This article is part of the supplement: 7th European Workshop on Immune-Mediated Inflammatory Diseases

Open Access Poster presentation

Autoimmunity and cystatin SA 1 deficiency behind chronic mucocutaneous candidiasis in autoimmune polyendocrine syndrome

Emma Lindh1*, Johan Brännström1, Petra Jones1, Fredrik Wermeling1, Signe Hässler1, Corrado Betterle2, Ben Z Garty3, Mats Stridsberg4, Björn Herrmann5, Mikael CI Karlsson1 and Ola Winqvist1

  • * Corresponding author: Emma Lindh

Author Affiliations

1 Translational Immunology Unit, Dept. of Medicine, Karolinska Institutet, Stockholm, Sweden

2 Endocrinology Unit, Dept. of Medical and Surgical Sciences, University of Padua, Padua, Italy

3 Dept. of Pediatrics, Schneider Children's Medical Center of Israel, Tel Aviv University, Israel

4 Section of Clinical Chemistry, Dept. of Medical Sciences, Uppsala University Hospital, Uppsala, Sweden

5 Section of Clinical Bacteriology, Dept. of Medical Sciences, Uppsala University Hospital, Uppsala, Sweden

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Journal of Translational Medicine 2012, 10(Suppl 3):P58 doi:10.1186/1479-5876-10-S3-P58


The electronic version of this article is the complete one and can be found online at: http://www.translational-medicine.com/content/10/S3/P58


Published:28 November 2012

© 2012 Lindh et al; licensee BioMed Central Ltd.

Poster presentation

Patients with the monogenic disease autoimmune polyendocrine syndrome type I (APS I) develop autoimmunity against multiple endocrine organs and suffer from chronic mucocutaneous candidiasis (CMC), a paradoxical complication with an unknown mechanism. We report that saliva from APS I patients with CMC was defective in inhibiting growth of C. albicans in vitro and had reduced levels of a salivary protein identified as cystatin SA1. In contrast, APS I patients with no CMC expressed salivary cystatin SA1 and could inhibit C. albicans to the same extent as healthy controls. We evaluated the anti-fungal activity of cystatin SA1 and found that synthesized full length cystatin SA1 efficiently inhibited growth of C. albicans in vitro. Moreover, APS I patients exhibited salivary IgA autoantibodies recognizing myosin-9, a protein expressed in the salivary glands that also produce cystatin SA1, thus linking autoimmunity to cystatin SA1 deficiency and CMC. This data suggests an autoimmune mechanism behind CMC in APS I and provide rationale for evaluating cystatin SA1 in antifungal therapy.