Immunoglobulin G4-related systemic disorder (IgG4-RD) is an under-diagnosed fibro-inflammatory disorder with unknown etiology and multiorgan involvement. IgG4-RD manifestations consist of lymphadenopathy, extrapancreatic bile duct lesions, lacrimal and salivary gland lesions, hypothyroidism, retroperitoneal fibrosis, and kidney mass. We report the case of a 46-yearold patient, with moderate persistent asthma and chronic hyperplastic rhinosinusitis, who presented at emergency department because of obstructive jaundice due to a mass in the pancreatic head. The pathology of the surgical specimen disclosed lymphoplasmacytic inflammation with fibrosis of intra and inter-lobular septs and ducts ectasia and abundant infiltration of plasmacytes, most of whom being IgG4-positive (> 200/hpf). These findings were consistent with the diagnosis of IgG4-RD. The patient’s medical history suggests pancreatitis is the last piece of the jigsaw puzzle that initiated years before with swelling of submandibular glands and and enlarged lacrimal glands.

Autoimmune pancreatitis: The Last Puzzle Piece of IgG4-Related Disease

BELLOCCHIA, Michela Maria;BOITA, MONICA;HEFFLER, Enrico Marco;Paolo, Solidoro;BUCCA, Caterina;ROLLA, Giovanni
2016-01-01

Abstract

Immunoglobulin G4-related systemic disorder (IgG4-RD) is an under-diagnosed fibro-inflammatory disorder with unknown etiology and multiorgan involvement. IgG4-RD manifestations consist of lymphadenopathy, extrapancreatic bile duct lesions, lacrimal and salivary gland lesions, hypothyroidism, retroperitoneal fibrosis, and kidney mass. We report the case of a 46-yearold patient, with moderate persistent asthma and chronic hyperplastic rhinosinusitis, who presented at emergency department because of obstructive jaundice due to a mass in the pancreatic head. The pathology of the surgical specimen disclosed lymphoplasmacytic inflammation with fibrosis of intra and inter-lobular septs and ducts ectasia and abundant infiltration of plasmacytes, most of whom being IgG4-positive (> 200/hpf). These findings were consistent with the diagnosis of IgG4-RD. The patient’s medical history suggests pancreatitis is the last piece of the jigsaw puzzle that initiated years before with swelling of submandibular glands and and enlarged lacrimal glands.
23
25
Michela, Bellocchia; Monica, Boita; Enrico, Heffler; Stefano, Pizzimenti; Domenico, Novero; Paolo, Solidoro; Caterina, Bucca; Giovanni, Rolla
File in questo prodotto:
File Dimensione Formato  
J_J_Aller_Immuno_3_1_023-IgG4.pdf

Accesso aperto

Tipo di file: PDF EDITORIALE
Dimensione 745.79 kB
Formato Adobe PDF
745.79 kB Adobe PDF Visualizza/Apri

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2318/1592877
Citazioni
  • ???jsp.display-item.citation.pmc??? ND
  • Scopus ND
  • ???jsp.display-item.citation.isi??? ND
social impact