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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
ADAMTS13 deficiency and immunological abnormalities in patients with systemic sclerosis
Zofia Gerlicz-Kowalczuk1, Jolanta D Torzecka2, Elżbieta Dziankowska-Zaborszczyk3
1Department of Psychodermatology, Medical University of Lodz, Lodz, Poland.
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.
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