Simultaneous occurrence of pancreatic mixed acinar-ductal adenocarcinoma and primary follicular lymphoma of the duodenum, accompanied by increased number of IgG4 plasma cells in tumor-free parenchyma as concomitant IgG4-related disease or reaction to tumor? A case report

Ranković, Branislava; Limbaeck-Stokin, Clara; Đokić, Mihajlo; Stanisavljevič, Dragoje; Volavšek, Metka

doi:10.5114/pjp.2017.67622

Cited by 3 publications

(3 citation statements)

References 18 publications

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“…A case of a sausage-like pancreas with high-levels of serum IgG4 (344 mg/dL, normal: 5-105 mg/dL) and anti-DNA antibody (14 IU/mL, normal: <6.0 IU/mL) was identified as adenocarcinoma after evaluation of a forceps biopsy from the stenotic site of the main pancreatic duct [40]. We also need to bear in mind the possibility of a simultaneous co-existence of AIP (or IgG4-related pathology) and pancreatic malignancies; a small invasive cancer within the lesion of AIP [42], and a concomitant pancreatic mixed acinar-ductal adenocarcinoma; and follicular lymphoma accompanied with IgG4-related pathology [46]. In the international guideline, a persistent pancreatic mass is an indication of steroid therapy after the negative work up for pancreatic malignancies by EUS-FNA, even in an asymptomatic case [32].…”

Section: Mimickers Of Aipmentioning

confidence: 99%

Steroid Therapy and Steroid Response in Autoimmune Pancreatitis

Matsubayashi

Ishiwatari

Imai

et al. 2019

IJMS

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Autoimmune pancreatitis (AIP), a unique subtype of pancreatitis, is often accompanied by systemic inflammatory disorders. AIP is classified into two distinct subtypes on the basis of the histological subtype: immunoglobulin G4 (IgG4)-related lymphoplasmacytic sclerosing pancreatitis (type 1) and idiopathic duct-centric pancreatitis (type 2). Type 1 AIP is often accompanied by systemic lesions, biliary strictures, hepatic inflammatory pseudotumors, interstitial pneumonia and nephritis, dacryoadenitis, and sialadenitis. Type 2 AIP is associated with inflammatory bowel diseases in approximately 30% of cases. Standard therapy for AIP is oral corticosteroid administration. Steroid treatment is generally indicated for symptomatic cases and is exceptionally applied for cases with diagnostic difficulty (diagnostic steroid trial) after a negative workup for malignancy. More than 90% of patients respond to steroid treatment within 1 month, and most within 2 weeks. The steroid response can be confirmed on clinical images (computed tomography, ultrasonography, endoscopic ultrasonography, magnetic resonance imaging, and 18F-fluorodeoxyglucose-positron emission tomography). Hence, the steroid response is included as an optional diagnostic item of AIP. Steroid treatment results in normalization of serological markers, including IgG4. Short- and long-term corticosteroid treatment may induce adverse events, including chronic glycometabolism, obesity, an immunocompromised status against infection, cataracts, glaucoma, osteoporosis, and myopathy. AIP is common in old age and is often associated with diabetes mellitus (33–78%). Thus, there is an argument for corticosteroid therapy in diabetes patients with no symptoms. With low-dose steroid treatment or treatment withdrawal, there is a high incidence of AIP recurrence (24–52%). Therefore, there is a need for long-term steroid maintenance therapy and/or steroid-sparing agents (immunomodulators and rituximab). Corticosteroids play a critical role in the diagnosis and treatment of AIP.

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Section: Mimickers Of Aipmentioning

confidence: 99%

Steroid Therapy and Steroid Response in Autoimmune Pancreatitis

Matsubayashi

Ishiwatari

Imai

et al. 2019

IJMS

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“…Consequently, it is rarely necessary to differentiate diffuse-type AIP from PAC. However, the possibility of simultaneous co-existence of AIP (or IgG4-related pathology) and pancreatic malignancies, such as a small invasive cancer within the lesion of AIP [11], a concomitant pancreatic mixed acinar-ductal adenocarcinoma, and follicular lymphoma accompanied with IgG4-related pathology should be considered [12].…”

Section: Discussionmentioning

confidence: 99%

“…An average of 47.8% Korean patients experienced relapse during a median 60 months (range, 24~197 months) of follow-up [6]. Other organ involvement or increase of IgG level was associated with relapse of AIP [6][7][8][9][10][11][12][13][14].…”

Section: Discussionmentioning

confidence: 99%

A Case of Autoimmune Pancreatitis Presenting as a Deterioration in Glycemic Control in a Patient with Pre-Existing Type 2 Diabetes

Kim¹,

Baek²,

Song³

et al. 2021

J Korean Diabetes

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The present article demonstrates an unusual case of type 1 autoimmune pancreatitis (AIP), focusing on the cause of deterioration in glycemic control and weight loss in a patient with pre-existing type 2 diabetes mellitus (DM). A 67-year-old man was diagnosed with type 2 DM 23 years prior and presented with weight loss of approximately 6 kg over a period of 3 months and hemoglobin A1c (HbA1c) of 9.1%. His carbohydrate antigen 19-9 (CA 19-9) level was elevated to 158.54 U/mL (normal, 0~37 U/mL). Considering these findings, we needed to rule out hidden malignancy. Computed tomography of the abdomen showed diffuse swelling of the pancreas uncinated process. The serum immunoglobulin G (IgG) level was elevated to 2,418 mg/dL (normal, 700~1,600 mg/dL), and IgG4 level was elevated to 1,115.0 mg/dL (normal range, 3.9~86.4 mg/dL). This case highlights that AIP should be considered as a cause of significant weight loss and a deterioration in glycemic control in patients with DM. Furthermore, a pancreatic imaging study should be considered in clinical practice to differentiate pancreatic cancer and AIP.

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