; Sweden; Immunopathology; Mucosa; Risk; Hemopathy; Malignancy; Malignant tumor; Coeliac disease; Epidemiology; Statistical study; Medicine; Treatment; Intestinal malabsorption; Healing agent; Cohort study; Lymphoproliferative syndrome; Risk factor; Digestive diseases; Intestinal disease; Population; Cicatrization; Public health; Cancer
• DOI: 10.7326/0003-4819-159-3-201308060-00006
• EISSN: 1539-3704
• ISSN: 0003-4819; 1539-3704

Celiac disease (CD) is associated with an increased risk for lymphoproliferative malignancy (LPM). Whether this risk is affected by the results of follow-up intestinal biopsy, performed to document mucosal healing, is unknown.
To examine the association between mucosal healing in CD and subsequent LPM.
Population-based cohort study.
28 pathology departments in Sweden.
7625 patients with CD who had follow-up biopsy after initial diagnosis.
The risk for LPM was compared with that of the general population by using expected rates. The rate of LPM in patients with persistent villous atrophy was compared with that of those with mucosal healing by using Cox regression.
Among 7625 patients with CD and follow-up biopsy, 3308 (43%) had persistent villous atrophy. The overall risk for LPM was higher than that in the general population (standardized incidence ratio [SIR], 2.81 [95% CI, 2.10 to 3.67]) and was greater among patients with persistent villous atrophy (SIR, 3.78 [CI, 2.71 to 5.12]) than among those with mucosal healing (SIR, 1.50 [CI, 0.77 to 2.62]). Persistent villous atrophy compared with mucosal healing was associated with an increased risk for LPM (hazard ratio [HR], 2.26 [CI, 1.18 to 4.34]). The risk for T-cell lymphoma was increased (HR, 3.51 [CI, 0.75 to 16.34]) but not for B-cell lymphoma (HR, 0.97 [CI, 0.21 to 4.49]).
No data on dietary adherence.
Increased risk for LPM in CD is associated with the follow-up biopsy results, with a higher risk among patients with persistent villous atrophy. Follow-up biopsy may effectively stratify patients with CD by risk for subsequent LPM.