title: Chronic Tubulo-interstitial Pyelonephritis with Retroperitoneal Fibrosis.
abstract: A large proportion of patients with retroperitoneal fibrosis (RPF) have been labelled as having idiopathic RPF historically. Recent reports of a subset of these patients having a fibro-inflammatory condition with characteristic histology and good response to therapy has helped in renewed understanding of an old disease. A 40 year old man presented with vague abdominal discomfort of 5 months duration. Ultrasound examination revealed soft tissue thickening with calcification over lower abdominal aorta. CT scan characterized the lesion as a retroperitoneal mass encasing the aorta with an associated lesion in upper pole of left kidney. A laparoscopic surgery and biopsy of the mass lesions were performed. It was reported as chronic tubulo-interstitial disease with retroperitoneal fibrosis. In the post-operative period, patient developed fever with weight loss. An 18F-FDG PET-CT done revealed FDG-avid retroperitoneal mass lesion. Serum IgG-4 levels were elevated and review of biopsy revealed features of IgG4 related disease. Patient had good response to treatment with steroids and azathioprine.
authors.affiliation: Department of Internal Medicine.
authors.forename: Manish
authors.initials: M
authors.lastname: Soneja
authors.affiliation: Department of Internal Medicine.
authors.forename: S
authors.initials: S
authors.lastname: Achintya
authors.affiliation: Department of Internal Medicine.
authors.forename: Neeraj
authors.initials: N
authors.lastname: Nischal
authors.affiliation: Department of Internal Medicine.
authors.forename: Piyush
authors.initials: P
authors.lastname: Ranjan
authors.affiliation: Radiodiagnosis.
authors.forename: Surabhi
authors.initials: S
authors.lastname: Vyas
authors.affiliation: Pathology.
authors.forename: Sudheer
authors.initials: S
authors.lastname: Arava
authors.affiliation: Professor and Head, Department of Medicine, All India Institute of Medical Sciences, New Delhi.
authors.forename: S K
authors.initials: SK
authors.lastname: Sharma
mesh_terms: D000328:Adult; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D001379:Azathioprine / Q000627:therapeutic use; D001706:Biopsy; D002908:Chronic Disease; D019788:Fluorodeoxyglucose F18; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology*; D010535:Laparoscopy; D008297:Male; D000072078:Positron Emission Tomography Computed Tomography / Q000379:methods*; D011704:Pyelonephritis / Q000188:drug therapy / Q000473:pathology*; D012185:Retroperitoneal Fibrosis / Q000000981:diagnostic imaging* / Q000276:immunology* / Q000628:therapy; D012187:Retroperitoneal Space / Q000473:pathology; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G; D019788:Fluorodeoxyglucose F18; D001379:Azathioprine