Preliminary communication

CLINICAL COURSE OF SYSTEMIC SCLEROSIS PATIENTS

Monika Chrzanowska ; Department of Internal Medicine and Rheumatology, Medical University of Silesia in Katowice
Magdalena Włoch-Targońska ; Department of Internal Medicine and Rheumatology, Medical University of Silesia in Katowice
Przemysław Kotyla ; Department of Internal Medicine and Rheumatology, Medical University of Silesia in Katowice
Eugeniusz J. Kucharz ; Department of Internal Medicine and Rheumatology, Medical University of Silesia in Katowice

Abstract

Background: Systemic sclerosis is a rare multisystem inflammatory connective tissue disorder of unclear etiology, characterized by progressive fibrosis of skin and internal organs as well as systemic vascular dysfunction. Disease severity is determined by the degree of internal organ involvement, especially pulmonary hypertension, interstitial lung disease, and peripheral vasculopathy.
Objectives: Th e aim of this study was to analyze a group of patients diagnosed with systemic sclerosis.
Methods: We retrospectively analyzed a group of 70 patients (42 women and 28 men) treated in the Department of Internal Medicine and Rheumatology, SUM, Katowice, Poland between 2013 and 2016. Patients were an average of 54.0 years old (+/–, range). Our study examined the incidence of cardiovascular and musculoskeletal symptoms as well as basic blood chemistry and imaging studies. We also assessed the degree of skin thickness by the Modified Rodnan Skin score (mRSS).
Results: Among study group participants, 67 patients tested positive for ANA, 51- Scl-70, 6-ACA antibodies while 3 patients were not tested. Most patients had an abnormal nailfold capillaroscopy with a sclerodermal pattern, joint pain and features of interstitial lung disease on HRCT. 30% of patients demonstrated pulmonary hypertension and anemia, 7.1% chronic kidney disease, and 15.7% finger ulcers. Most patients were treated with immunosuppressants, and 9 subjects underwent autologous of stem cell transplantation from peripheral blood.
Conclusions: Our results are comparable to other similar studies seen in the literature. A team based approach involving physicians of different specialties as well as immunosuppressive therapy (cyclophosphamide, mycophenolate mofetil) and autologous transplantation of stem cells from peripheral blood, can greatly increase quality of life and survival of patients with systemic sclerosis. It is clear that systemic sclerosis requires further extensive study.
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Keywords

Hrčak ID:

216017

URI

https://hrcak.srce.hr/216017

Publication date:

5.12.2018.

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