Autoimmune Pancreatitis

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Takashi Kobayashi - One of the best experts on this subject based on the ideXlab platform.

  • IgG4-related hypophysitis in patients with Autoimmune Pancreatitis
    Pituitary, 2019
    Co-Authors: Keitaro Kanie, Hironori Bando, Genzo Iguchi, Hidenori Fukuoka, Hitoshi Nishizawa, Hideyuki Shiomi, Atsuhiro Masuda, Yasunori Fujita, Arata Sakai, Takashi Kobayashi
    Abstract:

    Purpose IgG4-related disease involves various organs including the pituitary and pancreas. The prevalence of IgG4-related hypophysitis is relatively rare compared with IgG4-related Pancreatitis (Autoimmune Pancreatitis). Although several cases demonstrating both Autoimmune Pancreatitis and hypophysitis have been reported, the prevalence of IgG4-related hypophysitis in patients with Autoimmune Pancreatitis remains unknown. This study aimed at screening for IgG4-related hypophysitis to accurately determine its prevalence in patients with Autoimmune Pancreatitis. Methods In this cohort study, we screened IgG4-related hypophysitis via pituitary magnetic resonance imaging (MRI) and endocrinological examination in 27 patients who were undergoing follow-up for Autoimmune Pancreatitis at Kobe University Hospital between 2014 and 2018. Results Among 27 patients with Autoimmune Pancreatitis, 5 patients exhibited morphological abnormalities in the pituitary (18.5%). Among them, one patient (3.7%) met the criteria for hypophysitis with an enlarged pituitary and stalk concomitant with hypopituitarism. After glucocorticoid treatment, the enlarged pituitary shrank and became empty sella during the clinical course. Four patients (14.8%) revealed empty sella without obvious pituitary dysfunction. Four of 5 patients with morphological pituitary abnormalities showed multiple organ involvement in addition to pancreatic and pituitary involvement. Accordingly, multiple organ involvement was more prevalent in patients with morphological pituitary abnormalities (80%) compared to those without (48%). Conclusions Although a large-scale study is necessary to validate these results, these data suggest that the prevalence of hypophysitis in patients with Autoimmune Pancreatitis may be underestimated. Based on our findings, we recommend screening for hypophysitis, especially in patients with multiple organ involvement.

  • IgG4-related hypophysitis in patients with Autoimmune Pancreatitis
    Pituitary, 2019
    Co-Authors: Keitaro Kanie, Hironori Bando, Genzo Iguchi, Hidenori Fukuoka, Hitoshi Nishizawa, Hideyuki Shiomi, Atsuhiro Masuda, Yasunori Fujita, Arata Sakai, Takashi Kobayashi
    Abstract:

    IgG4-related disease involves various organs including the pituitary and pancreas. The prevalence of IgG4-related hypophysitis is relatively rare compared with IgG4-related Pancreatitis (Autoimmune Pancreatitis). Although several cases demonstrating both Autoimmune Pancreatitis and hypophysitis have been reported, the prevalence of IgG4-related hypophysitis in patients with Autoimmune Pancreatitis remains unknown. This study aimed at screening for IgG4-related hypophysitis to accurately determine its prevalence in patients with Autoimmune Pancreatitis. In this cohort study, we screened IgG4-related hypophysitis via pituitary magnetic resonance imaging (MRI) and endocrinological examination in 27 patients who were undergoing follow-up for Autoimmune Pancreatitis at Kobe University Hospital between 2014 and 2018. Among 27 patients with Autoimmune Pancreatitis, 5 patients exhibited morphological abnormalities in the pituitary (18.5%). Among them, one patient (3.7%) met the criteria for hypophysitis with an enlarged pituitary and stalk concomitant with hypopituitarism. After glucocorticoid treatment, the enlarged pituitary shrank and became empty sella during the clinical course. Four patients (14.8%) revealed empty sella without obvious pituitary dysfunction. Four of 5 patients with morphological pituitary abnormalities showed multiple organ involvement in addition to pancreatic and pituitary involvement. Accordingly, multiple organ involvement was more prevalent in patients with morphological pituitary abnormalities (80%) compared to those without (48%). Although a large-scale study is necessary to validate these results, these data suggest that the prevalence of hypophysitis in patients with Autoimmune Pancreatitis may be underestimated. Based on our findings, we recommend screening for hypophysitis, especially in patients with multiple organ involvement.

