Serum and histological IgG4-negative type 1 autoimmune pancreatitis

Takano, Yuichi; Kobayashi, Takahiro; Niiya, Fumitaka; Yamamura, Eiichi; Maruoka, Naotaka; Yokomizo, Kazuaki; Mizukami, Hajime; Tanaka, Junichi; Norose, Tomoko; Ohike, Nobuyuki; Nagahama, Masatsugu

doi:10.1007/s12328-018-0919-4

Cited by 4 publications

(2 citation statements)

References 16 publications

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“…There is evidence from previous studies that serum and histological IgG4 levels are not absolute diagnostic criteria for AIP‐1. Nakano and Takano reported two cases with negative serum and histological IgG4 levels, 6,7 and Hart et al also reported similar cases 8 . The pathogenic function of IgG4 in AIP is still unknown, and studies suggest that IgG4 antibodies have weak complement system activation and FC‐γ binding ability, and their elevated levels may be a result of chronic inflammation 9,10 .…”

Section: Discussionmentioning

confidence: 94%

Misdiagnosis and delayed diagnosis of atypical autoimmune pancreatitis: Experience from clinical cases

Guan

Liang

et al. 2023

Int J of Rheum Dis

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Autoimmune pancreatitis (AIP) is a fibro‐inflammatory disease characterized by inflammation and fibrosis of the pancreas. It is a systemic disease that can affect multiple organs, including the bile ducts, kidneys, lungs, and other organs. However, due to its complex presentation, AIP is often challenging to diagnose, and misdiagnosis with pancreatic tumors can occur. In our study, we reviewed three cases of atypical AIP where patients had normal serum IgG4 levels, leading to initial misdiagnosis with pancreatic tumors. Delayed diagnosis resulted in irreversible pathologies such as retroperitoneal fibrosis. All three patients had bile duct involvement, and imaging findings were similar to those of tumors, further complicating the diagnosis. The correct diagnosis was confirmed only after diagnostic therapy. Our study aims to raise awareness of atypical AIP and improve diagnostic efficiency by analyzing the clinical characteristics of these patients.

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Section: Discussionmentioning

confidence: 94%

Misdiagnosis and delayed diagnosis of atypical autoimmune pancreatitis: Experience from clinical cases

Guan

Liang

et al. 2023

Int J of Rheum Dis

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“…Recently, cases wherein the serum IgG4 level was normal and IgG4-positive cells were scarcely detected despite showing typical pathological findings of IgG4-RD (storiform fibrosis, obliterative phlebitis, and diffuse lymphoplasmacytic infiltration) have been reported [17–19]. Therefore, further research is warranted because IgG4 may not be a prerequisite for the pathology of the disease.…”

Section: Discussionmentioning

confidence: 99%

A Case of Ileocecal IgG4-Related Sclerosing Mesenteritis Diagnosed by Endoscopic Ultrasound-Guided Fine Needle Aspiration using Forward-Viewing Linear Echoendoscope

Takano

Niiya

Kobayashi

et al. 2019

Case Reports in Gastrointestinal Medicine

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A 25-year-old woman had undergone removal of a cryptogenic tumor in the left maxillary sinus 1 year prior to presentation. The patient experienced abdominal pain for 4 days with repeated vomiting episodes; therefore, she was transferred to our hospital by an ambulance. Contrast-enhanced computed tomography revealed a 3-cm tumor in the ileocecal region, which caused small bowel obstruction. Contrast imaging of the ileus tube showed extrinsic compression of the ileocecal region. Forward-viewing linear echoendoscope revealed an irregular hypoechoic tumor measuring 3 cm outside the gastrointestinal tract. Using a 25G needle, endoscopic ultrasound-guided fine needle aspiration (EUS–FNA) was performed. Pathological finding was an inflammatory fibrous tissue with diffuse lymphoplasmacytic infiltration, with more than 10 IgG4-positive cells detected in a high-power field. Re-examination of a pathology specimen of the maxillary sinus tumor provided by the previous attending physician revealed that the inflammatory tissue had diffuse lymphoplasmacytic infiltration, which were accompanied by storiform fibrosis and obliterative phlebitis. Immunostaining revealed more than 50 IgG4-positive cells in a high-power field, a finding suggestive of IgG4-related disease. The serum IgG4 level was 21 mg/dl, which was within the normal range. Treatment was initiated with prednisolone at a dose of 50 mg/day, and the dose was later tapered off. CT and MRI performed 2 months later showed complete disappearance of the ileocecal tumor. The final diagnosis was asynchronously occurring IgG4-related maxillary sinusitis and sclerosing mesenteritis.

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