The Management of Complicated Celiac Disease

Refractory celiac disease (RCD) is being defined as persisting or recurring villous atrophy with crypt hyperplasia and increased intraepithelial lymphocytes (IELs) in spite of a strict gluten-free diet (GFD) for 1 12 months or when severe persisting symptoms necessitate intervention independent of the duration of the GFD. RCD may not respond primarily or secondarily to GFD. All other causes of malabsorption must be excluded and additional features supporting the diagnosis of CD must be looked for, including the presence of antibodies in the untreated state and the presence of celiac-related HLA-DQ markers. In contrast to patients with a high percentage of aberrant T-cells, patients with RCD I seem to profit from an immunosuppressive treatment. RCD II is usually resistant to medical therapies. Response to corticosteroid treatment does not exclude underlying enteropathy-associated T-cell lymphoma. Cladribine seems to have a role, although it is less than optimal in the treatment of these patients. It may be considered, however, as the only treatment thus far studied that showed significant reduction of aberrant T cells, seems to be well tolerated, and may have beneficial long-term effects in a subgroup of patients showing significant reduction of the aberrant T-cell population. AuProf. C.J.J. Mulder University Medical Center, Department Gastroenterology PO Box 7057, NL–1005 MB Amsterdam (The Netherlands) Tel. +31 20 444 0613, Fax +31 20 444 0554 E-Mail [email protected] © 2007 S. Karger AG, Basel 0257–2753/07/0253–0230$23.50/0 Accessible online at: www.karger.com/ddi D ow nl oa de d by : 54 .7 0. 40 .1 1 11 /1 3/ 20 17 4 :5 2: 42 P M