Terumi Kamisawa - One of the best experts on this subject based on the ideXlab platform.

  • Management strategies for Autoimmune Pancreatitis.
    Expert Opinion on Pharmacotherapy, 2011
    Co-Authors: Terumi Kamisawa, Taku Tabata, Kensuke Takuma, Naoto Egawa, Yoshihiko Inaba, Seiichi Hara, Sawako Kuruma, Rajesh Gopalakrishna, Fumihide Itokawa, Takao Itoi
    Abstract:

    INTRODUCTION: Autoimmune Pancreatitis (AIP) is a newly developed concept for a peculiar type of Pancreatitis, and at present is recognized as a pancreatic lesion reflecting IgG4-related systemic disease. It is of utmost importance to differentiate AIP from pancreatic cancer to avoid unnecessary surgery. AREAS COVERED: The current management strategies for AIP, including its clinical features, diagnostic criteria, clinical subtypes, steroid therapy and prognosis are discussed, based on our 66 AIP cases and papers searched in PubMed from 1992 to March 2011, using the term 'Autoimmune Pancreatitis'. A new clinicopathological entity, an 'IgG4-related sclerosing disease' is also mentioned. EXPERT OPINION: AIP should be considered in the differential diagnosis in elderly male patients presented with obstructive jaundice and pancreatic mass. Steroids are a standard therapy for AIP, but their regimen including maintenance therapy should be evaluated in prospective trials.

  • Serum IgG4-negative Autoimmune Pancreatitis
    Journal of Gastroenterology, 2010
    Co-Authors: Terumi Kamisawa, Taku Tabata, Kensuke Takuma, Naoto Egawa, Koji Tsuruta, Tsuneo Sasaki, Yoshihiko Inaba, Tsunekazu Hishima, Takao Itoi
    Abstract:

    Background Autoimmune Pancreatitis (AIP) is considered to be a pancreatic lesion of IgG4-related systemic disease, but about 20% of AIP patients do not have elevated serum IgG4 levels. This study aimed to clarify the pathophysiology of AIP patients without elevated serum IgG4 levels.

  • Digestion and absorption of patients with Autoimmune Pancreatitis.
    Hepato-gastroenterology, 2006
    Co-Authors: Terumi Kamisawa, Hitoshi Nakajima, Naoto Egawa, Teruo Nakamura, Kouii Tsuruta, Atsutake Okamoto
    Abstract:

    Background/Aims: In patients with Autoimmune Pancreatitis, radiological, serological and pancreatic functional abnormalities usually improve with steroid therapy. However, no studies have specifically focused on digestion and absorption in such patients. This study aims to investigate digestion and absorption, in addition to pancreatic endocrine and exocrine function, and their responses to steroid therapy in patients with Autoimmune Pancreatitis. Methodology: Ideal body weight index, levels of serum albumin, total cholesterol and glucose, or glycosylated hemoglobin values were investigated in 22 patients with Autoimmune Pancreatitis. N-benzoyl-L-tyrosyl-paraminobenzoic acid excretion test was performed in 9 patients. In 10 patients treated with steroids, these factors were examined again about 2 months after medication. Results: Levels of serum albumin decreased, while ideal body weight index and levels of serum albumin and cholesterol significantly increased after steroid therapy. Eleven (50%) patients showed evidence of diabetes mellitus, but this improved in 6 of 8 patients treated with steroids. Pancreatic exocrine function was reduced in 88% of patients. Impaired pancreatic exocrine function improved after steroid therapy in 3 of 7 patients treated. Conclusions: Mild malnutrition as well as impaired pancreatic endocrine and exocrine function were frequently detected in patients with Autoimmune Pancreatitis. Steroid therapy was occasionally effective for these conditions.

