title: The many faces of Merlin: IgG4-associated pulmonary-renal disease.
abstract: Pulmonary-renal syndrome is a common and serious disorder with a broad differential diagnosis. We describe a case of a middle-aged man presenting with interstitial pulmonary disease and severe renal impairment caused by a hypocomplementemic immune-complex-mediated interstitial nephritis. Serum levels of IgG4 were elevated, and renal biopsy specimens revealed the presence of interstitial IgG4(+) plasma cells. There was a rapid improvement of both pulmonary and renal abnormalities after the initiation of corticosteroids. To our knowledge, this report is the first to show interstitial pulmonary disease in association with interstitial kidney disease as the predominant and presenting symptoms of IgG4-related disease.
authors: Department of Nephrology, University Hospitals Leuven, Leuven, Belgium.
authors: Ben
authors: B
authors: Sprangers
authors: Department of General Internal Medicine, University Hospitals Leuven, Leuven, Belgium.
authors: Pieter
authors: P
authors: Lioen
authors: Department of Nephrology, University Hospitals Leuven, Leuven, Belgium.
authors: Björn
authors: B
authors: Meijers
authors: Department of Pathology, University Hospitals Leuven, Leuven, Belgium.
authors: Evelyne
authors: E
authors: Lerut
authors: Department of Radiology, University Hospitals Leuven, Leuven, Belgium.
authors: Joke
authors: J
authors: Meersschaert
authors: Department of General Internal Medicine, University Hospitals Leuven, Leuven, Belgium.
authors: Daniel
authors: D
authors: Blockmans
authors: Department of Nephrology, University Hospitals Leuven, Leuven, Belgium. Electronic address: kathleen.claes@uzleuven.be.
authors: Kathleen
authors: K
authors: Claes
mesh_terms: D003404:Creatinine / Q000097:blood; D006801:Humans; D007105:Immune Complex Diseases / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology; D007074:Immunoglobulin G / Q000378:metabolism*; D007150:Immunohistochemistry; D017563:Lung Diseases, Interstitial / Q000175:diagnosis* / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009395:Nephritis, Interstitial / Q000175:diagnosis* / Q000276:immunology / Q000378:metabolism / Q000473:pathology
chemical_list: D007074:Immunoglobulin G; D003404:Creatinine