eISSN: 2299-0046
ISSN: 1642-395X
Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii
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2/2018
vol. 35
 
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abstract:
Original paper

ADAMTS13 deficiency and immunological abnormalities in patients with systemic sclerosis

Zofia Gerlicz-Kowalczuk
,
Jolanta D. Torzecka
,
Elżbieta Dziankowska-Zaborszczyk
,
Alicja Ograczyk
,
Anna M. Zalewska-Janowska
,
Anna Woźnicka
,
Bożena Dziankowska-Bartkowiak

Adv Dermatol Allergol 2018; XXXV (2): 182–184
DOI: https://doi.org/10.5114/ada.2018.75240
Online publish date: 2018/04/24
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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.

keywords:

systemic sclerosis, ADAMTST13
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