SISTEMSKA SKLEROZA: PREGLED
| العنوان: | SISTEMSKA SKLEROZA: PREGLED |
|---|---|
| المؤلفون: | Dušanka Martinović Kaliterna |
| المصدر: | Reumatizam Volume 64 Issue suppl. 1 |
| بيانات النشر: | Croatian Society for Rheumatology of the CMA, 2017. |
| سنة النشر: | 2017 |
| مصطلحات موضوعية: | Sistemska skleroza – dijagnoza, etiologija, komplikacije, liječenje, Transformirajući čimbenik rasta beta, Intersticijska bolest pluća – etiologija, Plućna hipertenzija – etiologija, Raynaudova bolest – etiologija, Kožni ulkus – etiologija, Teleangiektazije – etiologija, Fibroza – etiologija, Miokard – patologija, Gastrointestinalne bolesti – etiologija, Imunosupresivni lijekovi – terapijska primjena, Antagonisti endotelin a receptora – terapijska primjena, Monoklonska protutijela – terapijska primjena, Scleroderma, systemic – complications, diagnosis, etiology, therapy, Transforming growth factor beta, Lung diseases, interstitial – etiology, Hypertension, pulmonary – etiology, Raynaud disease – etiology, Skin ulcer – etiology, Telangiectasis – etiology, Fibrosis – etiology, Myocardium – pathology, Gastrointestinal diseases – etiology, Immunosuppressive agents – therapeutic use, Endothelin a receptor antagonists – therapeutic use, Antibodies, monoclonal – therapeutic use |
| الوصف: | Sistemska skleroza (SSc) ubraja se u autoimunosne bolesti vezivnog tkiva. Obilježena je obliterativnim i proliferativnim mikrovaskularnim zbivanjima, aktivacijom imunosnog sustava i ekcesivnom fi brozom tkiva i organa. Istražuje se mogući genetski utjecaj na progresiju bolesti, kao i važnost SSc-protutijela pri postavljanju dijagnoze. Kriteriji SSc-a, postavljeni 2013. prema American College of Rheumatology/European League Against Rheumatism, znatno su osjetljiviji od prethodnih. Modifi cirani Rodnanov kožni test ostaje i dalje najbolja metoda za objektivnu procjenu kožne zahvaćenosti. Mikrovaskularne promjene prate se videokapilaroskopijom koja je korisna za ranu dijagnozu i prognozu bolesti. Plućne komplikacije, koje podrazumijevaju intersticijsku bolest pluća i plućnu arterijsku hipertenziju, mogu se nadograđivati i glavni su uzrok smrti u SSc-u. Fibroza miokarda povezana je s dijastoličkom disfunkcijom i visokim rizikom od srčanih aritmija. Nakon uvođenja ACE-inhibitora bubrežne su komplikacije poput sklerodermijske bubrežne krize rjeđe, ali su bolesnici koji uzimaju glukokortikoide izloženi riziku od oštećenja bubrega. Zahvaćenost jednjaka i anorektalne regije prvi je znak zahvaćanja gastrointestinalnog sustava. Mogu se javiti Barrettov jednjak, GAVE ili želudac poput lubenice, intestinalna pseudodivertikuloza, kao i teleangiektazije kolona. Imunosupresivna terapija preporučuje se pri difuznome kožnom SSc-u s brzo progresivnom intersticijskom bolesti pluća. Liječenje počinje ciklofosfamidom i nastavlja se azatioprinom ili mikofenolat mofetilom. Antagonisti endotelin 1-receptora djelotvorni su kod digitalnih ulceracija i PAH-a. U terapiji PAH-a preporučuju se kombinacije sildenafi la i bosentana ili ambrisentana i tadalafi la. Rezultati autologne transplantacije hematopoetskih stanica dvojbeni su. Tirozin kinaza utječe na trombocitni čimbenik rasta i pretvorbeni čimbenik rasta beta. Imatinib, inhibitor tirozin kinaze pozitivno je utjecao na plućnu funkciju. Fresolimumab, monoklonsko protutijelo usmjereno na pretvorbeni čimbenik rasta beta poboljšava kožne promjene. Fibroblasti SSc-a stvaraju visoke razine interleukina 6. Stoga se od tocilizumaba, monoklonskog protutijela usmjerenog na interleukin 6, očekivao pozitivan učinak na bolest, ali javio se rizik od gastrointestinalnih komplikacija. Intravenski imunoglobulini pokazuju pozitivan učinak na kožne i gastrointestinalne promjene. Preporučena terapija za Raynaudov fenomen jesu blokatori kalcijeva kanala, a u drugoj liniji antagonisti unosa serotonina. Systemic sclerosis (SSc) is considered as autoimmune disease of connective tissue. It is characterised with obliterative and proliferative micro vascular involvement, activation of the immune system and excessive fi brosis of skin and internal organs. Th e possibility of genetic infl uences in disease progression and the role of SSc antibodies for diagnosis are exploring. Th e criteria for SSc established in 2013 by the American College of Rheumatology/European League Against Rheumatism are more sensitive than previous. Modifi ed Rodnan Skin Score remains the best method for the objective assessment of skin. Micro vascular changes are observed by videocapillaroscopy what is useful for early diagnosis and prognosis. Digital ulcers are considered as an early manifestation of vasculopathy. Lung complications including interstitial lung disease and pulmonary artery hypertension, can be superimposed and they are considered as the major cause of death in SSc. Myocardial fi brosis is associated with diastolic dysfunction and high risk of cardiac arrhythmias. Since the induction of ACE-inhibitors the kidney complications like renal crisis are less but the patients on glucocorticoids are on the great risk for kidney damage. Oesophageal and ano-rectal involvement are the earliest involvement of gastrointestinal tract. Barrett’s oesophagus, GAVE or watermelon stomach as well as intestinal pneumatosis, pseudodiverticulosis andcolonis telangiectasias may appear. Immunosuppressive therapy is recommended in diff use cutaneous SSc with rapidly progressive interstitial lung disease, starting with cyclophosphamide and next switching to azathioprine or mycophenolate mofetil. Endothelin1 receptor antagonist improved digital ulcers and PAH. Combination therapy of siledanfi l and bosentan or ambrisentan and tanadanafi l is recommended for PAH. Th e results of hematopoietic stem cell transplantation are doubtful. Platelet-derived growth factor and transforming growth factor-β are infl uenced by tyrosine kinase. Imatinib, the tyrosine kinase inhibitor showed the improvement of lung function. Fresolimumab, a monoclonal antibody to transforming growth factor-β, improved skin disease. SSc fi broblasts produce high levels of interleukin-6. Tocilizumab, monoclonal antibody against interleukin-6 is expected to improve the disease, but the risk of gastrointestinal complications appears. Intravenous immunoglobulin’s showed effectiveness in skin and gastrointestinal changes. Th e recommended therapies for Raynaud phenomenon are calcium channel blockers and for second line serotonin uptake antagonists. |
| وصف الملف: | application/pdf |
| اللغة: | Croatian |
| تدمد: | 2459-6159 0374-1338 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=od_______951::fa8a3ce6f377c950b09858e685a9cd0bTest https://hrcak.srce.hr/file/277610Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.od.......951..fa8a3ce6f377c950b09858e685a9cd0b |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 24596159 03741338 |
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