IgG4-related Systemic Disease in a Native American Man

• Published in: Internal Medicine Vol. 50; no. 8; pp. 931 - 934
• Main Authors: Patel, Sandeep M, Szostek, Jason H
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society of Internal Medicine 01.01.2011
• Subjects: Antibodies, Antineutrophil Cytoplasmic - metabolism; Autoimmune Diseases - diagnosis; Autoimmune Diseases - drug therapy; Autoimmune Diseases - immunology; Autoimmune Diseases - pathology; Cholangitis, Sclerosing - immunology; Cholangitis, Sclerosing - pathology; Dacryocystitis - immunology; Dacryocystitis - pathology; Humans; IgG4; IgG4-related systemic disease; Immunoglobulin G - blood; Immunoglobulin G - metabolism; Indians, North American; Male; Middle Aged; Nephritis, Interstitial - immunology; Nephritis, Interstitial - pathology; Pancreatitis - immunology; Pancreatitis - pathology; Pituitary Gland - immunology; Pituitary Gland - pathology; Plasma Cells - immunology; Plasma Cells - pathology; Pneumonia - immunology; Pneumonia - pathology; Prednisone - therapeutic use; Prostatitis - immunology; Prostatitis - pathology; Retroperitoneal Fibrosis - immunology; Retroperitoneal Fibrosis - pathology; Sialadenitis - immunology; Sialadenitis - pathology; Thyroiditis, Autoimmune - immunology; Thyroiditis, Autoimmune - pathology
• ISSN: 0918-2918; 1349-7235
• DOI: 10.2169/internalmedicine.50.4593

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.