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The Many Faces of MerlinIgG4 Disease Presented as Pulmonary-Renal Syndrome: IgG4-Associated Pulmonary-Renal Disease

Ben Sprangers, MD, PHD; Pieter Lioen, MD; Björn Meijers, MD, PHD; Evelyne Lerut, MD, PHD; Joke Meersschaert, MD, PHD; Daniel Blockmans, MD, PHD; Kathleen Claes, MD
Author and Funding Information

From the Department of Nephrology (Drs Sprangers, Meijers, and Claes), Department of General Internal Medicine (Drs Lioen and Blockmans), Department of Pathology (Dr Lerut), and Department of Radiology (Dr Meersschaert), University Hospitals Leuven, Leuven, Belgium.

Correspondence to: Kathleen Claes, MD, Department of Nephrology, University Hospitals Leuven, Herestraat 49, B-3000 Leuven, Belgium; e-mail: kathleen.claes@uzleuven.be


Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (http://www.chestpubs.org/site/misc/reprints.xhtml).


© 2011 American College of Chest Physicians


Chest. 2011;140(3):791-794. doi:10.1378/chest.10-3086
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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.

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