Kidney involvement in the Schnitzler syndrome, a rare disease

6Citations
Citations of this article
12Readers
Mendeley users who have this article in their library.

This article is free to access.

Abstract

The Schnitzler syndrome (SS) is a rare and underdiagnosed entity that associates a chronic urticarial rash, monoclonal IgM (or sometimes IgG) gammopathy and signs and symptoms of systemic inflammation. During the past 45 years, the SS has evolved from an elusive little-known disorder to the paradigm of a late-onset acquired auto-inflammatory syndrome. Though there is no definite proof of its precise pathogenesis, it should be considered as an acquired disease involving abnormal stimulation of the innate immune system, which can be reversed by the interleukin-1 receptor antagonist anakinra. It clearly expands our view of this group of rare genetic diseases and makes the concept of auto-inflammation relevant in polygenic acquired diseases as well. Increasing numbers of dermatologists, rheumatologists, allergologists, haematologists and, more recently, nephrologists, recognize the SS. The aim of this review is to focus on kidney involvement in the SS. Although the literature regarding kidney involvement in the SS is very poor it can be severe, as in our own case here reported, leading us to recommend the systematic search for nephropathy markers in the SS.

Cite

CITATION STYLE

APA

Basile, C., Rossi, L., Casucci, F., Teutonico, A., Libutti, P., Lisi, P., … Manna, R. (2017, December 1). Kidney involvement in the Schnitzler syndrome, a rare disease. Clinical Kidney Journal. Oxford University Press. https://doi.org/10.1093/ckj/sfx077

Register to see more suggestions

Mendeley helps you to discover research relevant for your work.

Already have an account?

Save time finding and organizing research with Mendeley

Sign up for free