BACKGROUND/AIMS: Type 2 autoimmune pancreatitis (AIP) has been considered extremely rare in East Asia. This study aimed to clarify the prevalence, clinical characteristics and radiological findings of type 2 AIP highlighting patients presenting as acute pancreatitis in a single center. METHODS: Type 2 AIP patients were classified according to International Consensus Diagnostic Criteria. Radiological findings were compared between type 2 AIP presenting as acute pancreatitis and gallstone pancreatitis. RESULTS: Among 244 patients with AIP, 27 (11.1%) had type 2 AIP (definite, 15 [55.5%] and probable 12 [44.5%]). The median age of patients with type 2 AIP was 29 years (interquartile range, 20 to 39 years). Acute pancreatitis was the most common initial presentation (n=17, 63%) while obstructive jaundice was present in only one patient. Ulcerative colitis (UC) was associated with type 2 AIP in 44.4% (12/27) of patients. Radiological pancreatic imaging such as delayed enhancement of diffusely enlarged pancreas, homogeneous enhancement of focal enlargement/mass, absent/minimal peripancreatic fat infiltration or fluid collection, and multifocal main pancreatic duct narrowings were helpful for differentiating type 2 AIP from gallstone pancreatitis. During follow-up (median, 32.3 months), two patients (2/25, 8%) experienced relapse. CONCLUSIONS: In South Korea, type 2 AIP is not as rare as previously thought. Overall, the clinical profile of type 2 AIP was similar to that of Western countries. Type 2 AIP should be considered in young UC patients with acute pancreatitis of uncertain etiology.
Colitis, Ulcerative ; Consensus ; Far East ; Follow-Up Studies ; Gallstones ; Humans ; Jaundice, Obstructive ; Korea ; Pancreas ; Pancreatic Ducts ; Pancreatitis ; Prevalence ; Recurrence

In the version of this article initially published, the fifth author's name was stated as “Joo Nam Lee.

BACKGROUND: Although pedobarographic measurement is increasingly used for clinical and research purposes, relatively few published studies have investigated normative data. This study examined pedobarographic findings in young healthy adults with regard to sex-related differences and correlations among measurement indices. METHODS: Twenty young healthy adults (mean age, 22.4 years; standard deviation, 1.2 years; and 10 males and 10 females) were included. Weight bearing anteroposterior (AP) and lateral foot radiographs were taken, and dynamic pedobarographic data during treadmill walking and maximum ankle dorsiflexion were obtained. AP talo-first metatarsal angle, naviculocuboid overlap, lateral talo-first metatarsal angle, and plantar soft tissue thickness were measured on foot radiographs. Pedobarographic data including peak pressure and pressure-time integral were measured on five plantar segments: medial forefoot (MFF), lateral forefoot (LFF), medial midfoot (MMF), lateral midfoot (LMF), and heel. RESULTS: Male and female subjects significantly differed in body mass index (BMI, p < 0.001), AP talo-first metatarsal angle (p = 0.018), soft tissue thickness under the metatarsal head (p = 0.040) and calcaneal tuberosity (p < 0.001), maximum dorsiflexion during stance phase (p = 0.041), peak pressure on the MFF (p = 0.005) and LFF (p = 0.004), and pressure-time integral on the MFF (p = 0.018) and heel (p = 0.001). BMI was significantly correlated with soft tissue thickness under the metatarsal head (r = 0.521, p = 0.018) and calcaneal tuberosity (r = 0.585, p = 0.007), peak pressure on the MFF (r = 0.601, p = 0.005) and LFF (r = 0.487, p = 0.029), pressure-time integral on the heel (r = 0.552, p = 0.012), and total pressure-time integral (r = 0.755, p < 0.001). Maximum dorsiflexion demonstrated significant negative correlations with pressure-time integral on the MFF (r = −0.595, p = 0.007) and total pressure-time integral (r = −0.492, p = 0.032). Pressure-time integral varus/valgus index was significantly correlated with pressuretime integral forefoot/heel index (r = 0.472, p = 0.036). CONCLUSIONS: Sex-related differences in pedobarographic examination were observed, which could provide useful information in setting appropriate treatment goals and obtaining appropriate control data. The effects of subtalar motion in distributing plantar pressure should be investigated in a future study.
Adult* ; Ankle ; Body Mass Index ; Female ; Foot ; Head ; Heel ; Humans ; Male ; Metatarsal Bones ; Sex Characteristics* ; Walking ; Weight-Bearing

