Autoimmune Pancreatitis and IgG4-related Cholangitis: A Single-center Experience
Kritsanat Lertussavavivat, MD1, Sasamon Chumjang, MD1, Supot Pongprasobchai, MD2
Affiliation : 1 Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand 2 Division of Gastroenterology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
Background : Autoimmune pancreatitis (AIP) and IgG4-related cholangitis (IRC) are newly-recognized diseases. Reports in Thailand
are scarce.
Objective : To evaluate clinical presentations, other organ involvement (OOI), serology, radiologic, endoscopic features and treatment
of AIP and IRC in one institute.
Materials and Methods : Patients with AIP and IRC, who were diagnosed and followed-up at Siriraj Hospital during 2005 to 2016
were retrospectively reviewed.
Results : There were 15 patients (75%) with AIP, 7 patients (37%) with IRC (4 isolated IRC and 3 IRC with AIP) and 1 (5%) with
IgG4-related disease without AIP or IRC (sialadenitis and retroperitoneal fibrosis). Male to female ratio was 1.9: 1. Median age of
onset was 64 years. Among the 15 AIP, all were type 1. Initial presentations were jaundice (60%), abdominal pain (40%), OOI (20%;
sialadenitis, orbital pseudotumor), and weight loss (7%). Clinical manifestations during the course were jaundice (80%), abdominal
pain (67%), weight loss (47%), OOI (47%; retroperitoneal fibrosis, IRC, sialadenitis, orbital pseudotumor, periurethral mass, lung
nodules) and steatorrhea (7%). Serum IgG4 was elevated in all patients. Radiography showed diffuse type in 8 cases (53%), focal
type in 5 (33%), and unknown in 2 (13%). More than half were suspicious of AIP before diagnosis. Corticosteroid was required in
10 patients (67%). Maintenance therapy was offered in 5 patients (33%) with steroid and azathioprine. Surgery was performed
before diagnosis in 7 patients (47%). Spontaneous remission occurred in 4 patients (27%). Among the 7 IRC, initial presentations
were jaundice (57%), weight loss (29%), abdominal pain (14%), prolonged fever (14%), and cholangitis (14%). Clinical
manifestations during the course were jaundice (57%), abdominal pain (57%), weight loss (29%), recurrent cholangitis (14%) and
retroperitoneal fibrosis (14%). Serum IgG4 was elevated in 5 patients (71%). Prednisolone was prescribed in 6 cases (86%). Five
patients (71%) required azathioprine. Surgery was performed before diagnosis in 4 patients (57%).
Conclusion : AIP and IRC were uncommon. The most common presentation was jaundice. All AIP were type 1 and more than half
were diffuse type. OOI were common. Half of IRC had AIP. Approximately half of AIP/IRC patients underwent surgery before
diagnosis. Corticosteroid was mainstay treatment.
DOI: 10.35755/jmedassocthai.2020.S08.12173
Keywords : Autoimmune pancreatitis, IgG4, Immunoglobulin G4, Sclerosing cholangitis, Thailand
