scholarly journals Autoimmune pancreatitis type 1 and type 2: A report of two cases

2017 ◽  
Vol 74 (4) ◽  
pp. 361-366
Author(s):  
Irina Brcerevic ◽  
Radoje Doder ◽  
Nenad Perisic ◽  
Stanko Petrovic ◽  
Jasna Jovic ◽  
...  

Introduction. Autoimmune pancreatitis is a disease associated with autoimmune mechanisms, clinically manifested mostly as obstructive icterus with or with no entire or partial enlargement of the pancreas, histological lymphoplas-mocytic infiltration, fibrosis or granulocytic epithelial lesions with a favourable therapeutic response to the application of corticosteroids. Type 1 autoimmune pancreatitis is a systemic disease befalling the group of IgG4-related diseases in contrast to type 2 which is specific for pancreas disease. Case report. We presented two cases. The first one was a 64-year-old male patient with autoimmune pancreatitis complaining of abdominal pain, weight loss, weakness and exhaustion. Clinical examination showed a rare IgG4 autoimmune pancreatitis. The second one was a 37-year-old male patient complaining of abdominal pain with diarrhea. The diagnosis made revealed the presence of type 2 autoimmune pancreatitis. Following the diagnosis, immunosuppressive therapy was administered to both patients leading to the improvement of their general condition. Conclusion. Autoimmune pancreatitis is a rare disease, sometimes not easy to differ from pancreatic tumor or bile duct tumor with poor prognosis. Thus, early recognition of the disease is very important, since adequate treatment significantly increases the course and the outcomes of the disease.

2020 ◽  
Vol 53 (1) ◽  
pp. 81-87 ◽  
Author(s):  
Gustavo Mesquita De Oliveira ◽  
Lia de Freitas Araújo Alves ◽  
Paloma Lucena Cabral ◽  
Ana Luiza Viana Pequeno ◽  
Clóvis Rêgo Coelho ◽  
...  

Type 1 autoimmune pancreatitis is a cause of chronic pancreatitis related to the systemic disease known as IgG4-related Sclerosing Disease. Case report: We report the case of a 64-year-old male patient who presented recurrent epigastric pain radiating to the back, associated with jaundice, xerostomia, nausea, and vomiting, since 2014, diagnosed two years later with an unresectable pancreatic adenocarcinoma. The diagnosis was questioned after a few follow-up months without clinical deterioration when it was suggested the possibility of type 1 autoimmune pancreatitis in its pseudotumoral form. The patient was then treated with glucocorticoids, obtaining significant clinical improvement. After two years of follow-up, he returned asymptomatic with images suggestive of sclerosing cholangitis and a large liver abscess. Importance of the issue: The present case denotes the difficulty found in this diagnosis due to clinical and radiological resemblances with pancreatic adenocarcinoma. Besides that, it presents a seldom described disease complication, the liver abscess.


2020 ◽  
Vol 95 (2) ◽  
pp. 114-118
Author(s):  
So Hyun Kim ◽  
Seung-Ho Baek ◽  
Hye Yeong Kim ◽  
Su Jin Choi ◽  
Ji Hoon Kim ◽  
...  

There are two forms of autoimmune pancreatitis (AIP). Type 1 is associated with immunoglobulin G4 (IgG4)-related systemic fibro- inflammatory disease, whereas type 2 AIP is localized to the pancreas and not associated with IgG4. The number of children presenting with type 2 AIP has recently increased. Here, we report a case of type 2 AIP in a 16-year-old adolescent who presented with clinical acute pancreatitis and associated pancreatic masses. He was diagnosed with type 2 AIP based on pancreatic biopsy results showing granulocytic epithelial lesions and supportive radiological imaging and steroid responsiveness.


Gut ◽  
2013 ◽  
Vol 62 (9) ◽  
pp. 1373-1380 ◽  
Author(s):  
Terumi Kamisawa ◽  
Suresh T Chari ◽  
Markus M Lerch ◽  
Myung-Hwan Kim ◽  
Thomas M Gress ◽  
...  

2018 ◽  
Author(s):  
Allison L Yang ◽  
Julia McNabb-Baltar

Autoimmune pancreatitis (AIP) is a subcategory of chronic pancreatitis that is highly responsive to steroids. The term was first proposed in 1995 by Yoshida and colleagues, and since its discovery, the diagnosis of AIP has dramatically increased. AIP is a chronic fibroinflammatory disease characterized by lymphoplasmacytic infiltrates and fibrosis on histology. There are two distinct subtypes: type 1 AIP is the pancreatic manifestation of a systemic serum immunoglobulin G subtype 4–related disease (IgG4-RD) and type 2 AIP is described clinically as idiopathic duct-centric pancreatitis and has no association with IgG4. Clinically, AIP presents most commonly as obstructive jaundice in type 1 AIP and can present as acute pancreatitis in type 2 AIP. The diagnostic criteria include histology, imaging findings, and responsiveness to steroids as well as laboratory findings and other organ involvement. The mainstay of treatment is steroid therapy, with immunomodulators such as rituximab used for maintenance or relapsing disease. Long-term complications of AIP include pancreatic insufficiency and are often associated with relapsing disease. This review contains 45 references, 1 figure, and 2 tables. Key Words: autoimmune pancreatitis, chronic pancreatitis, EUS-guided biopsy, IgG4, immunomodulatory, obstructive jaundice, pancreas mass, steroid


2021 ◽  
Author(s):  
Sara Nikolic ◽  
Poya Ghorbani ◽  
Raffaella Pozzi Mucelli ◽  
Sam Ghazi ◽  
Francisco Baldaque- Silva ◽  
...  

