Update on the diagnosis and treatment of autoimmune pancreatitis.

Autoimmune pancreatitis (AIP) is the pancreatic manifestation of a systemic fibroinflammatory disease (IgG4-related systemic disease) in which affected organs demonstrate dense lymphoplasmacytic infiltration with abundant IgG4-positive cells. The diagnosis of AIP and its differentiation from pancreatic cancer, its main differential diagnosis, remains a clinical challenge. The five cardinal features of AIP are characteristic histology, imaging, and serology; other organ involvement; and response to steroid therapy. Recent advances in our understanding of these features have resulted in enhanced recognition and diagnosis of this benign disease. This in turn has resulted in the avoidance of unnecessary surgical procedures for suspected malignancy. This article reviews recent updates in the diagnosis and treatment of autoimmune pancreatitis.

Model for end-stage liver disease score versus Child score in predicting the outcome of surgical procedures in patients with cirrhosis.

AIM: To determine factors affecting the outcome of patients with cirrhosis undergoing surgery and to compare the capacities of the Child-Turcotte-Pugh (CTP) and model for end-stage liver disease (MELD) score to predict that outcome. METHODS: We reviewed the charts of 195 patients with cirrhosis who underwent surgery at two teaching hospitals over a five-year period. The combined endpoint of death or hepatic decompensation was considered to be the primary endpoint. RESULTS: Patients who reached the endpoint had a higher MELD score, a higher CTP score and were more likely to have undergone an urgent procedure. Among patients undergoing elective surgical procedures, no statistically significant difference was noted in the mean MELD (12.8 +/- 3.9 vs 12.6 +/- 4.7, P = 0.9) or in the mean CTP (7.6 +/- 1.2 vs 7.7 +/- 1.7, P = 0.8) between patients who reached the endpoint and those who did not. Both mean scores were higher in the patients reaching the endpoint in the case of urgent procedures (MELD: 22.4 +/- 8.7 vs 15.2 +/- 6.4, P = 0.0007; CTP: 9.9 +/- 1.8 vs 8.5 +/- 1.8, P = 0.008). The performances of the MELD and CTP scores in predicting the outcome of urgent surgery were only fair, without a significant difference between them (AUC = 0.755 +/- 0.066 for MELD vs AUC = 0.696 +/- 0.070 for CTP, P = 0.3). CONCLUSION: The CTP and MELD scores performed equally, but only fairly in predicting the outcome of urgent surgical procedures. Larger studies are needed to better define the factors capable of predicting the outcome of elective surgical procedures in patients with cirrhosis.

Possible association between IgG4-associated systemic disease with or without autoimmune pancreatitis and non-Hodgkin lymphoma.

OBJECTIVES: IgG4-associated systemic disease (ISD) is a multiorgan fibroinflammatory disorder whose pancreatic manifestation is called autoimmune pancreatitis (AIP). We describe 3 patients who developed non-Hodgkin lymphoma during the follow-up of ISD. METHODS: At our institution's pancreas clinic, we have prospectively and retrospectively examined patients with ISD with (n = 101) or without (n = 10) AIP (mean age, 59 years; 90 males and 21 females). We reviewed the medical records of all 111 patients to identify patients who developed non-Hodgkin lymphoma during the follow-up since their first presentation of ISD. Standardized incidence rate was calculated. RESULTS: The 111 patients with ISD with or without AIP had 331 patient-years of observation during which 3 patients had a diagnosis of non-Hodgkin lymphoma 3 to 5 years after the diagnosis of ISD. In these patients who later developed lymphoma, ISD involved the pancreas (AIP) in 2 and salivary gland in 1. Non-Hodgkin lymphoma had extranodal involvement in all patients (liver [n = 2], adrenal glands [n=1], kidney [n= 1], and lung [n = 1]). Standardized incidence rate was 16.0 (95% confidence interval, 3.3-45.5). CONCLUSIONS: We report 3 cases of non-Hodgkin lymphoma that developed during the follow-up of ISD suggesting that patients with ISD may be at an increased risk of developing non-Hodgkin lymphoma.