  • Comparison of radiological and histological findings in Autoimmune Pancreatitis.
    Hepato-gastroenterology, 2006
    Co-Authors: Terumi Kamisawa, Hitoshi Nakajima, Atsutake Okamoto, Naoto Egawa, Kouji Tsuruta, Noriko Kamata, Nobuaki Funata
    Abstract:

    Background/aims Autoimmune Pancreatitis displays radiological findings that are sometimes difficult to differentiate from pancreatic carcinoma. To understand the essential radiological features of Autoimmune Pancreatitis (AIP), we compared imaging and histological findings in resected AIP specimens. Methodology Findings of ultrasonography, computed tomography, endoscopic retrograde cholangiopancreatography and angiography were examined retrospectively for 6 patients who underwent pancreatoduodenectomy on suspicion of pancreatic carcinoma, and compared with histological findings of the resected specimens. Results Ultrasonography showed an enlarged hypoechoic pancreas with sausage-like appearance and no lobulation in the contour of the pancreas. On computed tomography imaging, delayed enhancement of the swollen pancreatic parenchyma became evident. Dense lymphoplasmacytic infiltration with fibrosis involving peripancreatic tissue was observed throughout almost the entire pancreas. Periductal non-occlusive fibrosis with lymphoplasmacytic infiltration induced narrowing of the pancreatic duct. Stenosis of the common bile duct is frequently associated with Autoimmune Pancreatitis and is induced by diffuse thickening of the duct wall by the same inflammatory process as that of the pancreas. The fibroinflammatory process also involves blood vessels. Conclusions Characteristic radiological findings of Autoimmune Pancreatitis are induced with systemic histological changes of lymphoplasmacytic infiltration with fibrosis, and differ from schirrous invasion of pancreatic carcinoma.

  • Autoimmune Pancreatitis associated with retroperitoneal fibrosis.
    JOP : Journal of the pancreas, 2005
    Co-Authors: Terumi Kamisawa, Masakatsu Matsukawa, Masaomi Ohkawa
    Abstract:

    Context Autoimmune Pancreatitis is sometimes associated with other Autoimmune diseases. We have presented two cases of Autoimmune Pancreatitis with retroperitoneal fibrosis and compared our findings with those found in the literature. Case 1. A 71-year-old male developed anorexia and weight loss. Abdominal ultrasonography (US) and computed tomography (CT) showed diffuse swelling of the pancreas and the peritoneal soft tissue surrounding the aorta, associated with right hydronephrosis. Endoscopic retrograde pancreatography showed narrowing of the main pancreatic duct. He was diagnosed as having Autoimmune Pancreatitis associated with retroperitoneal fibrosis and underwent steroid therapy. After 3 weeks, a follow-up CT showed a marked reduction in the size of both the pancreas and retroperitoneal mass. Case 2. A 62-year-old male was admitted to another hospital complaining of obstructive jaundice. Abdominal CT and US showed swelling of the pancreas. Endoscopic retrograde cholangiopancreatography demonstrated stenosis of the lower bile duct and narrowing of the main pancreatic duct. With the diagnosis of pancreatic head carcinoma, a choledochojejunostomy and a gastrojejunostomy were performed. Histological examination of the biopsy of the pancreatic mass revealed marked fibrosis with lymphoplasmacytic infiltration. One year later, a retroperitoneal mass was detected on follow-up CT. He was treated with prednisolone for two years. Recurrence of retroperitoneal mass with left hydronephrosis occurred 18 months later. There was no sign of recurrence of the Autoimmune Pancreatitis. He was again treated with prednisolone, and the retroperitoneal mass was gradually reduced. Conclusions A total of 7 cases including the present cases have been reported. All were middle-aged males. Steroid therapy was effective for both the pancreatic and the retroperitoneal masses.

