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