Mortality rate and risk factors in patients with hereditary pancreatitis: uni- and multidimensional analyses.
OBJECTIVE
Patients with hereditary pancreatitis (HP) bear a high risk of pancreatic adenocarcinoma, but their life expectancy remains unknown. The objective of the study was to assess whether the high risk of cancer decreases survival.


METHODS
Inclusion criteria were the presence of a PRSS1 mutation with pancreatic symptoms or chronic pancreatitis in at least two first-degree relatives or three second-degree relatives without another cause. Survival rates were assessed according to risk factors. Excess mortality compared with the general French population was calculated (statistical Esteve model) for two periods (20-50 and 50-70 years), according to several risk factors.


RESULTS
The cohort comprised 189 patients. PRSS1 mutations were found in 66%. A total of 19 patients died at the median age of 60. In all, 10 deaths were attributable to HP, including 8 to pancreatic adenocarcinoma. Median overall survival for the whole cohort was 74 years (95% confidence interval (CI): 71-79). The presence of R122H mutation, gender, tobacco consumption in patients older than 18 years, and diabetes mellitus were not associated with differences in survival. Only patients with pancreatic cancer had decreased survival (P=0.008). Excess mortality risk compared with the general population was 0.02% between 20 and 50 years, and 0.61% between 50 and 70 years (NS). Gender, R122H mutation, diabetes, and tobacco use were not associated with excess mortality in these two periods.


CONCLUSIONS
Despite their high risk of cancer, HP patients do not have excess mortality risk compared with the general population, irrespective of gender, tobacco use, or diabetes mellitus. These data should be brought to the patient's attention.
Rebours
Vinciane
V
Pôle des Maladies de l'Appareil Digestif, Service de Gastroentérologie--Pancréatologie, Hôpital Beaujon, AP-HP, Université Denis Diderot-Paris VII, 92118 Clichy, France. vinciane.rebours@bjn.aphp.fr
Boutron-Ruault
Marie-Christine
MC
Jooste
Valérie
V
Bouvier
Anne-Marie
AM
Hammel
Pascal
P
Ruszniewski
Philippe
P
Lévy
Philippe
P
D000230:Adenocarcinoma / Q000209:etiology / Q000401:mortality*; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D005260:Female; D005602:France / Q000453:epidemiology; D020022:Genetic Predisposition to Disease; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009154:Mutation; D010190:Pancreatic Neoplasms / Q000209:etiology / Q000401:mortality*; D010195:Pancreatitis / Q000235:genetics* / Q000401:mortality*; D012307:Risk Factors; D014357:Trypsin; D014362:Trypsinogen / Q000235:genetics*; D055815:Young Adult
D014362:Trypsinogen; C489303:PRSS1 protein, human; D014357:Trypsin