BACKGROUND/AIMS: Differentially diagnosing focal-type autoimmune pancreatitis (f-AIP) and pancreatic cancer (PC) is challenging. Contrast-enhanced harmonic endoscopic ultrasound (CEH-EUS) may provide information for differentiating pancreatic masses. In this study, we evaluated the usefulness of CEH-EUS in differentiating f-AIP from PC. METHODS: Data were collected prospectively and analyzed on patients who underwent CEH-EUS between May 2014 and May 2015. Eighty consecutive patients were diagnosed with f-AIP or PC. PC and f-AIP were compared for enhancement intensity, contrast agent distribution, and internal vasculature. RESULTS: The study group comprised 53 PC patients and 27 f-AIP patients (17 with type-1 AIP [15 definite and two probable], two with probable type-2 AIP, and eight with AIP, not otherwise specified). Hyper- to iso-enhancement in the arterial phase (f-AIP, 89% vs PC, 13%; p < 0.05), homogeneous contrast agent distribution (f-AIP, 81% vs PC, 17%; p < 0.05), and absent irregular internal vessels (f-AIP, 85% vs PC, 30%; p < 0.05) were observed more frequently in the f-AIP group. The combination of CEH-EUS and enhancement intensity, absent irregular internal vessels improved the specificity (94%) in differentiating f-AIP from PC. CONCLUSIONS: CEH-EUS may be a useful noninvasive modality for differentially diagnosing f-AIP and PC. Combined CEH-EUS findings could improve the specificity of CEH-EUS in differentiating f-AIP from PC.
Biopsy, Fine-Needle ; Contrast Media ; Endosonography ; Humans ; Pancreatic Neoplasms* ; Pancreatitis* ; Prospective Studies ; Sensitivity and Specificity ; Ultrasonography*

A 40-year-old female previously diagnosed with primary biliary cirrhosis was referred to the hospital complaining of muscle weakness, arthralgia, Raynaud's phenomenon, and thick skin. After work-up, she was diagnosed with both mixed connective tissue disease (MCTD) and papillary thyroid cancer (PTC), based on the Alarcon-Segovia criteria and pathological examination, respectively. High-dose glucocorticoid and azathioprine were introduced to treat active myositis of MCTD, and total thyroidectomy was performed to treat PTC. This report highlights the possible association between MCTD and thyroid cancer, and suggests that MCTD is associated with PTC, similar to other autoimmune diseases including Sjögren's syndrome, systemic sclerosis, and systemic lupus erythematosus.
Adult ; Arthralgia ; Autoimmune Diseases ; Azathioprine ; Female ; Humans ; Liver Cirrhosis, Biliary* ; Lupus Erythematosus, Systemic ; Mixed Connective Tissue Disease* ; Muscle Weakness ; Myositis ; Scleroderma, Systemic ; Skin ; Thyroid Gland* ; Thyroid Neoplasms* ; Thyroidectomy

OBJECTIVES: The role of rheumatoid factor (RF) in vascular stiffness and cardiovascular risk in subjects without joint symptoms remains unclear. We investigated vascular stiffness in subjects without joint symptoms using pulse wave velocity (PWV), calculated Framingham risk scores (FRS), an estimator of cardiovascular risk, and analyzed whether vascular stiffness and FRS were affected by RF. METHODS: Two hundred forty-two subjects were included in this population-based study. RF was quantified with turbid immunometry using a cut-off of RF > 15 IU/ml to denote RF positivity. Information was then obtained on joint symptoms. Brachial-ankle PWV (baPWV) was measured using an automated device. RESULTS: Of the 242 subjects, 15 were RF-positive. RF-positive subjects without joint symptoms had a higher baPWV and FRS than RF-negative subjects without joint symptoms, but the difference did not reach statistical significance. However, when we stratified the subjects into two groups (group A – high RF: RF ≥ 40 IU/ml; group B – low RF: RF < 40 IU/ml), group A showed significantly higher baPWV (1640.7 ± 179.6 ㎝/s vs. 1405.7 ± 225.7 ㎝/s, P = 0.008) and FRS (25.7 ± 4.87 vs. 11.8 ± 9.6, P < 0.001). Multiple regression analysis was used to examine potential confounders, and RF exhibited significant but modest effects on baPWV (adjusted R-squared = 0.038, P = 0.030). CONCLUSIONS: In a sample of the general population without joint symptoms, higher levels of RF were associated with increased vascular stiffness, suggesting a pathophysiologic link between RF and endothelial dysfunction.
Joints* ; Pulse Wave Analysis ; Rheumatoid Factor* ; Vascular Stiffness*

