Natural history of pancreatitis associated with cystic fibrosis gene mutations.

BACKGROUND: An increased incidence of CFTR mutations has recently been reported in chronic and idiopathic pancreatitis. AIM: The aim of the study was to verify these data and describe the clinical, morphological and histological findings in 99 patients (59 males, 40 females, mean age 40+/-16 years), 45 suffering from idiopathic chronic pancreatitis and 54 from acute recurrent pancreatitis. METHODS: Each subject was screened for the 18 CFTR mutations: DF508, DI507, R1162X, 2183AA>G, 21303K, 3849+10KbC>T, G542X, 1717-1G>A, R553X, Q552X, G85E, 711+5G>A, 3132delTG, 2789+5G>A, W1282X, R117H, R347P, R352Q), which cover 72% of cystic fibrosis chromosomes in the Italian population, plus the 5-thymidine allele in intron 8 of the CFTR gene (IVS85T). RESULTS: Among the 99 patients, we found 14 patients with CFTR mutation (14.1%). Three idiopathic chronic pancreatitis patients had cystic fibrosis (compound mutations in two and a single mutation with a pathological sweat test in one) and 11 (11.1%) presented a single mutation (carriers) (seven idiopathic chronic pancreatitis and four acute recurrent pancreatitis). The incidence of patients with cystic fibrosis was 167.5 times higher than that observed in the general population, whereas the carrier frequency was 4.43 times higher for chronic pancreatitis and 2.11 times for acute recurrent pancreatitis than that observed in 428 unrelated partners of cystic fibrosis patients. The prevalence of IVS8-5T was similar (7.1%) to that of the general population (10%). All idiopathic chronic pancreatitis patients with one or more CFTR gene mutations had a long history of recurrent attacks of pancreatitis. The length of recurrences of pancreatitis before diagnosis of chronic pancreatitis was shorter in chronic pancreatitis patients with one or more CFTR gene mutations than in the other idiopathic chronic pancreatitis patients (7.4+/-5.8 vs. 2.1+/-2 years). In idiopathic chronic pancreatitis patients with one or more CFTR gene mutations, exocrine and endocrine insufficiency (diabetes and steatorrhoea) were rare or delayed events. CONCLUSIONS: The natural history of pancreatitis associated with CFTR gene mutations seems to be characterised by recurrences of pancreatitis which develops into chronic pancreatitis.

High serum levels of secretory immunoglobullin A in chronic pancreatitis.

BACKGROUND AND AIM: Elevated levels of secretory immunoglobulin A have been reported in patients with cholestatic hepatitis. Secretory immunoglobulin A is present in the biliary and pancreatic tract. Chronic pancreatitis is a disease characterized by dilatation of Wirsung's duct. The aim of the study was to evaluate secretory immunoglobulin A levels in patients suffering from chronic pancreatitis. PATIENTS AND METHODS: The study population consisted of 66 consecutive chronic pancreatitis patients (55 male, 11 female; mean age 49.6+/-10 years), 26 patients suffering from acute recurrent pancreatitis (9 males, 17 females; mean age 39.6+/-10.6 years) and 90 healthy controls, pair-matched for sex and age with the chronic pancreatitis patients. Secretory immunoglobulin A was determined by enzyme-linked immunosorbent assay, as were serum alanine transaminase and GGT. RESULTS: Secretory immunoglobulin A levels were significantly higher in chronic pancreatitis patients (35+/-23.7 mg/l) than in those acute recurrent pancreatitis group (16.1+/- 7.9) and in healthy controls (11.8+/-4.9 mg/l) (p<0.0001). Secretory immunoglobulin A was significantly higher in chronic pancreatitis patients with steatorrhoea, diabetes and calcifications and in those undergoing pancreatic surgery. Of 61 chronic pancreatitis patients, 14 (23%) had pathological GGT. When only chronic pancreatitis patients with normal GGT levels were analysed, the differences in secretory immunoglobulin A levels between groups of patients and between chronic pancreatitis subgroups remained statistically significant. CONCLUSIONS: This study demonstrates that secretory immunoglobulin A is elevated in chronic pancreatitis. Its value in the staging of patients needs to be further evaluated.