Introduction: Autoimmune pancreatitis (AIP) is a disease that may mimic malignant pancreatic lesions both in terms of symptomatology and imaging appearance. The aim of the present study is to analyse experiences of surgery in patients with AIP in one of the largest European cohorts. Methods: We performed a single-centre retrospective study of patients diagnosed with AIP at the Department of Abdominal Diseases at Karolinska University Hospital in Stockholm, Sweden, between January 2001 and October 2020. Results: There were 159 patients diagnosed with AIP, and among them 35 (22.0%) patients had surgery: 20 (57.1%) males and 15 (42.9%) females; average age at surgery was 59 years (range 37-81). Follow-up period after surgery was 67 months (range 1-235). AIP type 1 was diagnosed in 28 (80%) patients and AIP type 2 in 7 (20%) patients. Malignant and premalignant lesions were diagnosed in 8 (22.9%) patients for whom AIP was not the primary differential diagnosis but, in all cases, it was described as a simultaneous finding and recorded in retrospective analysis in histological reports of surgical specimens. Conclusions: Diagnosis of AIP is not always straightforward, and, in some cases, it is not easy to differentiate it from the malignancy. Surgery is generally not indicated for AIP but might be considered in patients when suspicion of malignant/premalignant lesions cannot be excluded after complete diagnostic work-up.


2017 ◽  
Vol 41 (8) ◽  
pp. 1129-1138 ◽  
Author(s):  
Yuna Ku ◽  
Seung-Mo Hong ◽  
Kohei Fujikura ◽  
Sung Joo Kim ◽  
Masayuki Akita ◽  
...  

2014 ◽  
Vol 50 (7) ◽  
pp. 805-815 ◽  
Author(s):  
Shigeyuki Kawa ◽  
◽  
Kazuichi Okazaki ◽  
Kenji Notohara ◽  
Mamoru Watanabe ◽  
...  

Pancreatology ◽  
2017 ◽  
Vol 17 (4) ◽  
pp. S40
Author(s):  
Tsukasa Ikeura ◽  
Kota Kato ◽  
Kazushige Uchida ◽  
Makoto Takaoka ◽  
Akiyoshi Nishio ◽  
...  

2016 ◽  
Vol 11 (1) ◽  
pp. 27-33 ◽  
Author(s):  
Lars K. Lunden ◽  
Inge P. Kleggetveit ◽  
Ellen Jørum

Abstract Background and aims Complex regional pain syndrome (CRPS) is a serious and disabling chronic pain condition, usually occurring in a limb. There are two main types, CRPS 1 with no definite nerve lesion and CRPS 2 with an identified nerve lesion. CRPS 1 and 2 may occur following an injury (frequently following fractures), surgery or without known cause. An early diagnosis and start of adequate treatment is considered desirable for patients with CRPS. From the clinical experience of the principal investigator, it became apparent that CRPS often remained undiagnosed and that the clinical conditions of many patients seemed to be worsened following orthopedic surgery subsequent to the initial eliciting event. The aim of the present retrospective study of 55 patients, all diagnosed with either CRPS 1 or 2, was to evaluate the time from injury until diagnosis of CRPS and the effect on pain of orthopedic surgical intervention subsequent to the original injury/surgery. Methods Clinical symptoms with an emphasis on pain were assessed by going through the patients’ records and by information given during the investigation at Oslo University Hospital, where the patients also were examined clinically and with EMG/neurography. Alteration in pain was evaluated in 27 patients who underwent orthopedic surgery subsequent to the eliciting injury. Results Of a total of 55 patients, 28 women and 27 men (mean age 38.7 (SD 12.3), 38 patients were diagnosed with CRPS type 1, and 17 with CRPS type 2. Mean time before diagnosis was confirmed was 3.9 years (SD1.42, range 6 months-10 years). The eliciting injuries for both CRPS type 1 and type 2 were fractures, squeeze injuries, blunt injuries, stretch accidents and surgery. A total of 27 patients (14 men and 13 women) were operated from one to 12 times at a later stage (from 6 months to several years) following the initial injury or any primary operation because of fracture. A total of 22 patients reported a worsening of pain following secondary surgical events, while four patients found no alteration and one patient experienced an improvement of pain. None of the 22 patients reporting worsening, were diagnosed with CRPS prior to surgery, while retrospectively, a certain or probable diagnosis of CRPS had been present in 17/22 (77%) patients before their first post-injury surgical event. Conclusions and implications A mean time delay of 3.9 years before diagnosis of CRPS is unacceptable. A lack of attention to more subtle signs of autonomic dysfunction may be an important contributing factor for the missing CRPS diagnosis, in particular serious in patients reporting worsening of pain following subsequent orthopedic surgery. It is strongly recommended to consider the diagnosis of CRPS in all patients with a long-lasting pain condition. We emphasize that the present report is not meant as criticism to orthopedic surgical practice, but as a discussion for a hopefully increased awareness and understanding of this disabling pain condition.


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