title: IgG4-related retroperitoneal fibrosis: A case report and review of literature.
abstract: Idiopathic Retroperitoneal fibrosis is a rare clinical condition recently identified as an autoimmune process related to Immunoglobulin G4 (IgG4) deposition. Herein we report a case of a 46 year old male presenting with 4 months history of backache, fever, flank pain and leg swelling for 2 weeks. Investigations revealed acute kidney injury diagnosed as a result of bilateral ureteric obstruction. This was later confirmed to be retroperitoneal fibrosis on CT scan and biopsy. Histopathology was consistent with IgG4 related disease. Treatment with immunosuppressive agents showed reduction in the fibrosis and normalization of the kidney functions. We discuss the IgG4 related retroperitoneal fibrosis in detail along with its varied presentations.
authors.affiliation: Department of Medicine, Division of Nephrology, Fatima Memorial Hospital, Nur International University, Lahore, Pakistan.
authors.forename: Azra
authors.initials: A
authors.lastname: Niaz
authors.affiliation: Department of Pathology, Shaukat Khanum Memorial Cancer Hospital, Lahore, Pakistan.
authors.forename: Asad Hayat
authors.initials: AH
authors.lastname: Ahmad
authors.affiliation: Department of Urology, Fatima Memorial Hospital, Nur International University, Lahore, Pakistan.
authors.forename: Muhammed Ahmad
authors.initials: MA
authors.lastname: Saeed
authors.affiliation: Department of Medicine, Division of Nephrology, Fatima Memorial Hospital, Nur International University, Lahore, Pakistan.
authors.forename: Omer
authors.initials: O
authors.lastname: Sabir
authors.affiliation: Department of Medicine, Division of Nephrology, Fatima Memorial Hospital, Nur International University, Lahore, Pakistan.
authors.forename: Nauman
authors.initials: N
authors.lastname: Tarif
mesh_terms: D034841:Abdominal Cavity / Q000000981:diagnostic imaging / Q000473:pathology; D058186:Acute Kidney Injury / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D001416:Back Pain / Q000175:diagnosis / Q000209:etiology; D001706:Biopsy / Q000379:methods; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis / Q000276:immunology; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D007677:Kidney Function Tests / Q000379:methods; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage*; D008875:Middle Aged; D012185:Retroperitoneal Fibrosis / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D015607:Stents; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome; D014517:Ureteral Obstruction / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D013520:Urologic Surgical Procedures / Q000379:methods*
chemical_list: D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D008775:Methylprednisolone
keywords: IgG4-related disease, Retroperitoneal fibrosis, Ureteric obstruction, Acute renal failure.