Access restricted to higher education institution's students and staff
master's thesis
Kožna obilježja sistemske skleroze (sklerodermije)

Viana Medanić (2016)
Metadata
TitleKožna obilježja sistemske skleroze (sklerodermije) : klinička slika, dijagnostika i suvremena terapija
AuthorViana Medanić
Mentor(s)Sandra Peternel (thesis advisor)
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.
Keywordssystemic sclerosis limited scleroderma diffuse scleroderma nailfold capillaroscopy Raynaud phenomenon autoimmunity fibrosis
Committee MembersMarija Kaštelan (committee chairperson)
Ines Brajac (committee member)
Srđan Novak (committee member)
GranterSveučilište u Rijeci
Medicinski fakultet
Lower level organizational unitsKatedra za dermatovenerologiju
PlaceRijeka
StateCroatia
Scientific field, discipline, subdisciplineBIOMEDICINE AND HEALTHCARE
Clinical Medical Sciences
Study programme typeuniversity
Study levelintegrated undergraduate and graduate
Study programmeMedicine
Academic title abbreviationdr. med.
Genremaster's thesis
Language Croatian
Defense date2016-07-15
Parallel 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.
Parallel keywords (Croatian)sistemska skleroza ograničena skleroderma difuzna skleroderma kapilaroskopija Raynaudov fenomen autoimunost fibroza
Resource typetext
Access conditionAccess restricted to higher education institution's students and staff
Terms of usehttp://rightsstatements.org/vocab/InC/1.0/
URN:NBNhttps://urn.nsk.hr/urn:nbn:hr:184:554741
CommitterBosa Licul