High rates of complications and substantial mortality in both types of refractory sprue.
BACKGROUND
Refractory sprue (RS) is a rare malabsorption syndrome defined by persisting small bowel villous atrophy despite a strict gluten-free diet. The clinical picture and long-term outcome of RS is highly variable and is not well described.


OBJECTIVE
To define underlying and accompanying diseases and clinical outcome in consecutive patients with RS.


METHODS
Clinical and histological data from patients with RS at our department were analyzed retrospectively. RS was defined as villous atrophy and malabsorption despite a strict gluten-free diet persisting without improvement for more than 6 months or requiring earlier therapeutic intervention.


RESULTS
Thirty-two patients with RS were identified (23 RS type I, nine RS type II). Follow-up period was 55 (12-372) months. Two patients progressed from RS type I into type II. Thrombembolic events occurred in nine cases, and additional autoimmune diseases were found in 17 patients. Overt intestinal T-cell lymphoma developed in four patients with RS type II. Three patients with RS type II died during the observation period owing to intestinal T-cell lymphoma and four with RS type I owing to infectious complications. Five-year cumulative survival was 90% (95% confidence interval 76-100) in patients with RS type I and higher than in patients with RS type II (53%, 12-94%; P<0.05).


CONCLUSIONS
RS comprises a very heterogenous group of patients with long-term survival seen even in single patients with RS type II. Overall, survival is shorter in RS type II in comparison with RS type I. Patients with RS type I, however, show similar rates of disease-related complications as well as substantial mortality.
Daum
Severin
S
Department of Medicine I, Gastroenterology, Rheumatology and Infectious Diseases, Charité-University Medicine Berlin, Campus Benjamin Franklin, Berlin, Germany. severin.daum@charite.de
Ipczynski
Rainer
R
Schumann
Michael
M
Wahnschaffe
Ulrich
U
Zeitz
Martin
M
Ullrich
Reiner
R
D000293:Adolescent; D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases / Q000150:complications; D001851:Bone Diseases, Metabolic / Q000209:etiology; D002446:Celiac Disease / Q000150:complications* / Q000178:diet therapy / Q000401:mortality*; D002908:Chronic Disease; D055050:Diet, Gluten-Free; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007414:Intestinal Neoplasms / Q000209:etiology; D007669:Kidney Calculi / Q000209:etiology; D008228:Lymphoma, Non-Hodgkin / Q000209:etiology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013923:Thromboembolism / Q000209:etiology; D020246:Venous Thrombosis / Q000209:etiology; D055815:Young Adult