Clinical characteristics and long-term outcome of patients with refractory sprue diagnosed at a single institution
Acta gastroenterol. latinoam
; 36(1): 10-22, mar. 2006. tab, graf
Article
in English
| BINACIS
| ID: bin-123355
Responsible library:
BR1.1
ABSTRACT
BACKGROUND:
Refractory sprue (RS) is a rare and severe celiac-like enteropathy not responding to a strict gluten-free diet. Although prognosis is generally poor, little is known about the long-term outcome of patients.AIM:
to report baseline characteristics and long-term outcome of a series of patients diagnosed and treated in a single institution. MATERIALS We report a retrospective cohort of 25 consecutive patients (15 females; mean age 46 yr; range 28-71) diagnosed with RS based on the presence of a non-responsive celiac-like enteropathy. All patients were intensively treated with a gluten-free diet, steroids, nutritional support and immunosupression.RESULTS:
Clinical and biological characteristics of patients suggest that, at least, 24 patients had clear evidences of celiac disease. HLA DQ2/DQ8 genes were present in all the 24 patients typed and autoimmune enteropathy was excluded in all. According to the genotyping, 12 patients had a polyclonal lymphocyte population (RS type I) and 13 exhibited monoclonal TCR-gamma gene rearrangements (RS type II). Sixteen patients had evidence of ulcerative jejunitis (UJ) (7 in RS type I and 9 in type II). Overall median follow-up time after diagnosis of RS was 29 mo/patient (range 7 to 204) (45 mo for type I and 24 mo for type II). Overall mortality was 48% (12 patients), 6 in each type. Eight patients with UJ (50%), 3 with lymphoma (two T-cell and one B-cell type) and 4 (44%) without ulcers died during follow-up. The causes of death were sepsis in the context of a progressive deterioration but without overt malignancies (n=5), vascular causes (n=3) and severe malnutrition (n=1). Three- and 5-yr survival rate after diagnosis of RS for the overall population was 60% and 56%. There was no differences between type I (67%, 58%) and type II RS patients (54% for both periods). Patients with UJ had lower but non-significant 3- and 5-yr survival rates (56% and 50%, respectively) compared with patients without ulcers... (AU)
Full text:
Available
Collection:
National databases
/
Argentina
Database:
BINACIS
Main subject:
Celiac Disease
Type of study:
Diagnostic study
/
Prognostic study
Limits:
Adult
/
Female
/
Humans
/
Male
Country/Region as subject:
South America
/
Argentina
Language:
English
Journal:
Acta gastroenterol. latinoam
Year:
2006
Document type:
Article
Institution/Affiliation country:
Carlos Bonorino Udaondo Gastroenterology Hospital/s.p
/
Universidad de Buenos Aires/Argentina