Clinical characteristics of Crohn's disease in 72 families

Registre des Maladies Inflammatoires du Tube Digestif du Nord-Ouest de la France (No. 92/R12), Unit of Epidemiology, Centre Hospitalier Régional et Universitaire, Lille, France.
Gastroenterology (Impact Factor: 13.93). 09/1996; 111(3):604-7. DOI: 10.1053/gast.1996.v111.pm8780563
Source: PubMed

ABSTRACT Familial aggregation argues for genetic susceptibility to Crohn's disease. The aim of this study was to compare the age of onset and the clinical features of Crohn's disease between patients with familial disease and those with sporadic disease and investigate the concordance for disease location and type among relatives with Crohn's disease.
Seventy-two families with 2 (n = 55), 3 (n = 8), 4 (n = 6), and 5 or more (n = 3) affected first-degree relatives were selected for the study. A population of 1377 patients with sporadic nonfamilial Crohn's disease was used for comparison.
Clinical data were obtained from 176 patients with familial Crohn's disease (79 men and 97 women). Median age at onset was younger in familial Crohn's disease than in sporadic cases: 22 vs. 26.5 years (P < 0.01). In familial cases, fewer patients had exclusively colonic involvement and more patients had both small bowel and colonic involvement. Among relatives of families with 2 affected members, 56% were concordant for disease location and 49% for disease type. These percentages reached 83% and 76%, respectively, within families with more than 2 affected members.
Patients with familial Crohn's disease are characterized by an early age at onset with more extensive disease and may represent a homogeneous clinical subgroup with a particularly strong genetic influence.

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    • "Geographic variation of IBD provides clues for researchers to investigate possible environmental etiologic factors. Northern France is characterized by a high incidence of CD with a continuous increase over 20 years, especially in adolescents and young adults (Chouraki et al. 2011; Colombel et al. 1996). Using a Bayesian hierarchical model, we recently reported spatial heterogeneity in the incidence of CD with a predominance of the disease in agricultural areas (Declercq et al. 2010). "
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    • "Both CD and UC patients can be subdivided into a number of subgroups based on the course of the disease, clinical markers, and pathology. These differences are to a certain extent genetically determined (Bayless et al. 1996; Bouma et al. 1998, 1999; Colombel et al. 1996). "
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    • "The disease incidence peaks in early adulthood and is rising worldwide , with a current prevalence of ∼1/1,000 in Western countries (Probert et al. 1996; Shivananda et al. 1996). Although the cause of IBD is unknown, a strong genetic susceptibility for IBD is shown by both consistent familial clustering of disease (Mayberry 1989; Orholm et al. 1991; Colombel et al. 1996; Satsangi et al. 1996a) and increased concordance in monozygotic twins (Tysk et al. 1988; Thompson et al. 1996). Significant prevalence differences exist among different ethnic groups living in the same geographic region, displayed by the twoto eightfold higher prevalence in Ashkenazi Jews versus non-Jews (Roth et al. 1989). "
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