Author/Authors :
Gerlicz-Kowalczuk, Zofia Department of Psychodermatology - Medical University of Lodz, Poland , Jolanta, DTorzecka Department of Dermatology and Venereology - Medical University of Lodz, Poland , Dziankowska-Zaborszczyk, Elżbieta Department of Preventive Medicine - Medical University of Lodz, Poland , Ograczyk, Alicja Department of Psychodermatology - Medical University of Lodz, Poland , Zalewska-Janowska, Anna M. Department of Psychodermatology - Medical University of Lodz, Poland , Woźnicka, Anna Department of Dermatology and Venereology - Medical University of Lodz, Poland , Dziankowska-Bartkowiak, Bożena Department of Dermatology and Venereology - Medical University of Lodz, Poland
Abstract :
Introduction
Systemic sclerosis (SSc) is a chronic autoimmune connective tissue disorder characterized by immunological deviations and generalized microvascular damage.
Aim
To determine the serum level of the von Willebrand factor cleaving protease (ADAMTS13) in 39 SSc patients and healthy controls.
Material and methods
ADAMTS13 serum level was determined in 39 SSc patients and 11 healthy controls. Complete history of the patients was recorded and thorough clinical, rheumatological, and dermatological examinations were performed.
Results
The serum levels of ADAMTS13 were significantly lower in SSc than in normal controls (455.47 ±128 vs. 702.01 ±142 ng/ml, p < 0.00001). However significant correlations among serum ADAMTS 13 levels and organ changes were not found in SSc patients.
Conclusions
We demonstrate a decreased serum level of ADAMTS13 in SSc patients, which may contribute to the vessel microangiopathy observed in systemic sclerosis.
