Santiago Cedeño
1*, Ursula Verdalles
1, Marian Goicoechea
1, Soledad García de Vinuesa
1, José Luño
11 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.