| Abstract | Sistemska skleroza je rijetka, autoimuna bolest vezivnog tkiva koja zahvaća nekoliko
organskih sustava, od kože do unutrašnjih organa poput srca, pluća, bubrega, probavnog i
muskuloskeletnog sustava. Etiopatološki, razmatraju se teorije o vaskularnoj, imunološkoj i
vezivno-tkivnoj podlozi bolesti koje su u složenoj interakciji te rezultiraju vaskulopatijom,
autoimunosti i progresivnom fibrozom tkiva. Stoga je i klinička slika raznolika te ovisi o fazi
bolesti. Kožna očitovanja su zadebljanje kože, Raynaudov fenomen, gubitak facijalne
ekspresije i ulceracije prstiju. Sistemska skleroza se dijeli na dva klinička tipa, ograničenu i
difuznu sklerodermiju. Ograničeni tip karakteriziraju promjene na distalnim dijelovima udova
i licu, a moguć je razvoj plućne arterijske hipertenzije. U difuznom obliku bolesti, fibrotične
promjene se šire proksimalnije, a zahvaćaju i vitalne organe s ranim smrtnim ishodom. Dolazi
do fibroze srca i pluća, bubrežne krize, probavnih poremećaja i artritisa. U dijagnostici bolesti
uzimaju se u obzir kliničke manifestacije, kapilaroskopija proksimalnog kožnog nabora nokta,
prisustvo karakterističnih autoantitijela, a u slučaju zahvaćenosti unutarnjih organa, redovito
se obavljaju pregledi specifični za taj organ. Liječenje se sastoji od općih mjera poput
izbjegavanja hladnoće, prestanka pušenja i nanošenja krema za njegu, farmakološkog
liječenja, fizikalne terapije te psihoterapije. Za liječenje Raynaudovog fenomena i ulceracija
prstiju koriste se blokatori kalcijskih kanala, analozi prostaciklina, sildenafil i antagonist
endotelinskih receptora bosentan. Antifibrotičko djelovanje imaju imunomodulatori
metotreksat, ciklofosfamid, mikofenolat mofetil, a potencijalno je korisna i biološka terapija
te transplantacija krvotvornih matičnih stanica. Zahvaćenost pojedinih organskih sustava
zahtijeva simptomatski i individualni pristup liječenju. |
| Abstract (english) | Systemic sclerosis is a rare autoimmune connective tissue disease which affects
several organ systems, from skin to internal organs such as heart, lungs, kidneys, digestive
and musculosceletal system. Etiopathologically, there are vascular, immunlogical and
connective tissue theories and their complex interaction results in vasculopathy,
autoimmunity and progressive tissue fibrosis. Therefore, clinical presentation varies greatly
and depends on the stage of the disease. Skin findings are skin sclerosis, Raynaud
phenomenon, impaired facial expression and digital ulcers. There are two clinical forms of
systemic sclerosis, limited and diffuse scleroderma. Limited form is characterized by distal
involvement of limbs and the face, but patients can also develop pulmonary arterial
hypertension. Diffuse form has more extensive fibrosis with internal organ involvement and
early death is possible. Fibrosis of heart and lungs, renal crisis, digestive disorders and
arthritis may occur. Diagnostic approach includes clinical findings, nailfold capillaroscopy,
presence of characteristic autoantibodies and, due to internal organ involvement, specific
organ examination must be regularly performed. Therapy consists of basic measures such as
protection from the cold, avoidance of smoking, application of creams, pharmacological
treatment, physical therapy and psychotherapy. Raynaud phenomenon and digital ulcers are
treated with calcium channel blockers, prostacyclin analogs, sildenafil and the endothelin
receptor antagonist bosentan. Antifibrotics that have been used are immunomodulators such
as methotrexate, cyclophosphamide, mycophenolate mofetil, and in some cases, biologic
immunotherapy and even haematopoietic stem cell transplantation are considered. Organbased
treatment requires symptomatic and individual therapeutic approach of this disease. |
