title: IgG4-related multiorgan disease: report of the first autopsy case.
abstract: IgG4-related disease (IgG4RD) is a chronic recurring fibro-inflammatory pathology that is considered to be of autoimmune origin. Histopathology is considered to be the gold standard method for diagnosis. IgG4RD affects multiple organs. IgG4RD was first identified in the pancreas and was called autoimmune pancreatitis (AIP). During the following years, the disease spectrum was expanded and it was realised that the extrapancreatic lesions can precede, coexist or appear after the diagnosis of AIP. At present, several illnesses such as Mikulicz disease, Küttner tumour, multifocal fibrosclerosis, etc, are considered to be part of the IgG4RD spectrum. The symptoms of the disease tend to appear over months and years and diagnosis is achieved on average 13.5 months (4-60 months) after the onset. The purpose of this report was to provide information about a case that was sadly fatal but that permitted a complete histopathological study of the damaged tissues.
authors: Department of Pathology, Hospital General de México, Mexico City, Mexico DF, Mexico.
authors: Minerva Lazos
authors: ML
authors: Ochoa
authors: Belem Gabiño
authors: BG
authors: López
authors: Raúl Romero
authors: RR
authors: Cabello
authors: Raúl Romero
authors: RR
authors: Feregrino
mesh_terms: D001327:Autoimmune Diseases / Q000097:blood / Q000276:immunology / Q000473:pathology*; D001344:Autopsy; D017809:Fatal Outcome; D005260:Female; D005355:Fibrosis / Q000097:blood / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D008875:Middle Aged; D008882:Mikulicz' Disease; D012185:Retroperitoneal Fibrosis / Q000151:congenital
chemical_list: D007074:Immunoglobulin G
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