Keitaro Kanie - One of the best experts on this subject based on the ideXlab platform.

  • IgG4-related hypophysitis in patients with Autoimmune Pancreatitis
    Pituitary, 2019
    Co-Authors: Keitaro Kanie, Hironori Bando, Genzo Iguchi, Hidenori Fukuoka, Hitoshi Nishizawa, Hideyuki Shiomi, Atsuhiro Masuda, Yasunori Fujita, Arata Sakai, Takashi Kobayashi
    Abstract:

    Purpose IgG4-related disease involves various organs including the pituitary and pancreas. The prevalence of IgG4-related hypophysitis is relatively rare compared with IgG4-related Pancreatitis (Autoimmune Pancreatitis). Although several cases demonstrating both Autoimmune Pancreatitis and hypophysitis have been reported, the prevalence of IgG4-related hypophysitis in patients with Autoimmune Pancreatitis remains unknown. This study aimed at screening for IgG4-related hypophysitis to accurately determine its prevalence in patients with Autoimmune Pancreatitis. Methods In this cohort study, we screened IgG4-related hypophysitis via pituitary magnetic resonance imaging (MRI) and endocrinological examination in 27 patients who were undergoing follow-up for Autoimmune Pancreatitis at Kobe University Hospital between 2014 and 2018. Results Among 27 patients with Autoimmune Pancreatitis, 5 patients exhibited morphological abnormalities in the pituitary (18.5%). Among them, one patient (3.7%) met the criteria for hypophysitis with an enlarged pituitary and stalk concomitant with hypopituitarism. After glucocorticoid treatment, the enlarged pituitary shrank and became empty sella during the clinical course. Four patients (14.8%) revealed empty sella without obvious pituitary dysfunction. Four of 5 patients with morphological pituitary abnormalities showed multiple organ involvement in addition to pancreatic and pituitary involvement. Accordingly, multiple organ involvement was more prevalent in patients with morphological pituitary abnormalities (80%) compared to those without (48%). Conclusions Although a large-scale study is necessary to validate these results, these data suggest that the prevalence of hypophysitis in patients with Autoimmune Pancreatitis may be underestimated. Based on our findings, we recommend screening for hypophysitis, especially in patients with multiple organ involvement.

  • IgG4-related hypophysitis in patients with Autoimmune Pancreatitis
    Pituitary, 2019
    Co-Authors: Keitaro Kanie, Hironori Bando, Genzo Iguchi, Hidenori Fukuoka, Hitoshi Nishizawa, Hideyuki Shiomi, Atsuhiro Masuda, Yasunori Fujita, Arata Sakai, Takashi Kobayashi
    Abstract:

    IgG4-related disease involves various organs including the pituitary and pancreas. The prevalence of IgG4-related hypophysitis is relatively rare compared with IgG4-related Pancreatitis (Autoimmune Pancreatitis). Although several cases demonstrating both Autoimmune Pancreatitis and hypophysitis have been reported, the prevalence of IgG4-related hypophysitis in patients with Autoimmune Pancreatitis remains unknown. This study aimed at screening for IgG4-related hypophysitis to accurately determine its prevalence in patients with Autoimmune Pancreatitis. In this cohort study, we screened IgG4-related hypophysitis via pituitary magnetic resonance imaging (MRI) and endocrinological examination in 27 patients who were undergoing follow-up for Autoimmune Pancreatitis at Kobe University Hospital between 2014 and 2018. Among 27 patients with Autoimmune Pancreatitis, 5 patients exhibited morphological abnormalities in the pituitary (18.5%). Among them, one patient (3.7%) met the criteria for hypophysitis with an enlarged pituitary and stalk concomitant with hypopituitarism. After glucocorticoid treatment, the enlarged pituitary shrank and became empty sella during the clinical course. Four patients (14.8%) revealed empty sella without obvious pituitary dysfunction. Four of 5 patients with morphological pituitary abnormalities showed multiple organ involvement in addition to pancreatic and pituitary involvement. Accordingly, multiple organ involvement was more prevalent in patients with morphological pituitary abnormalities (80%) compared to those without (48%). Although a large-scale study is necessary to validate these results, these data suggest that the prevalence of hypophysitis in patients with Autoimmune Pancreatitis may be underestimated. Based on our findings, we recommend screening for hypophysitis, especially in patients with multiple organ involvement.

