A disclosed diagnosis for 24 year’s unknown illness

• Published in: BMJ case reports Vol. 2017; p. bcr-2017-221317
• Main Authors: Hanai, Shogo, Kinjo, Kazushi, Uezato, Madoka, Tokuda, Yasuharu
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group LTD 20-12-2017; BMJ Publishing Group
• Series: Case Report
• Subjects: Abdomen; Abdominal Pain - diagnosis; Abdominal Pain - etiology; Anti-Inflammatory Agents - therapeutic use; Australasia; Autoimmune diseases; Autoimmune Diseases - diagnosis; Biopsy; Case reports; Cholangitis - diagnosis; Cholangitis - physiopathology; Chronic illnesses; Diagnosis, Differential; Endoscopy; Epidemiology; Family medical history; Fatal Outcome; Female; Histology; Humans; Immunoglobulin G - blood; Kidney diseases; Male; Medical diagnosis; Medical imaging; Middle Aged; New Disease; Pain; Pancreatic cancer; Pancreatitis; Pancreatitis, Chronic - diagnosis; Pancreatitis, Chronic - physiopathology; Patients; Prednisolone - therapeutic use; Retroperitoneal Fibrosis - diagnosis; Retroperitoneal Fibrosis - physiopathology; Stroke; Time Factors; Tomography, X-Ray Computed; Tumors; White; Chronic Renal Failure; Pancreatitis
• ISSN: 1757-790X; 1757-790X
• DOI: 10.1136/bcr-2017-221317

IgG4-related disease (IgG4-RD) is a newly described illness over the last several years. A 57-year-old man, who had been followed for chronic kidney disease (CKD), chronic pancreatitis and history of operated cholangitis, was admitted to our hospital for abdominal pain and worsening renal function. Serum levels of IgG and IgG4 were elevated. CT scan showed the characteristic findings of IgG4-related retroperitoneal fibrosis, pancreas and kidney disease. An endoscopic biopsy revealed the finding compatible with IgG4-RD. Steroid therapy led to the remission of his abdominal pain. Patients with CKD of unknown aetiology may have IgG4-RD.