A case of IgG4-related disease presented with a duodenal ulcer to improve the understan-ding of IgG4-related diseases was reported. A 70-year-old male presented with cutaneous pruritus and abdominal pain for four years and blackened stools for two months. Four years ago, the patient went to hospital for cutaneous pruritus and abdominal pain. Serum IgG4 was 3.09 g/L (reference value 0-1.35 g/L), alanine aminotransferase 554 U/L (reference value 9-40 U/L), aspartate aminotransferase 288 U/L (reference value 5-40 U/L), total bilirubin 54.16 μmol/L (reference value 2-21 μmol/L), and direct bilirubin 29.64 μmol/L (reference value 1.7-8.1 μmol/L) were all elevated. The abdominal CT scan and magnetic resonance cholangiopancreatography indicated pancreatic swelling, common bile duct stenosis, and secondary obstructive dilation of the biliary system. The patient was diagnosed with IgG4-related disease and treated with prednisone at 40 mg daily. As jaundice and abdominal pain improved, prednisone was gradually reduced to medication discontinuation. Two months ago, the patient developed melena, whose blood routine test showed severe anemia, and gastrointestinal bleeding was diagnosed. The patient came to the emergency department of Beijing Hospital with no improvement after treatment in other hospitals. Gastroscopy revealed a 1.5 cm firm duodenal bulb ulcer. After treatment with omeprazole, the fecal occult blood was still positive. The PET-CT examination was performed, and it revealed no abnormality in the metabolic activity of the duodenal wall, and no neoplastic lesions were found. IgG4-related disease was considered, and the patient was admitted to the Department of Rheumatology and Immunology of Beijing Hospital for further diagnosis and treatment. The patient had a right submandibular gland mass resection history and diabetes mellitus. After the patient was admitted to the hospital, the blood test was reevaluated. The serum IgG4 was elevated at 5.44 g/L (reference value 0.03-2.01 g/L). Enhanced CT of the abdomen showed that the pancreas was mild swelling and was abnormally strengthened, with intrahepatic and extrahepatic bile duct dilation and soft tissue around the superior mesenteric vessels. We pathologically reevaluated and stained biopsy specimens of duodenal bulbs for IgG and IgG4. Immunohistochemical staining revealed remarkable infiltration of IgG4-positive plasma cells into duodenal tissue, the number of IgG4-positive cells was 20-30 cells per high-powered field, and the ratio of IgG4/IgG-positive plasma cells was more than 40%. The patient was treated with intravenous methylprednisolone at 40 mg daily dosage and cyclophosphamide, and then the duodenal ulcer was healed. IgG4 related disease is an immune-medicated rare disease characterized by chronic inflammation and fibrosis. It is a systemic disease that affects nearly every anatomic site of the body, usually involving multiple organs and diverse clinical manifestations. The digestive system manifestations of IgG4-related disease are mostly acute pancreatitis and cholangitis and rarely manifest as gastrointestinal ulcers. This case confirms that IgG4-related disease can present as a duodenal ulcer and is one of the rare causes of duodenal ulcers.
Aged ; Humans ; Male ; Abdominal Pain/drug therapy* ; Acute Disease ; Bilirubin ; Duodenal Ulcer/etiology* ; Immunoglobulin G ; Immunoglobulin G4-Related Disease/diagnosis* ; Pancreatitis/drug therapy* ; Positron Emission Tomography Computed Tomography ; Prednisone/therapeutic use* ; Pruritus/drug therapy*

Acute esophageal necrosis (AEN) is a very rare disorder typically presenting as a diffuse black esophageal mucosa on upper endoscopy. For this reason, AEN is often considered to be synonymous with 'black esophagus'. The pathogenesis of entity is still unknown. We report a case of AEN with duodenal ulcer causing partial gastric outlet obstruction. A 53-year-old man presented with hematemesis after repeated vomiting. The upper gastrointestinal endoscopy revealed circumferential black coloration on middle 315 to lower esophageal mucosa that stopped abruptly at the gastroesophageal junction. Pyloric ring deformity and active duodenal ulceration with extensive edema was observed. After conservative management with NPO and intravenous proton pump inhibitor, he showed clinical and endoscopic improvement. He resumed an oral diet on day 7 and was discharged. In our case the main pathogenesis of disease could be accounted for massive esophageal reflux due to transient gastric outlet obstruction by duodenal ulcer and following local ischemic injury.
Acute Disease ; Duodenal Ulcer/drug therapy/etiology ; Endoscopy, Gastrointestinal ; Esophageal Diseases/complications/*diagnosis/drug therapy ; Esophagus/*pathology ; Gastric Outlet Obstruction/*complications/pathology ; Humans ; Ischemia/pathology ; Male ; Middle Aged ; Necrosis ; Proton Pump Inhibitors/therapeutic use ; Tomography, X-Ray Computed

OBJECTIVETo study the long-term curative effects of suture plus proximal gastric vagotomy (PGV) and suture plus triad-therapy (omeprazole, amoxycillin and flagyl taken orally) for the treatment of duodenal ulcer with acute perforation.

METHODSThree hundred and twenty-nine patients with duodenal ulcer and acute perforation were treated with 2 different methods, respectively. Method A was suture plus PGV (group A, 153 cases), and method B was suture plus triad-therapy (group B, 176 cases). Follow-up was made by means of correspondence, outpatient reexamination and cooperation with local hospitals in 5 to 8 years after operation. The contents of follow-up included symptom acquisition (such as upper abdominal pain or distention, pyrosis, belch, acid regurgitation, vomiting, diarrhea and conditions of living or working), gastroscopy and Helicobacter pylori (HP) detection. The curative effects were evaluated by the Visick scale.

RESULTSThree hundred and one patients were followed up (group A 142 and group B 159). According to the Visick scale, 97 (68.3%), 19 (13.4%), 13 (9.15%) and 13 (9.15%) patients in group A, and 31 (19.5%), 28 (17.6%), 24 (15.1%) and 76 (47.8%) in group B were classified as Visick I, II, III and IV respectively (Z = -9.818, P < 0.01). As for HP detection, there were 130 (91.5%) patients in group A and 94 (59.1%) in group B (chi(2) = 41.438, P < 0.01).

CONCLUSIONSThe long-term curative effects of suture plus PGV were superior to those of suture plus triad-therapy for duodenal ulcer with acute perforation although HP positive rate was higher in group A than in group B. HP infection is one of the etiological factors of duodenal ulcer. The increased excitability of the vagus nerve remains to play an important role in duodenal ulcer.

Acute Disease ; Adolescent ; Adult ; Aged ; Amoxicillin ; administration & dosage ; Drug Therapy, Combination ; Duodenal Ulcer ; drug therapy ; etiology ; surgery ; Female ; Follow-Up Studies ; Humans ; Male ; Metronidazole ; administration & dosage ; Middle Aged ; Omeprazole ; administration & dosage ; Peptic Ulcer Perforation ; drug therapy ; surgery ; Suture Techniques ; Vagotomy, Proximal Gastric ; methods