Raffaele Pezzilli - One of the best experts on this subject based on the ideXlab platform.

  • Immunosuppressive drugs for the treatment of Autoimmune Pancreatitis
    Immunotherapy, 2014
    Co-Authors: Raffaele Pezzilli
    Abstract:

    Autoimmune Pancreatitis is one of the few diseases of the pancreas characterized by the possibility of curing the illness using immunosuppressant drugs. In this paper, the therapeutic approach used to treat Autoimmune Pancreatitis patients and the clinical outcome related to each treatment modality were reviewed. Steroids are useful in alleviating the symptoms of the acute presentation of Autoimmune Pancreatitis, but some questions remain open, such as a shared definition of the disease’s remission as well as Autoimmune Pancreatitis relapse, the dosage of steroids in the symptomatic phase of the disease and the duration of steroid therapy. Finally, it should be determined if other immunosuppressive nonsteroidal drugs could become first-line therapy in patients with Autoimmune Pancreatitis without jaundice and without atrophic pancreas.

  • Therapeutic management and clinical outcome of Autoimmune Pancreatitis.
    Scandinavian journal of gastroenterology, 2011
    Co-Authors: Raffaele Pezzilli, Giulio Cariani, Donatella Santini, Lucia Calculli, Riccardo Casadei, Antonio Maria Morselli-labate, Roberto Corinaldesi
    Abstract:

    Abstract Background. Autoimmune Pancreatitis, in comparison to other benign chronic pancreatic diseases, is characterized by the possibility of curing the illness with immunosuppressant drugs. The open question is whether to differentiate Autoimmune Pancreatitis as a primary or secondary disease based on the presence or absence of other Autoimmune diseases or whether to consider Autoimmune Pancreatitis a clinical and pathological systemic entity, called IgG4-related sclerosing disease, since this aspect is also very important from a therapeutic point of view. Methods. In this paper, we reviewed the conventional therapeutic approach used to treat Autoimmune Pancreatitis patients and the clinical outcome related to each treatment modality. We also reviewed some aspects which are important for the correct management of Autoimmune Pancreatitis, such as the surgical approach, the outcome of surgically treated Autoimmune Pancreatitis patients, whether medical treatment is always necessary, and, finally, when me...

  • Autoimmune Pancreatitis, pancreatic cancer and immunoglobulin-G4.
    JOP : Journal of the pancreas, 2010
    Co-Authors: Raffaele Pezzilli, Roberto Corinaldesi
    Abstract:

