title: IgG4-related systemic disease in a Native American man.
abstract: IgG4-related systemic disease is a recently described entity that can elude even the most astute diagnostician. Patients with the disease, characterized by the infiltration of polyclonal IgG4-positive plasmacytes, can present with single or multi-organ involvement. Manifestations include dacryoadenitis, sialadenitis, thyroiditis, pneumonitis, retroperitoneal fibrosis, pancreatitis, sclerosing cholangitis, tubulointerstitial nephritis, prostatitis, and hypophysitis. We describe a biopsy-confirmed case with extensive multi-organ involvement, including hypophysitis, dacryoadenitis, retroperitoneal fibrosis and tubulointerstitial nephritis. By reporting this case, we hope to bring IgG4-related systemic disease to the attention of the broader medical community as it is an elusive disease that commonly responds to systemic corticosteroids.
authors.affiliation: Department of Medicine, Mayo Clinic, USA.
authors.forename: Sandeep M
authors.initials: SM
authors.lastname: Patel
authors.forename: Jason H
authors.initials: JH
authors.lastname: Szostek
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic / Q000378:metabolism; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D015209:Cholangitis, Sclerosing / Q000276:immunology / Q000473:pathology; D003607:Dacryocystitis / Q000276:immunology / Q000473:pathology; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000378:metabolism*; D007198:Indians, North American; D008297:Male; D008875:Middle Aged; D009395:Nephritis, Interstitial / Q000276:immunology / Q000473:pathology; D010195:Pancreatitis / Q000276:immunology / Q000473:pathology; D010902:Pituitary Gland / Q000276:immunology / Q000473:pathology; D010950:Plasma Cells / Q000276:immunology* / Q000473:pathology*; D011014:Pneumonia / Q000276:immunology / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use; D011472:Prostatitis / Q000276:immunology / Q000473:pathology; D012185:Retroperitoneal Fibrosis / Q000276:immunology / Q000473:pathology; D012793:Sialadenitis / Q000276:immunology / Q000473:pathology; D013967:Thyroiditis, Autoimmune / Q000276:immunology / Q000473:pathology
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D007074:Immunoglobulin G; D011241:Prednisone