Original articles—liver, pancreas, and biliary tractPresentation and Management of Post-treatment Relapse in Autoimmune Pancreatitis/Immunoglobulin G4-Associated Cholangitis
Section snippets
Patients and Methods
This was a prospective case series and included all patients who were diagnosed with AIP in our center between February 2004 and December 2007 and who received a course of steroid therapy. The diagnosis was based on recognized international criteria, including those from the Japan Pancreas Society12 and, since 2006, the HISORt criteria from the Mayo Clinic.4 Features included a focal pancreatic mass or a diffusely enlarged pancreas (characteristically termed sausage pancreas); focal or diffuse
Patient Profile and Clinical Disease at Diagnosis
AIP was diagnosed in 28 patients (M:F, 24:4) with median age at diagnosis of 61 years (range, 27–81 years) (Table 1). The initial presentation included obstructive jaundice in 23 of 28 patients (82%), whereas 4 (14%) presented with abdominal/back pain alone. In 1 patient an incidental pancreatic mass was noted on follow-up imaging for an abdominal aortic aneurysm. Five patients (18%) had undergone surgery after their initial presentation (before the diagnosis of AIP) in view of the suspicion of
Discussion
It is now generally accepted that AIP represents one aspect of a multisystem disease characterized by the involvement of affected tissues by an IgG4-positive plasma cell infiltrate.13, 14, 15 The term IgG4-related sclerosing disease16 has also been advocated, and IAC has been used to describe the biliary disease with which AIP is frequently associated.10
Although spontaneous improvement of pancreatic masses and biliary strictures can occur,3, 17, 18 a rapid response to steroid therapy is often
References (38)
- et al.
Lymphoplasmacytic sclerosing pancreatitis with cholangitis: a variant of primary sclerosing cholangitis extensively involving pancreas
Hum Pathol
(1991) - et al.
Treating patients with autoimmune pancreatitis: results from a long-term follow-up study
Pancreatology
(2005) - et al.
Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience
Clin Gastroenterol Hepatol
(2006) - et al.
Involvement of the biliary system in autoimmune pancreatitis: a follow-up study
Clin Gastroenterol Hepatol
(2003) - et al.
Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy
Gastroenterology
(2008) - et al.
The use of immunoglobulin g4 immunostaining in diagnosing pancreatic and extrapancreatic involvement in autoimmune pancreatitis
Clin Gastroenterol Hepatol
(2007) - et al.
Clinical factors predictive of spontaneous remission or relapse in cases of autoimmune pancreatitis
Gastrointest Endosc
(2007) - et al.
Clinical analysis of autoimmune-related pancreatitis
Am J Gastroenterol
(2000) - et al.
Hypophysitis presented as inflammatory pseudotumor in immunoglobulin G4-related systemic disease
Hum Pathol
(2007) - et al.
IgG4-related sclerosing disease incorporating sclerosing pancreatitis, cholangitis, sialadenitis and retroperitoneal fibrosis with lymphadenopathy
Pancreatology
(2006)
Primary sclerosing cholangitis in childhood
Gastroenterology
Rituximab therapy for refractory biliary strictures in immunoglobulin G4-associated cholangitis
Clin Gastroenterol Hepatol
Immunoglobulin G4 associated cholangitis: description of an emerging clinical entity based on review of the literature
Hepatology
Autoimmune pancreatitis: clinical and radiological features and objective response to steroid therapy in a UK series
Am J Gastroenterol
Long-term outcome of autoimmune pancreatitis after oral prednisolone therapy
Intern Med
Long-term prognosis of autoimmune pancreatitis with and without corticosteroid treatment
Gut
Nephropathy in IgG4-related systemic disease
Am J Surg Pathol
High-rate pulmonary involvement in autoimmune pancreatitis
Intern Med J
Autoimmune related pancreatitis
Gut
Cited by (179)
Mimickers of immunoglobulin G4-related hepatobiliary disease on biopsy
2024, Seminars in Diagnostic PathologyAutoimmune pancreatitis - What we know and what do we have to know?
2021, Autoimmunity ReviewsCitation Excerpt :Clinical presentation of AIP may vary from completely asymptomatic to being part of other affected organs in IgG4 RD. Type 1 commonly presents in an elderly male with painless obstructive jaundice and nonspecific symptoms, such as nausea, vomiting or weight loss [44]. Given 80% of patients with AIP type 1 also have IgG4-associated cholangitis, involvement of other organs as part of IgG4-RD should be investigated [44,45]. Patients may have synchronous or metachronous involvement of other organ systems with diffusely enlarged or localized lesions with a histological picture consistent with IgG4-RD [6,36,37].
IgG4-related pancreatobiliary diseases could be associated with onset of pancreatobiliary cancer: A multicenter cohort study
2024, Journal of Hepato-Biliary-Pancreatic Sciences
Conflicts of interest The authors disclose no conflicts.