J Nephropathol. 2019;8(3): e32.
doi: 10.15171/jnp.2019.32
  Abstract View: 117
  PDF Download: 138

Case Report

An old disease, a new diagnosis; A 49 year-old man with nephrotic syndrome

Santiago Cedeño 1 * , Ursula Verdalles 1, Marian Goicoechea 1, Soledad García de Vinuesa 1, José Luño 1

1 Hospital General Universitario Gregorio Marañón, Madrid, Spain

Abstract

Background: IgG4-related disease (IgG4-RD) is a systemic immune-mediated disease that typically manifests as fibro-inflammatory masses that can affect nearly any organ system.

Case Report: We present here a case of a 49-year old man with forgotten old disease (Mikulicz disease) with membranous nephropathy (MN).

Conclusions: This entity is currently included within the spectrum of IgG4-related disease. The development of renal disease shortly after the suspension of rituximab suggests another probable pathway involved. To our knowledge the transforming growth factor may be responsible for existing pattern of fibrosis in this disease. The lack of response or at least partial response to rituximab can be explained by greater involvement of regulatory T lymphocyte in the pathophysiology of this entity

Implication for health policy/practice/research/medical education:

According to the criteria of IgG4-RD, it is probable that some of the patients diagnosed formerly as Sjogren’s syndrome actually match with this old disease known as Mikulicz’s disease and called IgG4-related sialoadenitis in the modern era.

Please cite this paper as: Cedeño S, Verdalles U, Goicoechea M, García de Vinuesa S, Luño J. An old disease, a new diagnosis; A 49 year-old man with nephrotic syndrome. J Nephropathol. 2019;8(3):e32. DOI: 10.15171/jnp.2019.32.

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Submitted: 14 Apr 2018
Accepted: 19 Jun 2019
First published online: 08 Aug 2019
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