Prethodno priopćenje

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

Sažetak

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.
References:
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383–98. doi: 10.1016/j.rdc.2015.04.003. Epub 2015 May 27.
2. Launay D, Sobanski V, Hachulla E, Humbert M. Pulmonary hypertension in systemic sclerosis: diff erent phenotypes, Eur Respir Rev.
2017 Sep 27;26(145). pii: 170056. doi: 10.1183/16000617.0056–2017. Print 2017 Sep 30.
3. Ingegnoli F, Ardoino I, Boracchi P, Cutolo M; EUSTAR co-authors. Nailfold capillaroscopy in systemic sclerosis: data from the EULAR
scleroderma trials and research (EUSTAR) database. Microvasc Res. 2013 Sep;89:122–8. doi: 10.1016/j.mvr.2013.06.003. Epub 2013
Jun 17.
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sclerosis: where do we stand?, European Respiratory Review 2015 24: 411–419; DOI: 10.1183/16000617.00002915
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online 2015 Apr 15. doi: 10.3389/fi mmu.2015.00167

Ključne riječi

Hrčak ID:

216017

URI

https://hrcak.srce.hr/216017

Datum izdavanja:

5.12.2018.

Posjeta: 593 *