PURPOSE: We propose a quantitative Tc-99m diethylenetriaminepentaacetic acid (DTPA) single-photon emission computed tomography/computed tomography (SPECT/CT) for glomerular filtration rate (GFR) measurement.METHODS: Quantitative SPECT/CT data obtained at 2–3 min post-Tc-99m DTPA injection (370 MBq) were used to determine % injected doses (%IDs) for individual kidneys. The reproducibility of %ID measurement was tested and compared with planar scintigraphy. Cr-51 ethylenediaminetetraacetic acid (EDTA) GFR was used as reference standard. Nine young volunteers, representing normal GFR, and ten older volunteers, reflecting impaired GFR, were enrolled. The established GFR equation derived from these volunteerswas applied to 19 renal tumor patients post-partial nephrectomy.RESULTS: At 2–3 min, %ID was most reproducible with the highest intraclass correlation (ICC) (0.9379) and lowest % coefficient of variation (CV) (6.5259%), which were more reliable than the ICC (0.9368) and %CV (6.7689%) of planar scintigraphy. Cr-51 EDTA GFR (93.16 ± 24.81 ml/min) correlated significantly with %ID (7.66 ± 2.15%, r = 0.7906, p = 0.0001), yielding an equation: Cr-51 EDTA GFR (ml/min) = (%ID × 9.1462) + 23.0653. This equation revealed significant decreases in total and nephrectomized kidney GFR (p = 0.0012 and p < 0.0001, respectively) from preoperative to 3-month postoperative measurements.CONCLUSIONS: Quantitative Tc-99m DTPA SPECT/CT produces reliable and clinically applicable %ID estimates that translate to the GFR of individual kidneys.
Edetic Acid ; Glomerular Filtration Rate ; Humans ; Kidney ; Nephrectomy ; Pentetic Acid ; Radionuclide Imaging ; Volunteers

BACKGROUND/AIMS: The aim of this study was to establish a pathogenetic mechanism of pancreatitis in celiac disease and IgG4-related disease using gluten-sensitive human leukocyte antigen (HLA)-DQ8 transgenic mice. METHODS: Transgenic mice expressing HLA-DQ8 genes were utilized. Control mice were not sensitized but were fed gliadin-free rice cereal. Experimental groups consisted of gliadin-sensitized and gliadin-challenged mice; nonsensitized mice with cerulein hyperstimulation; and gliadin-sensitized and gliadin-challenged mice with cerulein hyperstimulation. RESULTS: Gliadin-sensitized and gliadin-challenged mice with cerulein hyperstimulation showed significant inflammatory cell infiltrates, fibrosis and acinar atrophy compared with the control mice and the other experimental groups. The immunohistochemical analysis showed greater IgG1-positive plasma cells in the inflammatory infiltrates of gliadin-sensitized and gliadin-challenged mice with cerulein hyperstimulation compared with the control mice and the other experimental groups. Gliadin-sensitized and gliadin-challenged mice with cerulein hyperstimulation or gliadin-sensitized and gliadin-challenged mice showed IgG1-stained inflammatory cell infiltrates in the extrapancreatic organs, including the bile ducts, salivary glands, kidneys, and lungs. CONCLUSIONS: Gliadin-sensitization and cerulein hyperstimulation of gluten-sensitive HLA-DQ8 transgenic mice resulted in pancreatitis and extrapancreatic inflammation. This animal model suggests that chronic gliadin ingestion in a susceptible individual with the HLA-DQ8 molecule may be associated with pancreatitis and extrapancreatic inflammation.
Animals ; Animals* ; Atrophy ; Autoimmune Diseases ; Bile Ducts ; Celiac Disease ; Ceruletide ; Eating ; Edible Grain ; Fibrosis ; Gliadin* ; Humans ; Inflammation* ; Kidney ; Leukocytes ; Lung ; Mice* ; Mice, Transgenic ; Models, Animal* ; Pancreatitis* ; Plasma Cells ; Salivary Glands

In systemic sclerosis, digital ulcers and gangrene are somewhat common clinical characteristics of obliterative vasculopathy. These manifestations increase morbidities, such as pain, infections, and acroosteolysis. However, patient responses to the appropriate treatments are often inadequate. We treated a patient with systemic sclerosis who had a refractory digital ulcer and gangrene with bosentan, an endothelin receptor antagonist, and observed improvement. Here we systematically review this case.
Acro-Osteolysis ; Gangrene* ; Humans ; Receptors, Endothelin ; Scleroderma, Systemic* ; Ulcer*

Gallstones and alcohol consumption are well-known causes of acute pancreatitis, which usually follows a mild and self-limited course. Although extremely rare, hypercalcemia is a possible cause of acute pancreatitis. There are only few reported cases, all of which were mild and self-limited. Here we report a patient with iatrogenic hypercalcemia-induced necrotizing pancreatitis that progressed to serious adverse events such as biliary obstruction, peripancreatic fluid collection with walled-off necrosis, and acute cholecystitis. The patient was successfully treated with appropriate endoscopic and radiologic interventions, and recovered well.
Alcohol Drinking ; Calcium Compounds ; Cholecystitis, Acute ; Gallstones ; Humans ; Hypercalcemia* ; Necrosis ; Pancreatitis ; Pancreatitis, Acute Necrotizing*