Review of IgG4-related disease

Sánchez-Oro, Raquel; Alonso-Muñoz, Elsa María; Romero, Lidia Martí

doi:10.1016/j.gastre.2019.08.002

Cited by 21 publications

(36 citation statements)

References 49 publications

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“…If patients present with any typical clinical, serological or radiographic results, the clinical diagnosis sometimes can be made without pathological biopsy examinations[ 17 ]. If the patient has typical imaging findings of IgG4-RD, such as sausage-shaped pancreas[ 3 , 18 ] and periarteritis affecting the aorta below the renal artery, combined with relevant clinical manifestations and serological data, clinical diagnosis of IgG4-RD should be considered[ 10 ].…”

Section: Discussionmentioning

confidence: 99%

Immunoglobulin G4-related disease involving multiple systems: A case report

An¹,

Ma²,

Liu³

2022

WJCC

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BACKGROUND IgG4-related disease (IgG4-RD), an immune-mediated chronic progressive fibroinflammatory disease, can affect the functions of several organs. Some common characteristics can be observed in different IgG4-RDs, such as higher prevalence in middle-aged and elderly male patients, raised serum IgG4 levels, abundant infiltration of IgG4-positive cells and fibrosis, diffuse or localized swelling of the affected organs, and good response to glucocorticoids treatment. CASE SUMMARY A 72-year-old man complained of left upper abdominal pain 3 mo ago, and he was diagnosed with acute onset of chronic cholecystitis and acute pancreatitis in the local hospital. Pain improved after relevant treatment. Several days ago, his abdominal pain worsened, and he was admitted to our hospital for further treatment. Doppler ultrasound showed that the pancreas presented with sausage-like swelling and the parenchymal echo was diffusely reduced. Gallbladder volume was increased, while the wall was rough and thickened with bilateral signs. Furthermore, the left submandibular gland was enlarged, accompanied with significantly increased blood flow signals. Finally, we found that the adventitia of the abdominal aorta and right iliac artery was thickened locally. Serum IgG4 was elevated to 12600 mg/L. Therefore, the patient was diagnosed with IgG4-RD. After treatment with methylprednisolone, he had an uneventful course and was discharged in good condition. CONCLUSION IgG4-RD can involve almost any organs. Ultrasound has a significant role in timely and accurately diagnosis.

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Section: Discussionmentioning

confidence: 99%

Immunoglobulin G4-related disease involving multiple systems: A case report

An¹,

Ma²,

Liu³

2022

WJCC

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“…IgG4-AIH is not recognized as a diagnostic category in the AIH guideline specification[ 13 ]. IgG4-RD is an immune disease of the systemic system, and its pathological features include non-infectious and non-neoplastic inflammation and fibrosis[ 1 ]. Therefore, it is reasonable to believe that the liver is not an organ that can avoid injury.…”

Section: Discussionmentioning

confidence: 99%

“…IgG4-related disease (IgG4-RD) is a new autoimmune disease mediated by immunity[ 1 ]. This kind of disease is closely related to IgG4 lymphocytes, with increased serum IgG4 levels and IgG4-positive plasma cells infiltrating many organs and tissues.…”

Section: Introductionmentioning

confidence: 99%

Is simultaneous presence of IgG4-positive plasma cells and giant-cell hepatitis a coincidence in autoimmune hepatitis? A case report

Ya¹,

Wang²,

Chen³

2021

WJCC

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BACKGROUND The immune-mediated invasion of IgG4-positive plasma cells in the liver is found in some autoimmune hepatitis. Giant-cell hepatitis (GCH) is a very rare pathological feature in adults, and the clinical characteristics of the simultaneous appearance of the two pathological phenomena are not clear. CASE SUMMARY A 68-year-old woman was hospitalized with fatigue, poor appetite, and yellow urine for 20 d. Liver function tests and immunological indexes were significantly abnormal and accompanied by elevated serum IgG4 levels. Liver pathology revealed severe inflammation of the interface between the portal tract and hepatocytes, portal area inflammation, plasma cell infiltration, formation of rosette cells, IgG4-positive plasma cells > 10/high-power field, IgG4/IgG > 40%, and multinucleated liver cell swelling. IgG4-related autoimmune hepatitis (AIH) combined with GCH was diagnosed, and methylprednisolone was administered at 40 mg/day. Two weeks later, the clinical symptoms disappeared, and the liver function and immunological indicators were significantly improved. Methylprednisolone was reduced at a rate of 4–8 mg per week to 8 mg/day for maintenance. A second liver biopsy 48 wk later indicated that liver inflammation and fibrosis were significantly improved. IgG4-positive plasma cells and GCH were not detected. A literature search was conducted to analyze articles reporting similar pathological phenomena. CONCLUSION AIH with simultaneous IgG4-positive plasma cell infiltration and GCH, liver inflammation, and fibrosis is possibly more severe than typical AIH but sensitive to corticosteroids.

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“…When IgG4-RD is accompanied by a malignancy, the malignancy may occur in multiple organs [ 16 ], which can occur before, during, or after the diagnosis of IgG4-RD. The sequence of the two diseases remains uncertain.…”

Section: Discussionmentioning

confidence: 99%

Focal IgG4-related autoimmune pancreatitis with distal choledochal adenocarcinoma: a rare case report

et al. 2021

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Background Autoimmune pancreatitis (AIP) is a rare disease that manifests as pancreatic involvement in systemic IgG4-related disease (IgG4-RD), a special type of chronic pancreatitis caused by autoimmune abnormalities. The main imaging manifestations of IgG4-related AIP consist of diffuse or localized pancreatic enlargement and irregular pancreatic duct narrowing. The diagnosis of AIP is challenging because it can present with focal lesions, similar to radiologically bile duct cancer or pancreatic cancer. Case presentation A 55-year-old male patient was admitted with painless jaundice and multiple radiographic findings of pancreatic head mass, as well as intrahepatic and extrahepatic bile duct dilatation. Various imaging methods indicated pancreatic cancer. However, the endoscopic ultrasonography guided fine needle aspiration (EUS-FNA) and a laparoscopic pancreatic biopsy suggested an IgG4-related AIP. After one month, magnetic resonance imaging showed that the lesion had slightly grown. Combined with CA19-9 and other indexes, the possibility of malignancy was high and there were still surgical indications. The pathological analysis following a pancreaticoduodenectomy revealed poorly differentiated adenocarcinoma in the distal common bile duct. Conclusion To date, few reports have described pancreatic or extrapancreatic malignancies in AIP patients, and no association between AIP and bile duct adenocarcinoma has been previously confirmed. This case discuss the differentiation between AIP and malignancy, recent research progress, and the correlation between the two diseases, highlights the importance of carefully evaluating patients with AIP to rule out potential tumors, as well as the critical need for follow up treatment.

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Review of IgG4-related disease

Cited by 21 publications

References 49 publications

Immunoglobulin G4-related disease involving multiple systems: A case report

Immunoglobulin G4-related disease involving multiple systems: A case report

Is simultaneous presence of IgG4-positive plasma cells and giant-cell hepatitis a coincidence in autoimmune hepatitis? A case report

Focal IgG4-related autoimmune pancreatitis with distal choledochal adenocarcinoma: a rare case report

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