    The occurrence of pancreatic cancer associated with Autoimmune Pancreatitis (AIP) has sometimes been reported [1, 2, 3, 4]. Of the four cases reported, pancreatic cancer was diagnosed simultaneously with AIP in two cases [1, 4]; one case of pancreatic cancer developed five years after a pancreaticoduodenectomy for AIP [2] and in the other case, the pancreatic cancer developed three years after steroid therapy was begun [3]. The possibility that pancreatic cancer may develop in AIP patients has recently been pointed out by Kamisawa et al. [5]. These authors assessed the possible relationship between AIP and pancreatic cancer by analyzing the K-ras mutation in the pancreatobiliary tissue of patients with AIP and they found a significant K-ras mutation in the pancreatobiliary region of patients with AIP suggesting that Autoimmune Pancreatitis may be a risk factor for pancreatobiliary cancer. On the basis of these observations, it is important to evaluate a biological marker of Autoimmune Pancreatitis, such as immunoglobulin G4 (IgG4), and to evaluate whether IgG4 is useful in the differential diagnosis between AIP and pancreatic cancer. We should remember that it has recently been shown that IgG4 serum levels do not have a good specificity for this purpose [6]. A new test has recently been developed [7] by an Italian group. To identify pathogenetically relevant autoantigen targets, the authors screened a random peptide sampling with pooled IgG obtained from 20 patients with Autoimmune Pancreatitis. Peptide-specific antibodies were detected in serum specimens obtained from the patients. Among the peptides detected, peptide AIP(1-7) was recognized by the serum specimens from 18 of 20 patients with Autoimmune Pancreatitis and by serum specimens from 4 of 40 patients with pancreatic cancer, but not by serum specimens from healthy controls. The peptide showed homology with an amino acid sequence of the plasminogen-binding protein (PBP) of Helicobacter pylori and with ubiquitin-protein ligase E3 component n-recognin 2 (UBR2), an enzyme highly expressed in acinar cells of the pancreas. Antibodies against the PBP peptide were detected in 19 of 20 patients with Autoimmune Pancreatitis (95.0%) and in 4 of 40 patients with pancreatic cancer (10.0%). Such reactivity was not detected in patients with alcoholinduced chronic Pancreatitis or intraductal papillary mucinous neoplasms. The results were also validated in another series of patients with Autoimmune Pancreatitis or pancreatic cancer; 14 of 15 patients with Autoimmune Pancreatitis (93.3%) and 1 of 70 patients with pancreatic cancer (1.4%) had a positive test for anti-PBP peptide antibodies. When the test and validation groups were combined, the test was positive in 33 of 35 patients with Autoimmune Pancreatitis (94.3%) and in 5 of 110 patients with pancreatic cancer (4.5%). In conclusion, this marker is also an imperfect test for distinguishing AIP from pancreatic cancer. Serum leptin has also been proposed as marker to differentiate Autoimmune Pancreatitis from pancreatic cancer [8]; however, in this study, the number of AIP patients was quite low and these promising results should be confirmed in a larger population. At present, a possible method for distinguishing AIP from pancreatic cancer come from the study of Song et al. [9]. These authors aimed at determining whether the combined measurement of total IgG and IgG4 could increase the diagnostic sensitivity for AIP while maintaining specificity as compared to IgG4 alone. They prospectively measured total serum IgG and IgG4 together in 82 consecutive patients with AIP, and

  • Steroid responsiveness and treatment regimen of steroids in acute Autoimmune Pancreatitis.
    Minerva gastroenterologica e dietologica, 2008
    Co-Authors: Raffaele Pezzilli, Antonio Maria Morselli-labate
    Abstract:

    Autoimmune Pancreatitis is the pancreatic manifestation of a systemic inflammatory disease in which affected organs are characterized by intense lymphoplasmacytic infiltration. The diagnosis of Autoimmune Pancreatitis sometimes is puzzling and its differentiation from pancreatic cancer is a clinical challenge. The features of Autoimmune Pancreatitis are its typical histological pattern, imaging, serology (increase of immunoglobulins and the presence of autoantibodies), associated non-pancreatic organ involvement and response to steroid therapy. Recent advances in identifying these features have resulted in enhanced recognition, diagnosis of this benign disease and the avoidance of unnecessary surgical procedures for suspected malignancy. In this article, the authors will review recent updates in the steroid treatment of Autoimmune Pancreatitis.

Arata Sakai - One of the best experts on this subject based on the ideXlab platform.

