AIM:To investigate differences in clinical features between diffuse-and focal-type autoimmune pancreatitis(AIP).METHODS:Based on radiological findings by computed tomography and/or magnetic resonance imaging,we divide...AIM:To investigate differences in clinical features between diffuse-and focal-type autoimmune pancreatitis(AIP).METHODS:Based on radiological findings by computed tomography and/or magnetic resonance imaging,we divided 67 AIP patients into diffuse type(D type) and focal type(F type).We further divided F type into head type(H type) and body and/or tail type(B/T type) according to the location of enlargement.Finally,we classified the 67 AIP patients into three groups:D type,H type and B/T type.We compared the three types of AIP in terms of clinical,laboratory,radiological,functional and histological findings and clinical course.RESULTS:There were 34 patients with D-type,19 with H-type and 14 with B/T-type AIP.Although obstructive jaundice was frequently detected in D-typepatients(88%) and H-type patients(68%),no B/T-type patients showed jaundice as an initial symptom(P < 0.001).There were no differences in frequency of abdominal pain,but acute pancreatitis was associated more frequently in B/T-type patients(36%) than in D-type patients(3%)(P = 0.017).Serum immunoglobulin G(IgG)4 levels were significantly higher in D-type patients(median 309 mg/dL) than in B/T-type patients(133.5 mg/dL)(P = 0.042).Serum amylase levels in B/T-type patients(median:114 IU/L) were significantly greater than in H-type patients(72 IU/L)(P = 0.049).Lymphoplasmacytic sclerosing pancreatitis(LPSP) was histologically confirmed in 6 D-type,7 H-type and 4 B/T-type patients;idiopathic duct-centric pancreatitis was observed in no patients.Marked fibrosis and abundant infiltration of CD20-positive B lymphocytes with few IgG4-positive plasma cells were detected in 2 B/T-type patients.Steroid therapy was effective in all 50 patients(31 D type,13 H type and 6 B/T type).Although AIP relapsed during tapering or after stopping steroids in 3 D-type and 3 H-type patients,no patients relapsed in B/T type.During follow-up,radiological features of 6 B/T-type patients were not changed and 1 B/T-type patient improved naturally.CONCLUSION:Clinical features of H-type AIP were similar to those of D-type,but B/T-type differed from D and H types.B/T-type may involve diseases other than LPSP.展开更多
基金Supported by The Research Committee of Intractable Pancreatic Diseases (Principal investigatorTooru Shimosegawa) provided by the Ministry of Health, Labour and Welfare of Japan
文摘AIM:To investigate differences in clinical features between diffuse-and focal-type autoimmune pancreatitis(AIP).METHODS:Based on radiological findings by computed tomography and/or magnetic resonance imaging,we divided 67 AIP patients into diffuse type(D type) and focal type(F type).We further divided F type into head type(H type) and body and/or tail type(B/T type) according to the location of enlargement.Finally,we classified the 67 AIP patients into three groups:D type,H type and B/T type.We compared the three types of AIP in terms of clinical,laboratory,radiological,functional and histological findings and clinical course.RESULTS:There were 34 patients with D-type,19 with H-type and 14 with B/T-type AIP.Although obstructive jaundice was frequently detected in D-typepatients(88%) and H-type patients(68%),no B/T-type patients showed jaundice as an initial symptom(P < 0.001).There were no differences in frequency of abdominal pain,but acute pancreatitis was associated more frequently in B/T-type patients(36%) than in D-type patients(3%)(P = 0.017).Serum immunoglobulin G(IgG)4 levels were significantly higher in D-type patients(median 309 mg/dL) than in B/T-type patients(133.5 mg/dL)(P = 0.042).Serum amylase levels in B/T-type patients(median:114 IU/L) were significantly greater than in H-type patients(72 IU/L)(P = 0.049).Lymphoplasmacytic sclerosing pancreatitis(LPSP) was histologically confirmed in 6 D-type,7 H-type and 4 B/T-type patients;idiopathic duct-centric pancreatitis was observed in no patients.Marked fibrosis and abundant infiltration of CD20-positive B lymphocytes with few IgG4-positive plasma cells were detected in 2 B/T-type patients.Steroid therapy was effective in all 50 patients(31 D type,13 H type and 6 B/T type).Although AIP relapsed during tapering or after stopping steroids in 3 D-type and 3 H-type patients,no patients relapsed in B/T type.During follow-up,radiological features of 6 B/T-type patients were not changed and 1 B/T-type patient improved naturally.CONCLUSION:Clinical features of H-type AIP were similar to those of D-type,but B/T-type differed from D and H types.B/T-type may involve diseases other than LPSP.