  • IgG4-related hypophysitis in patients with Autoimmune Pancreatitis
    Pituitary, 2019
    Co-Authors: Keitaro Kanie, Hironori Bando, Genzo Iguchi, Hidenori Fukuoka, Hitoshi Nishizawa, Hideyuki Shiomi, Atsuhiro Masuda, Yasunori Fujita, Arata Sakai, Takashi Kobayashi
    Abstract:

    Purpose IgG4-related disease involves various organs including the pituitary and pancreas. The prevalence of IgG4-related hypophysitis is relatively rare compared with IgG4-related Pancreatitis (Autoimmune Pancreatitis). Although several cases demonstrating both Autoimmune Pancreatitis and hypophysitis have been reported, the prevalence of IgG4-related hypophysitis in patients with Autoimmune Pancreatitis remains unknown. This study aimed at screening for IgG4-related hypophysitis to accurately determine its prevalence in patients with Autoimmune Pancreatitis. Methods In this cohort study, we screened IgG4-related hypophysitis via pituitary magnetic resonance imaging (MRI) and endocrinological examination in 27 patients who were undergoing follow-up for Autoimmune Pancreatitis at Kobe University Hospital between 2014 and 2018. Results Among 27 patients with Autoimmune Pancreatitis, 5 patients exhibited morphological abnormalities in the pituitary (18.5%). Among them, one patient (3.7%) met the criteria for hypophysitis with an enlarged pituitary and stalk concomitant with hypopituitarism. After glucocorticoid treatment, the enlarged pituitary shrank and became empty sella during the clinical course. Four patients (14.8%) revealed empty sella without obvious pituitary dysfunction. Four of 5 patients with morphological pituitary abnormalities showed multiple organ involvement in addition to pancreatic and pituitary involvement. Accordingly, multiple organ involvement was more prevalent in patients with morphological pituitary abnormalities (80%) compared to those without (48%). Conclusions Although a large-scale study is necessary to validate these results, these data suggest that the prevalence of hypophysitis in patients with Autoimmune Pancreatitis may be underestimated. Based on our findings, we recommend screening for hypophysitis, especially in patients with multiple organ involvement.

  • IgG4-related hypophysitis in patients with Autoimmune Pancreatitis
    Pituitary, 2019
    Co-Authors: Keitaro Kanie, Hironori Bando, Genzo Iguchi, Hidenori Fukuoka, Hitoshi Nishizawa, Hideyuki Shiomi, Atsuhiro Masuda, Yasunori Fujita, Arata Sakai, Takashi Kobayashi
    Abstract:

    IgG4-related disease involves various organs including the pituitary and pancreas. The prevalence of IgG4-related hypophysitis is relatively rare compared with IgG4-related Pancreatitis (Autoimmune Pancreatitis). Although several cases demonstrating both Autoimmune Pancreatitis and hypophysitis have been reported, the prevalence of IgG4-related hypophysitis in patients with Autoimmune Pancreatitis remains unknown. This study aimed at screening for IgG4-related hypophysitis to accurately determine its prevalence in patients with Autoimmune Pancreatitis. In this cohort study, we screened IgG4-related hypophysitis via pituitary magnetic resonance imaging (MRI) and endocrinological examination in 27 patients who were undergoing follow-up for Autoimmune Pancreatitis at Kobe University Hospital between 2014 and 2018. Among 27 patients with Autoimmune Pancreatitis, 5 patients exhibited morphological abnormalities in the pituitary (18.5%). Among them, one patient (3.7%) met the criteria for hypophysitis with an enlarged pituitary and stalk concomitant with hypopituitarism. After glucocorticoid treatment, the enlarged pituitary shrank and became empty sella during the clinical course. Four patients (14.8%) revealed empty sella without obvious pituitary dysfunction. Four of 5 patients with morphological pituitary abnormalities showed multiple organ involvement in addition to pancreatic and pituitary involvement. Accordingly, multiple organ involvement was more prevalent in patients with morphological pituitary abnormalities (80%) compared to those without (48%). Although a large-scale study is necessary to validate these results, these data suggest that the prevalence of hypophysitis in patients with Autoimmune Pancreatitis may be underestimated. Based on our findings, we recommend screening for hypophysitis, especially in patients with multiple organ involvement.